IgG-4 Related Retroperitoneal Fibrosis: A Rare Association with Riedel’s Thyroiditis Jon Pacella MS4a, Soamsiri Niwattisaiwong MDb, David Newman MDb aUniversity of North Dakota School of Medicine & Health Sciences, Grand Forks ND; bDepartment of Endocrinology, Sanford Health, Fargo ND

Case Presentation Case Discussion Conclusion

A 53-year-old male with history of RT previously treated IgG4-RD is an immune-mediated fibroinflammatory condition capable of affecting multiple organs. It is • Presence of inclusion, absence with isthmectomy for compressive symptoms relief who characterized by extensive fibrosis in various organs including the pancreato-hepato-biliary system, presented with one week of severe localized lower retroperitoneum, mesentery, aorta, salivary and lacrimal glands. of exclusion, and inclusion abdominal and suprapubic pain. He denied any fever, gastrointestinal symptoms, genitourinary symptoms, or Retroperitoneal fibrosis in IgG4-RD can present with poorly localized pain in the back or lower abdomen, leg criteria = 26 weight loss. He was initially diagnosed with acute edema, or from ureteral or prostate involvement. prostatitis and was treated with ciprofloxacin without Figure 1: Initial Imaging Figure 2: Pathology • Met criteria to diagnose IgG4-RD improvement of symptoms, which prompted him the second visit to the emergency room. The physical exam • Steroid treatment lead to demonstrated a flat, soft abdomen with normal bowel decreasing fibrosis in sounds and no palpable masses, but with diffuse tenderness across the lower abdomen, especially in the surrounding retroperitoneal right lower quadrant and suprapubic region. organs The patient underwent a non-contrast CT scan of the abdomen and pelvis with findings significant for an • Likely represents a rare case of extensive, predominantly right-sided retroperitoneal mass encircling the aorta, inferior vena cava and IgG4-RD RF in RT patient proximal right producing severe of the right with massive distention of the pyelocaliceal system and proximal ureter and References marked loss of renal cortex (Figure 1). The patient underwent right ureteral stent placement, which partially resolved hydronephrosis and restored 1. Umehara H, Okazaki K, Masaki Y, et al. A kidney function. The CT-guided biopsy of the Figure 1: predominantly right-sided aortic novel clinical entity, IgG4-related disease retroperitoneal mass revealed fibro-inflammatory tissue (IgG4RD): general concept and details. without specific features (Figure 2). The Retroperitoneal encircling aorta and IVC Figure 2: H&E and IgG4 stain immunohistochemistry staining was notable for IgG4 Mod Rheumatol 2012; 22:1. positive plasma cells (Figure 2) and CD68 positive Figure 3: Follow-Up Imaging Figure 4: ACR/ELAR 2019 IgG4-RD Criteria 2. Kamisawa T, Zen Y, Pillai S, Stone JH. histiocytes. IgG4-related disease. Lancet. 2015 Apr The patient was finally diagnosed with IgG4-related ▪ Presence of Inclusion criteria AND 11;385(9976):1460-71 systemic fibrosclerosis. The additional lab testing ▪ Absence of exclusion criteria AND 3. Khosroshahi A, Stone JH. A clinical showed normal LDH, uric acid and IgG4 levels. He was ▪ overview of IgG4-related systemic disease. started on high-dose prednisone at 60 mg daily. Inclusion criteria ≥20 Curr Opin Rheumatol 2011; 23:57. Throughout this time, he developed acute renal failure ▪ Histopathologic findings 4. Brito-Zerón P, Bosch X, Ramos-Casals M, requiring additional stent placement by as well ▪ Lymphocytic infiltrate, IgG4/hpf, as refractory pain necessitating the use of narcotics. Stone JH. IgG4-related disease: Advances Over the next few months, along with continuing IgG4/IgG ratio in the diagnosis and treatment. Best Pract decrease in the size of the retroperitoneal mass on the ▪ Serologic findings Res Clin Rheumatol. 2016 Apr;30(2):261- follow-up CT (Figure 3), his analgesic requirements ▪ Imaging findings 278 began to decline. His renal function improved and he 5. Carruthers MN, Khosroshahi A, Augustin T, was able to taper prednisone to a lower dose. et al. The diagnostic utility of serum IgG4 Given the presence of an IgG4 positive plasma cell concentrations in IgG4-related disease. Ann infiltrate, retroperitoneal fibrosis and history of RT, the Figure 2: Decreased size (7.7 vs 8.1 cm) of mass diagnosis of IgG4-related retroperitoneal fibrosis in a Rheum Dis 2015; 74:14. patient with Riedel’s thyroiditis was made. RT is considered an IgG4-RD of the thyroid gland. Approximately one third of patients with RT eventually 6. Khosroshahi A, Wallace ZS, Crowe JL, et manifest other signs of systemic fibrosis over a 10-year period, although there are only rare case reports of al. International Consensus Guidance RT and RF. Recognition of the risk of developing other features of IgG4-RD in patients with RT is important. Statement on the Management and Prompt recognition of extrathyroidal IgG4-RD will aid early diagnosis and treatment. Treatment of IgG4-Related Disease. Arthritis Rheumatol 2015; 67:1688. Diagnosing Kryptonite: an abnormal presentation of thyrotoxic periodic paralysis Kirsten Hager, MS3 University of North Dakota School of Medicine & Health Sciences, Grand Forks, ND 58012-9037

Introduction Case Description Discussion Closing Notes

• Thyrotoxic periodic paralysis (TPP) is a potentially fatal complication • A 33-year-old male presented to the ER for the second time in one • TPP is highly prevalent among young Asian males, specifically • Diagnosis of TPP during the initial presentation is often hindered of hyperthyroidism and usually presents with acute muscle weakness day; with a complaint of weakness of bilateral lower and upper Chinese, Japanese, Vietnamese, Filipino, Koreans, Malays, and and confused with the more common etiologies of hypokalemia and Indian.5,7 lower-extremity paralysis, partly because of the subtleness of the and hypokalemia.1 extremities of a few hours' duration. thyrotoxicosis and partly because of unawareness about the disorder • Diagnosis is confirmed by the presence of both hypokalemia and an • Diagnosed with Graves’ disease approximately one month prior and • Variation in HLA antigen subtypes such as DRw8, A2, Bw22, Aw19, amongst physicians. increased level of T4 and T3. started on methimazole. Medication was discontinued after the B17, B5, and Bw46 in particular ethnic populations may make them • Occurrences of TPP have been rising due to increasing Hispanics development of a whole-body rash accompanied by severe itching more susceptible to TPP.9,10 • Treatment includes correction of hypokalemia for the reversal of and he was started on Benadryl. and Asians populations, within the USA and physicians may come paralysis and restoration of euthyroid state for the prevention of • TPP is a rare complication of hyperthyroidism usually presenting across this disorder more frequently. future attacks.5 • The rash and itching continued over the long weekend, so the patient between ages 30-50.1-7 It is sporadic in its pattern of onset and is • Treatment with potassium supplements and non-selective beta- went to the ER where he was given epinephrine, solumedrol, and consistently a consequence of thyrotoxicosis.4 Benadryl for the rash and was sent home. blockers should be initiated as soon as a diagnosis is made, and • The hypokalemia is the result of the intracellular shift of K+ due to monitoring of the serum potassium level should be performed • Pertinent physical exam findings: diminished strength in bilateral increased activity of the Na+/K+-ATPase pump under the influence frequently to prevent rebound hyperkalemia. lower (1/5) and upper (3/5) extremities. No patellar reflexes of increased thyroid hormones.1-5 appreciated, but normal reflexes elsewhere. • Sudden onset weakness is typical; starting in the proximal muscles of • Abnormal labs were as noted in figure 2; other lab values including the lower extremities and progressing to involve all four extremities CBC, renal and liver function tests were within normal ranges. in 80% of cases. The patient can suffer weakness to total paralysis which then may last from a few hours to three days.1 • Treatment includes disruption of the shift of potassium by using potassium replacement, non-selective beta-blockade, and amending Figure 2. Pertinent Patient Labs the underlying hyperthyroid state.14 K+ 1.5 Normal - 3.6 to 5.2 mmol/L • Patients are given K+ to quicken muscle recovery and shield from Free T3 4.9 Normal - 2.3 to 4.1 pg/mL Figure 3.Na+/K+-ATPase Pump any cardiopulmonary complications TSH <0.01 Normal – 0.5 to 3.0 uIU/mL • Rebound hyperkalemia occurs in roughly 40% of patients, especially References 1. Garla VV, Gunturu M, Kowuru KR, Salim SA. Thyrotoxic periodic paralysis: case report and review of the literature. if they were administered >90 mEq of potassium chloride within the Electron Physician. 2018 Aug 25; 10(8):7174-9.

first 24 hours11 due to the release of the intracellularly sequestered 2. Al Moteri BL, Aslam M. Thyrotoxic periodic paralysis: A case report. Int J Health Sci (Qassim). 2017 Jan-Mar; 11(1):1-2. Treatment 3. Tamai H, Tanaka K, Komaki G, Matsubayashi S, Hirota Y, Mori K, Kuma K, Kumagai LF, Nagataki S. HLA and thyrotoxic potassium and phosphate following initial treatment.13 • 40 mEq KCl was provided over the next 16 hours with the potassium periodic paralysis in Japanese patients. J Clin Endocrinol Metab. 1987;64(5):1075–1078. level correcting to 5.5 mmol/L and the weakness resolving. • Propranolol can alleviate TPP where KCl is not effective as it 4. Lin SH. Thyrotoxic periodic paralysis. Mayo Clin Proc. 2005;80(1):99–105. obstructs the intracellular shift of potassium and phosphate by 5. Majhi S, Mehta KD, Rohil V. Thyrotoxic hypokalaemic periodic paralysis in a man from Nepal. BMJ Case Rep. 2009; 2009. • The next day, the patient experienced another drop of potassium Pii: bcr05.2009.1836 lessening the hyperadrenergic stimulation of Na+/ K+–ATPase.12 6. Meseeha M, Parsamehr B, Kissell K, Attia M. Thyrotoxic periodic paralysis: a case study and review of the literature. J accompanied by the return of weakness in his lower extremities. Community Hosp Intern Med Prospect. 2017 Jun 6; 7(2):103-6. • Restoration of an euthyroid state to helps to prevent future attacks. 7. Sinharay R. Hypokalemic thyrotoxic periodic paralysis in an Asian man in the United Kingdom. Emerg Med J. 2004 Jan; • Another 60 mEq of KCl was administered over the next 16 hours to 21(1):120-1. This may be obtained using methimazole or propylthiouracil, but the normalize patient’s potassium levels. 8. Salih M, van Kinschot CMJ, Peeters RP, de Herder WW, Duschek EJJ, van der Linden J, van Noord C. Thyrotoxic periodic use of anti-thyroid medications led to relapse in 56% of patients paralysis: an unusual presentation of hyperthyroidism. Neth J Med. 2017 Oct; 75(8):315-20 • Patient was discharged to follow up with his endocrinologist to within 7 months. 9. Tachamo N, Lohani S, Nazir S, Juliano N. Paralysis that easily reverses: a case of thyrotoxic periodic paralysis. BMJ Case 1 Rep. 2017 Jan 30; 2017. Pii: bcr2016218951.

discuss continued treatment of the Graves’ disease. 10. Lam L., Nair R.J., Tingle L. Thyrotoxic Periodic Paralysis. Baylor Univ. Med. Cent. Proc. 2006;19:126–129. doi: • Most literatures suggest radioiodine ablation or thyroidectomy as the 10.1080/08998280.2006.11928143. definitive treatment of hyperthyroidism for the resolution of TPP. 11. Lu KC, Hsu YJ, Chiu JS, Hsu YD, Lin SH. Effects of potassium supplementation on the recovery of thyrotoxic periodic paralysis. Am J Emerg Med. 2004;22(7):544–547.

12. Manoukian MA, Foote JA, Crapo LM. Clinical and metabolic features of thyrotoxic periodic paralysis in 24 episodes. Arch Intern Med. 1999;159(6):601–606.

13. Tassone H, Moulin A, Henderson SO. The pitfalls of potassium replacement in thyrotoxic periodic paralysis: a case report and review of the literature. J Emerg Med. 2004;26(2):157–161. Figure 1. Events Leading to TPP Presenting as Obstructive Jaundice Mikayla Forness, MSIIIa, Laura Nichols, MDb University of North Dakota School of Medicine and Health Sciences, Grand Forks, ND; University of North Dakota Department of Medicine, Fargo, ND

Rates of Case Presentation Discussion Chest Radiography Stage Spontaneous Suggested Treatment Results • A 21-year-old male presented with acute epigastric pain and • Diagnosis of sarcoidosis relies on three criteria:1 Resolution obstructive jaundice. – A compatible clinical and radiographic presentation 0 Normal – None • CT of the abdomen and pelvis demonstrated a mass in the – Pathologic evidence of noncaseating granulomas Bilateral hilar porta hepatis region, appearing to compress the common I 55% to 90% None lymphadenopathy bile duct. – Exclusion of other diseases with similar findings (e.g. malignancy, infection) • Labs upon admission showed: alkaline phosphate 194, AST Bilateral hilar 102, ALT 252, total bilirubin 3.5, and direct bilirubin 2.8. • Sarcoidosis is a granulomatous disease characterized by the formation of II lymphadenopathy and 40% to 70% None non-caseating granulomas with epithelioid giant cells in affected organs. The pulmonary infiltrates • Gastroenterology was consulted and performed upper most commonly involved organs are the mediastinal lymphatic system (95- Pulmonary infiltrates esophageal ultrasound (EUS), which revealed three Steroids or 98%) and lungs (>90%) with presenting signs ranging from asymptomatic III without bilateral hilar 10% to 20% enlarged lymph nodes in the porta hepatis region Immunosuppressants disease to shortness of breath, fatigue, fever and weight loss2,3,4,5,6 lymphadenopathy surrounding and compressing the portal vein and distal • Hepatic sarcoidosis is relatively common with incidence of over 50% and common bile duct. Diffuse pulmonary Steroids or peripheral lymphadenopathy can be seen in about 30% of cases. Intra- IV 0% to 5% • Pathology from FNA revealed necrotizing granulomas with a infiltrates with fibrosis immunosuppressants abdominal lymphadenopathy commonly involves nodes in the porta hepatis mixed B- and T-lymphocyte population and negative AFB or surrounding the biliary tract. Biliary tract involvement is much less common Table 1: Radiographic Staging of Sarcoidosis6 and GMS staining. and can manifest in several ways, including mimicking primary biliary • Fungal serology and QuantiFERON were performed cirrhosis, primary sclerosing cholangitis, or extrahepatic biliary Conclusions revealing only histoplasma M band, which was ultimately felt obstruction.1,2,3,4,6 Sarcoidosis can present asymptomatically in many cases and to be reactive due to prior infection. • Cases presenting with obstructive jaundice due to extrapulmonary can affect many organ systems other than the lungs. As our • A chest CT was ordered due to concern for sarcoidosis and lymphadenopathy, such as the patient we present, have rarely been reported. case illustrates, it is important for clinicians to understand both revealed a prominent left hilar lymph node as well as • In nearly two-thirds of patients, spontaneous remission may occur; therefore, the variety of presentations of sarcoidosis, including biliary peribronchial capsular micronodules at the posterior aspect obstruction, as well as the complexities of management of the left upper lobe, consistent with sarcoidosis. (Image 1) treatment is not indicated with asymptomatic stage I or II sarcoidosis progressive cases.1,4,5,6 (Table 1) depending on presenting symptoms. • Pulmonology was consulted and diagnosed the patient with asymptomatic stage II pulmonary sarcoidosis, which was not • Our patient presented a therapeutic challenge given the obstructive jaundice References even in the context of his asymptomatic pulmonary disease, which prompted an indication for initiation of steroid therapy. 1. Buxbaum J, et al. Biliary Sarcoidosis: Early Diagnosis Minimizes the Need for . Am referral for treatment consideration. Journal of Respiratory and Critical Care Medicine. 2013;187(5). • Biliary stent placement did lead to improvement of liver https://doi.org/10.1164/ajrccm.187.5.556. enzyme levels. 2. Blich M and Edoute Y. Clinical Manifestations of Sarcoid Liver Disease. Jour of Gastroenterology and Hepatology. 2004;19(7):732-737. https://doi.org/10.1111/j.1440- • Referral was placed to a tertiary care center for continued 1746.2003.03335.x. management and consideration of steroid treatment for the Image 1: Chest CT with 3. Gaduputi V, Ippili R, Sakam S, et al. Extrahepatic biliary obstruction: an unusual presentation of hepatic sarcoidosis. Clin Med Insights Gastroenterol. 2015;8:19-22. patient’s extrapulmonary lymphadenopathy and biliary contrast showing prominent doi:10.4137/CGast.S22809. stricture secondary to sarcoidosis. 4. Kumar M, Herrera JL. Sarcoidosis and the Liver. Clin Liver Dis. 2019;23(2):331-343. left hilar lymph node doi:10.1016/j.cld.2018.12.012. 5. Salah S, et al. Sarcoidosis. J Fr Ophtalmol (2018), https://doi.org/10.1016/j.jfo.2018.10.002. 6. Soto-Gomez N, Peters JI, Nambiar AM. Diagnosis and Management of Sarcoidosis. Am Fam Physician. 2016;93(10):840-848. Porphyria Cutanea Tarda: A Rare Presentation of Hemochromatosis Michael Storandt, MSIVa and Erik Heitkamp, MDb aUniversity of North Dakota School of Medicine & Health Sciences, Grand Forks, ND; bAdult Hospitalist, Sanford Health, Fargo, ND

Case Presentation Diagnosis Conclusions A 61-year-old female with history of end-stage renal Based on elevated serum porphyrins and urine porphyrins in Porphyria cutanea tarda as the initial presentation disease on dialysis presented with a three-week conjunction with her monoallelic UROD mutation, the patient was for hemochromatosis is a rare phenomenon and history of a blistering rash on the dorsum of her hands diagnosed with porphyria cutanea tarda. This prompted further work- has been infrequently reported [8-10]. However, and face. She had previously undergone a biopsy of up, which demonstrated iron overload in the liver. Genetic testing for hereditary or secondary hemochromatosis should the lesions which demonstrated a pauci-inflammatory hereditary hemochromatosis was negative, allowing for diagnosis of be suspected in any patient presenting with PCT. cell-poor subepidermal cleft. Immunofluorescence secondary hemochromatosis. demonstrated thick homogenous deposition within the walls of superficial dermal vessels with IgG, IgA, and Discussion fibrinogen, a pattern that is seen in sun-damaged skin, References • Porphyria cutanea tarda results from intrahepatic deficiency of but also in porphyria. Figure 1: Hands demonstrating uroporphyrinogen decarboxylase (UROD), which functions to bullae and skin lesions 1. Singal AK. Porphyria cutanea tarda: Recent update. Mol Genet She was admitted, and on exam, was noted to have convert uroporphyrinogen to coproporphyrinogen in the heme Metab. 2019;128(3):271–81. large bullae present on the dorsum of her hands and 2. Vieira FMJ, Nakhle MC, Abrantes-Lemos CP, Cançado ELR, biosynthetic pathway [1]. Reis VMS dos. Precipitating factors of porphyria cutanea tarda in fingers, sparing her palms (Figures 1 and 2). She also Brazil with emphasis on hemochromatosis gene (HFE) had lesions involving her face. She was noted to have • Even with a UROD mutation, other susceptibility factors, such as alcohol abuse, estrogen use, HIV, or mutations. Study of 60 patients. An Bras Dermatol. a dark pigmentation, significantly changed from the hemochromatosis, are required to express the phenotype [1-3]. One study found that 92% of patients 2013;88(4):530–40. presenting with PCT had at least 3 of these susceptibility factors present [4]. 3. Bulaj Z, Phillips J, Ajioka R, Franklin M, Griffen L, Guinee D, et picture seen in her chart (Figure 3). al. Hemochromatosis genes and other factors contributing to the Serum porphyrins were found to be elevated and • Hemochromatosis is a disorder of iron regulation, which may present with cirrhosis, arthralgias, pathogenesis of porphyria cutanea tarda. Blood. subsequent plasma porphyrin fractionation 2000;95(5):1565–71. skin hyperpigmentation, diabetes mellitus, cardiomyopathy, hypogonadism, hepatocellular 4. Egger NG, Goeger DE, Payne DA, Miskovsky EP, Weinman SA, demonstrated elevated uroporphyrin, carcinoma, or osteoporosis [5]. Anderson KE. Porphyria Cutanea Tarda: Multiplicity of Risk heptacarboxyporphyrin, and hexacarboxyporphyrin, Factors Including HFE Mutations, Hepatitis C, and Inherited supporting a diagnosis of porphyria cutanea tarda. • The relationship between PCT and hemochromatosis is due to the impact of iron overload on Uroporphyrinogen Decarboxylase Deficiency. Dig Dis Sci. UROD function as UROD is inactivated through an iron-dependent process [6]. Additionally, clinical 2002;47(2):419–26. This was further confirmed via urine porphyrin 5. Crownover BK, Covey CJ. Hereditary hemochromatosis. Am fractionation and genetic testing showing a remission has been linked to therapeutic depletion of iron stores through phlebotomy [7] Fam Physician. 2013 Feb [cited 2017 Sep 27]. ;87(3):183–90. monoallelic mutation in uroporphyrinogen 6. Elder GH. Porphyria cutanea tarda. Semin Liver Dis. decarboxylase (UROD). 1998;18(1):67–75. 7. Feder JN, Gnirke A, Thomas W, Tsuchihashi Z, Ruddy DA, At presentation, her skin pigmentation was darkened, Basava A, et al. A novel MHC class I-like gene is mutated in and it was noted that ferritin obtained one-month prior patients with hereditary haemochromatosis. Nat Genet. 1996 Aug;13(4):399–408. was elevated. MRI of the liver subsequently 8. Bovenschen HJ, Vissers WHPM. Primary hemochromatosis demonstrated iron overload. She underwent genetic presented by porphyria cutanea tarda: a case report. Cases J. testing, which did not detect C282Y or H63D 2009 Jun;2:7246. mutations, supporting a diagnosis of secondary 9. Edwards M V, Ray JM, Bacon BR. Sporadic Porphyria Cutanea Tarda as the Initial Manifestation of Hereditary hemochromatosis. Hemochromatosis. ACG case reports J. 2019 Nov;6(11):e00247. Figure 2: Bullae and desquamation on Figure 3: Image from patient’s chart (left) 10. Mehrany K, Drage LA, Brandhagen DJ, Pittelkow MR. Association of porphyria cutanea tarda with hereditary dorsum of hand, sparing the palms compared to image taken at presentation (right) hemochromatosis. J Am Acad Dermatol. 2004 Aug;51(2):205–11. Bullous Fixed Drug Eruption Following Administration of the Recombinant Adjuvant Shingrix Vaccine Hallie Anderson, MSIV; Laura Nichols, MD; and Tania Gonzalez Santiago, MD University of North Dakota School of Medicine and Health Sciences and Sanford Health-Fargo Case Presentation Conclusion A 51 year-old female with a past medical history This case highlights the possibility of an acute bullous reaction significant for Crohn’s disease on infliximab, who to the recombinant adjuvant Shingrix vaccine. The vaccine has presented to her primary care provider with a bullous been shown to be very effective at preventing VZV reactivation and rash on her left arm, axilla, and lateral chest wall postherpetic neuralgia and is well tolerated in most patients, so the (Figure 1) with associated subjective fever. Two days benefits of receiving the vaccination outweigh the risks. prior to presentation, she received her second dose of Nonetheless, recognition of acute reactions associated with the recombinant adjuvant Shingrix vaccine. She was not vaccine such as the bullous drug eruption seen in this patient taking any new medications at the time, had not used should be recognized and treated early. Additionally, clinicians any new topical products, and not had a similar rash should be aware of the potential for increased risk in patients with in the past. She was taking infliximab for her Crohn’s preexisting autoimmune conditions. Further investigation and post- disease and was due for her next dose the following marketing monitoring of the recombinant shingles vaccination should focus on delineating the frequency of these reactions and week. On physical exam the patient had diffuse Figure 1: The patient’s Figure 2: Pathology showing epidermal erythema and swelling extending from the mid chest rash two days following necrosis with superficial and deep predisposing patient risk factors. to axilla and down the upper arm with associated vaccination. inflammation with eosinophils and neutrophils bullae, some of which had a central dusky Discussion References appearance. Dermatologic reactions have been reported immediately following 1. Alexander KE, Tong PL, Macartney K, et. al. Liver zoster vaccination in an immunocompromised patient leading to death secondary to disseminated varicella The patient was referred to dermatology and biopsy vaccine administration in previous cases, but this is the first known case zoster virus infection. Vaccine 2018;36: 3890-3893. doi: was performed. PCR from one of the bullae for HSV1, of a reaction to the recombinant Shingrix vaccine. The live Zostavax 10.1016/j.vaccine.2018.05.078 HSV2, and VZV was negative. Punch biopsy revealed an vaccine has been shown to cause fatal disseminated varicella zoster virus acute stratum corneum with epidermal necrosis leading to infections in both immunocompromised 1,2 and immunocompetent 2. Ortiz-Brizuela E, Leal-Vega F, Cuellar-Rodriguez J, et al. Vaccine-derived varicella 3 zoster infection in a kidney transplant recipient after zoster vaccine live bullae formation along with a superficial and deep patients, which is one of the factors that led to the development of an administration. Vaccine 2019;37: 3576-3579. doi: 10.1016/j.vaccine.2019.05.017 interstitial inflammation with numerous eosinophils and inactivated vaccine for the prevention of VZV reactivation. However, this scattered neutrophils (Figure 2). The changes were case provides evidence that immunologically mediated reactions can 3. Harada K, Heaton H, Chen J, et. al. Zoster vaccine-associated primary varicella consistent with a bullous fixed drug eruption in response occur with recombinant shingles vaccine administration despite their infection in an immunocompetent host. BMJ Case Reports 2017. doi: 10.1136/bcr- 2017-221166 to the vaccine. The patient was prescribed prednisone 40 improved safety profile compared to the live vaccination. As in this case, mg daily for 5 days with subsequent taper and patients with may be at increased risk given the 4. Dharamsi FM, Bichener MD, Warner Dharamsi J. Bullous fixed drug eruption triamcinolone cream applied twice daily. Because results immune dysregulation associated with their underlying condition. masquerading as recurrent stevens-johnson syndrome. The Journal of Emergency Medicine 2015; doi: 10.1016/j.jemermed.2014.09.049. of the viral PCR were not available at the time, the patient Development of a bullous fixed drug eruption to medications and was also given a prescription for valacyclovir 1000 mg vaccinations is a rare complication that can appear similar to bullous 5. McNeil MM, DeStefano F. Vaccine-associated hypersensitivity. The Journal of three times a day for seven days. pemphigoid, Stevens-Johnson Syndrome (SJS) and toxic epidermal Allergy and Clinical Immunology 2018;141: 463-472. Doi:10.1016/j.jaci.2017.12.971 necrolysis (TEN).4 The development of a bullous rash is likely due to a delayed-type hypersensitivity reaction, marked by T-cell hyperactivation.5 The reaction is generally self-limited and treated with discontinuation of the offending agent and corticosteroids. A rare presentation of seizure secondary to tricuspid valve endocarditis Kristal Hudson & Dinesh Bande, MD Learning Objectives Discussion • Risk of systemic embolization in IE occurs in 50% of cases. 1 • Identify the neurological complications and their 1,2 pathogenesis that can present in septic patients with – Cranial embolic events occur in <65% of cases. infective endocarditis. • Neurological complications are the most severe extracardiac 3 • Appreciate the role of brain imaging during the complications of IE. progression of neurologic symptoms and managing – Cerebrovascular (stroke, hemorrhage, mycotic aneurysm) the risk of cranial septic embolic in infective A B C – Infectious (meningitis, brain abscess, etc.) endocarditis. Figure 1: Chest CTA from posterior to anterior coronal views (A-C), showing multiple nodular cavitating lesions (yellow circles) – Systemic (encephalopathy, seizure) • Mycotic aneurysms are arterial dilations caused by the infection Case Presentation & Clinical Course spreading outwardly through the vessel wall. 3 A B – Mortality rate of mycotic aneurysm is dependent on unruptured (30%) • 31-year old female with a significant history of IVDU presents to and ruptured (80%) vessels. ED with chest pain and shortness of breath for four days after use of heroin and methamphetamine. Associated symptoms • Patients with new neurologic symptoms undergo brain imaging (CT or include arthralgias, weakness, and fatigue. Patient found MRI), especially during acute phase to thoroughly search for 3,4 tachypneic, hypoxic, and tachycardic. Labs revealed asymptomatic embolisms. leukocytosis, thrombocytopenia, elevated creatinine, liver – MRI can show lesions not visible on CT, especially in IE. enzymes, and lactic acid. Blood cultures were drawn, and she – A study showed that nearly half of asymptomatic patients with IE had was given Vancomycin and Zosyn empirically for severe sepsis. early evidence of cerebral embolization via MRI. • Chest CTA (Figure 1) revealed multiple nodular cavitating lesions • Early appropriate antibiotic therapy remains the cornerstone of IE throughout both lungs. TTE (Figure 2) showed moderate Figure 2: TTE showing tricuspid mobile vegetations (yellow circles); management and decreases the risk of neurologic embolic events. 4 tricuspid regurgitation with mobile vegetations measuring 11 mm A. Parasternal long axis view B. Apical 4 chamber view • Optimal timing of surgery for IE with cerebrovascular complications x 18 mm. Blood cultures positive for MSSA. remains controversial. 4 • Diagnosis of infective endocarditis (IE) was established. A B C • Patient continued to deteriorate to respiratory failure requiring Take Home points sedation and intubation. After a month of intensive care, she was • Neurological complications can arise early in disease course and could discharged to Vibra to continue ventilation weaning and IV Abx. potentially be reduced with early aggressive medical and/or surgical • 10-days later, patient presented after a rapid response involving management. seizure with head trauma. Brain MRI (Figure 3) showed diffuse • The role of neuro imaging in the timing of surgery can aid in reducing leptomeningeal enhancement consistent with meningitis and neurologic comorbidities in IE patients but timing is still unclear. cerebritis. LP showed no clear infection. EEG showed diffuse encephalopathy and no electrographic seizures. Antimicrobial References therapy restarted for which she developed a rash and since 1. Baddour, L. M., Wilson, W. R., Bayer, A. S., Fowler Jr, V. G., Tleyjeh, I. M., Rybak, M. J., ... & Bolger, A. F. (2015). Infective endocarditis in adults: diagnosis, antimicrobial therapy, and management of complications: a scientific statement for healthcare experienced other complications and prolonged hospital course. professionals from the American Heart Association. Circulation, 132(15), 1435-1486. 2. Ruttmann, E., Willeit, J., Ulmer, H., Chevtchik, O., Höfer, D., Poewe, W., ... & Müller, L. C. (2006). Neurological outcome of septic Figure 3: Brain MRI T2 FLAIR from caudal to cranial axial views (A-C); leptomeningeal enhancements cardioembolic stroke after infective endocarditis. Stroke, 37(8), 2094-2099. (yellow arrows) 3. García-Cabrera, E., Fernández-Hidalgo, N., Almirante, B., Ivanova-Georgieva, R., Noureddine, M., Plata, A., ... & Martínez-Marcos, F. J. (2013). Neurological complications of infective endocarditis: risk factors, outcome, and impact of cardiac surgery: a multicenter observational study. Circulation, 127(23), 2272-2284. 4. Novy, E., Sonneville, R., Mazighi, M., Klein, I. F., Mariotte, E., Mourvillier, B., ... & Wolff, M. (2013). Neurological complications of A Rare Renal Complication in a Patient with Alagille Syndrome Noelle Torrance, MSIV; and Lakshmi Pathak, M.D. University of North Dakota School of Medicine and Health Sciences and Sanford Health-Fargo Introduction Case Description Discussion • Alagille syndrome is an autosomal dominant disorder caused by • A 34-year-old male with a past medical history of Alagille • FSGS is rarely reported in the literature to be associated with Alagille a mutation of the Notch signaling pathway, most commonly in the syndrome consisting of secondary biliary cirrhosis, syndrome JAG1 gene hyperlipidemia, hyperuricemia, and recurrent gout initially – Thought to be related to high circulating lipids seen in this syndrome • High degree of penetrance but variable expressivity presented in 2011 with nephrotic range proteinuria and normal that deposit within the glomerulus and may contribute to podocyte creatinine 7 • Frequency of 1 in 30,000 live births1 injury • His serological workup was negative • Diagnosis is based on five • The patient described has proteinuria, CKD stage 4, and FSGS which is ”classical” criteria: • He underwent a renal biopsy which showed secondary FSGS felt to be related to his underlying Alagille syndrome with reduced glomeruli and sclerosis 1. Intrahepatic bile duct • Liver disease is a major cause of morbidity in Alagille syndrome, whereas paucity • Lisinopril was started initially, but not tolerated due to dizziness cardiac and vascular involvement account for most of the mortality 2. Cardiac malformations • He was switched to Losartan, but lost to follow up for a year • Since renal disease can impact liver transplantation, additional studies on (pulmonary artery • He later presented with worsening creatinine and proteinuria and renal involvement in Alagille syndrome are necessary to develop stenosis) most seen recently in the follow up clinic with a creatinine of 3.07 guidelines on evaluation and management 3. Skeletal involvement mg/dL and 1.2 gm proteinuria. (butterfly vertebrae) • The patient is currently being managed with Losartan for the Take Home Points 4. Ocular anomalies proteinuria, allopurinol, and a low salt diet. (posterior embryotoxon) • He has been referred to Mayo clinic for a simultaneous kidney 1. A renal evaluation consisting of serum biochemistry, renal 5. Distinctive facial features and liver transplant. ultrasound, and urinalysis should be standard of care in (prominent forehead, Alagille Syndrome pointed chin, deep set Pathology 2. Additional studies on renal involvement in Alagille syndrome eyes)2 are necessary as renal disease can impact liver transplantation • Figure 2. Renal biopsy • Figure 1. Hallmarks of Alagille Syndrome. BD, bile duct; CV, central vein; HA, of a 5-year-old girl with 3. Studies have suggested that renal involvement be considered hepatic artery; OA, overriding aorta; PS, pulmonary stenosis; PT, portal triad; PV, Alagille Syndrome. a sixth “classical” criterion for diagnosis portal vein; VSD, ventricular septation defect. Reproduced/ adapted with Trichrome staining 3 permission from Development. shows FSGS in two • 3 out of 5 criteria are required for the diagnosis, or 2 if there is glomeruli (dense, pale 4 blue areas); arrows References a family history show podocytes with prominent red • Renal involvement has been seen in 19 to 74% of patients 1. Mitchell E, Gilbert M, Loomes KM. Alagille syndrome. Clinics in Liver Disease. 2018 Nov 1;22(4):625-41. cytoplasmic vacuoles; 2. Shimohata H, Imagawa K, Yamashita M, Ohgi K, Maruyama H, Takayasu M, Hirayama K, Kobayashi M. An Adult Patient – This is consistent with the role of the Notch signaling inset shows an oil-red- with Alagille Syndrome Showing Mainly Renal Failure and Vascular Abnormality without Liver Manifestation. Internal pathway in renal organogenesis5 O stain which Medicine. 2020:4780-20. highlights lipids (red) 3. Mašek J, Andersson ER. The developmental biology of genetic Notch disorders. Development. 2017 May 15;144(10):1743- • This case report is of an adult with Alagille syndrome with deposited in a single 63. doi: 10.1242/dev.148007 capillary loop. 6 4. Kamath BM, Podkameni G, Hutchinson AL, Leonard LD, Gerfen J, Krantz ID, Piccoli DA, Spinner NB, Loomes KM, Meyers Focal Segmental Glomerulosclerosis (FSGS), which highlights K. Renal anomalies in Alagille syndrome: A disease‐defining feature. American journal of medical genetics Part A. 2012 Jan;158(1):85-9. the association of renal manifestations with this syndrome 5. Saleh M, Kamath BM, Chitayat D. Alagille syndrome: clinical perspectives. The application of clinical genetics. 2016;9:75. 6. Davis J, Griffiths R, Larkin K, Rozansky D, Troxell M. Glomerular basement membrane lipidosis in Alagille syndrome. Pediatric Nephrology. 2010 Jun;25(6):1181-4. 7. Joyce Emily, et al. Nephrology. Zitelli and Davis’ Atlas of Pediatric Physical Diagnosis, Pittsburgh: Elsevier. 2018. Epstein Barr Virus Infectious Mononucleosis complicated by profound lymphadenopathy, ruptured duodenal ulcer and sepsis

Kemin Fena, MS3. University of North Dakota School of Medicine and Health Sciences, Grand Forks, North Dakota

Introduction • In hospital, she developed worsening abdominal pain and signs of Discussion • Epstein Barr Virus (EBV) is a common illness causing sepsis. Abdominal CT was ordered, revealing free fluid and • Well-known complications of EBV-IM include hepatosplenomegaly infectious mononucleosis (IM). pneumoperitoneum. Exploratory laparotomy discovered perforated with elevated LFTs and risk of splenic rupture, lymphoproliferative duodenal ulcer which was repaired with a Graham patch. Hepatic • Worldwide, >90% of adults are infected with EBV. IM affects disorders, and lymphomas. Mild hepatitis is not uncommon though artery lymph node biopsy found follicular hyperplasia consistent with up to 70% of adolescents and adults, while infants and steatosis was likely pre-existing in this case. EBV-associated lymphadenitis. children are typically asymptomatic with primary infection. • Multiple cases reported lymphoepithelioma-like carcinoma or • Post-op, she developed acute respiratory failure, aspiration, and signs • The most common clinical symptoms include fatigue, fever, gastric adenocarcinoma contributed to EBV. Oral, skin or colonic of sepsis. She was intubated and moved to the ICU. pharyngitis, and cervical lymphadenopathy1. ulcers are established complications in . • The patient’s symptoms gradually improved with intensive care. She However, only 3 cases reported benign gastroduodenal ulcers in • Although most acute infections are mild and self-limiting, the was discharged home after a 25-day hospital course. immunosuppressed patients2-4, and 4 cases reported gastric or complications vary widely. duodenal ulcers in immunocompetent patients with EBV-IM5-8. • Few cases have reported EBV with concurrent gastric or • In one study, EBV serology was tested in 50 patients with peptic duodenal ulcers, and ulcer perforation significantly increases ulcer disease were tested for EBV-DNA. 35 of 50 ulcer samples had the risk of severe complications. significantly higher EBV viral load versus normal gastric tissue9. • A systematic review cited 6 different studies describing EBV and Case Description coinfection with H. pylori. Their conclusions supported preliminary 10 • A 39-year-old previously healthy woman presented with one- evidence that H. pylori may increase lytic activity of EBV . week history of pharyngitis, cervical lymphadenitis, fever, and • Non-steroidal anti-inflammatory drugs (NSAIDs) likely contributed to fatigue. Monospot test was negative. She was sent home on ulcer formation in this case as she took frequent doses prior to antibiotics. With 3 doses, she failed to improve and developed hospitalization for cervical lymphadenitis. NSAID use with EBV nausea and diarrhea. infection could render the gastric mucosa more susceptible to ulcer • Two days later, she was admitted for acute kidney injury due to formation, but this requires further investigation which is challenging dehydration. Serology was positive for EBV nuclear antigen, IgG due to rare concurrence of these conditions. and IgM. Cervical lymph node biopsy showed lymphoid hyperplasia typical of EBV without evidence of lymphoma. Conclusion • Abdominal imaging revealed splenomegaly and multiple enlarged • Most patients with EBV infections recover uneventfully. lymph nodes consistent with EBV infection. She also had hepatic However, few patients may develop severe complications steatosis with elevated liver enzymes, intrahepatic biliary duct including sepsis and profound lymphadenopathy as dilatation, and mild pancreatic duct dilation. demonstrated in this case. • CT abdomen (Figure) shows prominent gastrohepatic nodes.

Figure. CT abdomen and pelvis noted prominent iliac as well as gastrohepatic lymph nodes (arrow). ACE inhibitor-induced isolated angioedema of the gut presenting as an acute abdomen Marley Foertsch, MS-4, Spencer Campbell, PGY-3, Rajendra Potluri, MD Department of Internal Medicine, Sanford Medical Center, Fargo, ND

Introduction Discussion Conclusion ACE inhibitor-induced angioedema occurs in Four and a half months after his initial This patient experienced symptoms for This patient initially underwent an exploratory only 0.7% of patients who take ACE inhibitors, presentation, the patient went to the ED for a months before the source of his intestinal laparotomy, which confirmed what was seen but with over 40 million patients taking this third time. CT once again revealed small edema was identified. A literature review on CT: gut edema. Given the acuity of his medication it is a noteworthy side effect1. Most bowel wall edema. Upon admission to the suggests that such a delay is not uncommon. presentation (severe hypotension and lactic attention is paid to the life-threatening airway medicine team, ACE inhibitor-induced Virtually all patients presented with abdominal acidosis), a surgery may not have been obstruction ACE inhibitors can cause, and angioedema was considered, and lisinopril pain which, as in this case, may mimic an avoided, but the additional two ED visits, intestinal angioedema is a less well-known was stopped. The next day, MRI enterogram acute abdomen. A case report about hospitalization, and 4.5 months of symptoms manifestation. This can result in delay of showed significant improvement, confirming angioedema from acquired C1 esterase may not have occurred had an etiology of diagnosis and several unnecessary the diagnosis. inhibitor deficiency stated that up to one-third ACE-inhibitor induced angioedema been interventions in patients who experience of patients undergo exploratory laparotomy, considered. One should have a low index of isolated angioedema of the gut. appendectomy, or both during attacks3. suspicion when apparent cause of an Another study reported that 35% of patients acute abdomen is not clear after initial with intestinal angioedema experienced investigations in a patient taking an ACE longstanding abdominal pain before being inhibitor. Case Summary diagnosed2. A 38-year-old male with treated As of 10 months since lisinopril was with lisinopril presented to the emergency If there’s suspicion for angioedema in a discontinued, this patient has had no attacks room after two days of diffuse, debilitating patient taking an ACE inhibitor, the medication requiring additional care. abdominal pain. He was hypotensive and in should be discontinued until another etiology lactic acidosis. Abdominal examination is determined. Imaging can support this revealed diffuse rigidity and tenderness. CT decision, and CT or ultrasound findings References was concerning for viscus perforation, so include mesenteric edema, intestinal wall surgery was consulted. Exploratory thickening, dilated bowel, and/or ascites. One 1. Banerji A, Blumenthal KG, Lai KH, Zhou L. article referred to this as a “stacked coin” Epidemiology of ACE Inhibitor Angioedema Utilizing laparotomy revealed an edematous small a Large Electronic Health Record. J Allergy Clin 3 bowel without ischemia or perforation. The appearance . Most cases involve the small Immunol Pract. 2017;5(3):744–749. patient’s abdomen was left open and he spent intestine, with the majority occurring in the doi:10.1016/j.jaip.2017.02.018 one day in the ICU on pressors before re- jejunum2. 2. Bloom A, Schranz C. Angiotensin-Converting exploration and closure. He stabilized and Enzyme Inhibitor-Induced Angioedema of the Smal was discharged without a diagnosis, and his After cessation of the ACE inhibitor, Bowel—A Surgical Abdomen Mimic. Journal of angioedema should subside in 24-72 hours. If Emergency Medicine. 2015;48(6):e127-e129. lisinopril was restarted. Six weeks later he Accessed through Clinical Key. this condition is not identified and the again presented to the ED with abdominal 3. Ciaccia D, Brazer SR, Baker ME. Acquired C1 pain. He said his abdomen was on fire, and he medication continues, the angioedema will esterase inhibitor deficiency causing intestinal could barely talk due to weakness. He recur with increasing frequency and severity. angioedema: CT appearance. American Journal of improved after pain medications, and his Patients may still experience episodes for Roentgenology. 1993;161(6):1215-1216. symptoms were felt to be due to constipation. several months, but these should decrease in 4. Beltrami L, Zanichelli A, Zingale L, Vacchini R, number and intensity over time4. If symptoms Carugo S, Cicardi M. Long-term follow-up of 111 do not subside, alternative causes of patients with angiotensin-converting enzyme inhibitor-related angioedema. J Hypertens. angioedema such as hereditary angioedema 2011;29(11):2273. and acquired C1 inhibitor deficiency should be explored. Reactive Arthritis: An Unusual Presentation of Acute Clostridium difficile Colitis 1 Anna Reinholz, MS3 2 Devendranath Mannuru, MD 3 Abhishek Matta, MD University of North Dakota School of Medicine and Health Sciences and Fargo Sanford Health

Introduction Labs and Imaging Conclusions • Reactive arthritis is a rare presentation of acute Clostridium difficile Test Urgent Care Hospital Admission Reference • Undiagnosed IBD can also present as C. colitis and requires a high level of clinical suspicion to diagnose WBC 10.3 15.7 (H) 4.0 – 11.0 K/uL difficile colitis, making the diagnosis of Hemoglobin 13.8 12.6 13.5-17.5 g/dL reactive arthritis difficult • Classically, reactive arthritis is associated with Salmonella, Shigella, Platelets 372 329 140 – 400 K/uL Campylobacter, Chlamydia, and Yersinia ESR 86 73 0 – 15 mm/Hr • Diagnosis was made with features of • Clostridium difficile has only been cited as the etiology of reactive Autoimmune testing Urgent Care Hospital Admission Reference aseptic arthritis, a positive pathogen arthritis roughly 50 times in the literature thus far Rheumatoid Factor <15 0-29 IU/mL documentation and negative tissue ANA Screen Negative Negative biopsies of the colon ruling out other ANCA Screen Negative Negative etiologies for diarrhea Initial Presentation to Urgent Care Myeloperoxidase < 0.2 < 0.4 U Antibody • Antibiotics coupled with NSAID therapy • 20-year-old Caucasian male with history of psoriasis presented to Proteinase 3 Antibody < 0.2 < 0.4 U results in excellent prognosis of reactive urgent care clinic with a 2 week history of polyarthralgia and a 3 week Saccharomyces Cervisiae < 10.0 <= 20.0 U arthritis history of nonbloody diarrhea • Extensive workup showed normal rheumatoid factor and was negative Significance for basic enteric panel, tick borne illnesses, and others • C. difficile colitis cases continue to rise • Sent home with prednisone and plan for follow up with rheumatology with widespread antibiotic use and Infectious agent testing Urgent Care Facility Hospital Admission hospital acquired infection Basic Enteric Pathogen Panel Not Detected Toxigenic C. difficile (PaLoc) Not Detected • Important to recognize C. difficile as an Giardia Negative etiological agent of reactive arthritis, Cryptosporidium Negative despite fewer number of case Lactoferrin for leukocytes Positive documentations Babesia species, Negative • Clinicians should think of reactive Ehrlichia/Anaplasma Negative Lyme Disease Nonreactive arthritis when chief complain is severe polyarthralgia developing within 1-4 Mono Screen Negative Figure 1. CT showing abnormal Figure 2. Colonoscopy showing weeks of an enteric infection West Nile Negative cecal wall thickening and concern pseudomembranous colitis which Bacterial blood cultures No growth for inflammatory bowel disease prompted aspirate for C. difficile References Hospital Admission and Course 1. Marwat A, Mehmood H, Hussain A, Khan M, Ullah • Aspirate returned positive for C. difficile toxin and vancomycin was started • Two days later came to the emergency department with worsening A, Joshi M. Clostridium difficile Colitis Leading to • At this point, still more likely that his polyarthralgia was due to undiagnosed Reactive Arthritis: A Rare Complication Associated severe polyarthralgia and diarrhea associated with chills and fatigue inflammatory bowel disease (IBD) flare; however, tissue biopsy confirmed With a Common Disease. J Investig Med High Impact • Physical exam revealed extreme tenderness to palpation of joints diagnosis of reactive arthritis in the setting of C. difficile colitis Case Rep. 2018;6:2324709618767689. Published without erythema or swelling 2018 Mar 30. doi:10.1177/2324709618767689 • No histopathological evidence of chronicity to suggest ulcerative colitis or 2. Legendre P, Lalande V, Eckert C, et al. Clostridium • Denied oral antibiotic use within the past month Crohn’s as the etiology, ruling out IBD difficile associated reactive arthritis: Case report and literature review. Anaerobe. 2016;38:76-80. doi:10.1016/j.anaerobe.2015.12.011 Rituximab: a rare cause of Progressive Multifactorial Leukoencephalopathy Aishwarya Sharma MSI1, Mounika Guduru, MD2, Dinesh Bande, MD1,3, Abhishek Matta, MD1,3 1 University of North Dakota School of Medicine and Health Sciences; 2 Creighton University Medical Center; 3 Sanford Health, Fargo ND Case Description Discussion • A 79-year-old woman with a known history of rheumatoid arthritis on • PML is a rare fatal central nervous system disorder characterized by long-term prednisone (5mg daily) and rituximab (1000mg every six Figure 1: Brain CT axial (a) and infection-induced demyelination of white matter due to the opportunistic months), presented to the emergency room with multiple falls, gait coronal (b) sections showing a reactivating of John Cunningham (JC) polyomavirus in an right cerebellar hypodense lesion difficulty, slurred speech, and confusion. immunocompromised patient [1,2]. with no significant surrounding • On hospital admission, her physical examination revealed right spastic edema or mass effect and left • JC-virus primary infection often occurs during childhood or early hemiparesis (a known residual from her old cerebrovascular insult), cortical and subcortical parietal adolescence but the virus stays dormant in body tissues for long-periods in axial and appendicular ataxia, slurred dysarthria, and vertical encephalomalacia (on coronal immunocompetent individuals. Several studies in the literature reported that nystagmus. section). up to 50% of adults may be seropositive for JC-virus [3]. • She was negative for HIV, HbsAg and Anti-HCV. • However, an intact immune system suppresses viral activation. In an • Computed tomography (CT) scan of the brain showed right pontine and immunocompromised state, the virus becomes reactivated, resulting in right cerebellum hypotenuse irregular lesion without significant disease [4]. surrounding edema or mass effect and left parietal cortical and • Immunocompromised states such as HIV infection, leukemias, lymphomas, subcortical encephalomalacia, likely sequel are of an old vascular insult and different malignancies are well-known to be associated with PML. (Figure 1). Several reported cases in the literature show a strong association of PML • Magnetic resonance imaging (MRI) with gadolinium of the brain and with some immunosuppressants such as natalizumab, fingolimod, dimethyl posterior fossa revealed the right pontine lesion (at the brachial pontis) Figure 2: MRI brain with fumarate, and rituximab [4-6]. as involving the right cerebellar white matter. The lesion was isointense contrast showing an irregular • Rituximab is an anti-CD20 monoclonal antibody used in the treatment of on T1 and hyper intense on T2 and FLAIR sequences. The outer borders right pontine isointense lesion many lymphoproliferative conditions and immune-mediated diseases such on T1 (a), a hyper intense of the right pontocerebellar lesion were more defined than the inner signal on T2 (c) and FLAIR (d) as non-Hodgkin’s lymphoma, neuromyelitis optica, psoriasis, and border and there was minimal surrounding edema, with no mass effect sequences, with the extension rheumatoid arthritis [7] via various mechanisms of action such as antibody- on the adjacent fourth ventricle. of the lesion to the right dependent cytotoxicity, cell-mediated cytotoxicity, apoptosis, and direct • The lesions showed diffusion restriction on diffusion-weighted images cerebellar hemisphere showing sensitization of cells to chemotherapy [8]. (DWI) and apparent diffusion coefficient (ADC) maps. The contrast minimal edema adjacent to fourth ventricle. The lesion Conclusion study showed heterogenous leading edge enhancement at the right shows restriction on DWI (e) • As PML can be fatal, patients receiving rituximab should be meticulously pontocerebellar lesion (Figure 2). A smaller T2 and FLAIR hyper and ADC map (f), and monitored. intense lesion was also noted in the left middle cerebellar peduncle, heterogenous enhancement at • Regular follow-up of CD4b is essential, and abrupt discontinuation of extending to the left cerebellar hemisphere with heterogenous the margins (b, g, and h). enhancement. Another T2 hyper intense rituximab is fundamental to reduce mortality if PML is suspected. • Although there is no definitive FDA approved medication for PML to date, • These radiological features, along with the clinical features in a smaller lesion is demonstrated at the left middle cerebellar rituximab-treated immunocompromised patient are highly suggestive of plasma exchange to remove circulating drug as well as stopping the peduncle and left cerebellar offending drug represent the treatment of choice for these cases. Progressive multifocal leukoencephalopathy (PML). hemisphere (c) and • Treatment options were discussed with the patient, However, the patient heterogenous enhancement (h). References 1. Palazzo E, Yahia SA: Progressive multifocal leukoencephalopathy in autoimmune diseases. Vol. 79, Joint Bone Spine. 2012. p, 351:5. and her family decided to adopt a palliative approach, and the patient 2. Pavlovic D, Patera AC, Nyberg F, Gerber M, Liu M: Progressive multifocal leukoencephalopathy: Current treatment options and future was discharged from the hospital to a nursing home where she died perspectives. Vol. 8, Therapeutic Advances in Neurological Disorders. 2015. p, 255:73. 10.1177/1756285615602832 3. Chen Y, Bord E, Tompkins T, Miller J, Tan CS, Kinkel RP.: Asymptomatic Reactivation of JC Virus in Patients Treated with Natalizumab. N Engl J within a month. Med [Internet. 2009:1067-74. 4. Grebenciucova E, Berger JR: Progressive Multifocal Leukoencephalopathy. Vol. 36, Neurologic Clinics. 2018. p, 739:50. 5. Kanda T: Neuropathology of natalizumab-associated progressive multifocal leukoencephalopathy. Brain and Nerve. 2015, 67:891-901. 26160817 6. Bohra C, Sokol L, Dalia S: Progressive Multifocal Leukoencephalopathy and Monoclonal Antibodies: A Review. Cancer Control. 2017, 24:1- 9. 10.1177/1073274817729901 7. Clifford DB, Ances B, Costello C, Rosen-Schmidt S, Andersson M, Parks D.: Rituximab-associated progressive multifocal leukoencephalopathy in rheumatoid arthritis. Arch Neurol. 2011, 68:1156-64. 1108037 8. Gea-Banacloche JC: Rituximab-associated infections. Semin Hematol. 2010, 47:187-98. Infectious Complications of Recreational Urethral Sounding with Retained Foreign Body Aishwarya Sharma MSI1, Dubert Guerrero, MD1,2 ; 1 University of North Dakota School of Medicine and Health Sciences; 2 Sanford Health, Fargo ND Case Description Imaging • A 62-year-old male was admitted for evaluation and management of • Foreign bodies in the urinary tract increase the risk of urinary tract 3 weeks of progressive back pain radiating to his legs with a history of infection [1]. Such infections are often recurrent as a result of bacteria nicotine and amphetamine abuse, hypertension, mood disorder, and persistence within or on the foreign body and sometimes because of poor neuropathic pain. Figure 1. Magnetic resonance drainage. imaging showing abnormal • Physical examination including neurologic assessment was • Bacteremia and risk of endotoxemia should always be borne in mind and enhancement at level T12-L1 risk may increase in the process of extraction of the foreign body. unremarkable. consistent with diskitis. • White blood cell count was normal at 8.6 K/ul but inflammatory • Complications such as calculus formation have been widely reported in markers erythrocyte sedimentation rate and C-reactive protein were association with migrated intrauterine contraceptive devices and surgical significantly elevated at 94 mm/hr and 110 mg/L respectively. needles [3,4]. • Magnetic resonance imaging (MRI) of the back revealed abnormal • Radiologic evaluation is necessary to determine the exact size, number, marrow signal and enhancement in the T12 and L1 vertebral bodies and nature of the foreign body. Ultrasonography is usually able to localize centered at the T12-L1 disc space likely secondary to diskitis. There is Figure 2. Computed the foreign body to the bladder and determine the exact size and number corresponding abnormal paraspinal edema and enhancement with a tomography urogram but is unable to evaluate the exact nature. probable 1.7 cm intramuscular abscess in the left psoas muscle demonstrating coiled tube • Cystoscopy confirms the diagnosis, and some foreign bodies are (Figure 1). like structure in the urinary successfully removed during the process [5]. Endoscopic and minimally • The abscess was aspirated and grew Staphylococcus epidermidis. He bladder. invasive techniques should be encouraged. was placed on intravenous cefazolin. • Our patient was treated for recurrent urinary tract infections of the same • He also complained of dysuria and hematuria. Further history pathogen for over 6 months before he eventually ended up with revealed frequent urinary tract infections with S. epidermidis for bacteremia and deep-seated infectious complication of diskitis that was which he received different courses of oral antibiotics without relief. managed both medically and surgically. • A computed tomography urogram was done for persistent urinary Figure 3. Foreign body Conclusion symptoms and identified a tubular 1.5cm diameter peripherally extracted from open • The presence of a foreign body in the urinary bladder from urethral calcified 10-12cm structure with tapered distal ends and intermediate cystotomy. 16.9 cm in length sounding is rare and therefore requires a high index of suspicion for internal attenuation coiled in the urinary bladder (Figure 2). and 1.5 cm in diameter diagnosis. • After careful history, he admitted his girlfriend inserted a sex toy • Presentation is sometimes delayed with complications that may be life- shaped like a fishing worm into his few months back, but he threatening. did not remember if it was removed. • It should be considered in patients with recurrent • He underwent cystoscopy and open cystolithotripsy in which the and poor response to antibiotic therapy. Such patients should have foreign body was extracted (Figure 3). The patient was discharged to Discussion radiologic evaluation in order to facilitate diagnosis and treatment. skilled nursing facility to complete IV antibiotics for 6 weeks followed • Foreign bodies localized to the urogenital tract represent a relatively rare by oral cephalexin for additional 6 weeks. pathology and their presence may predispose to infection, calculus formation, References • Repeat MRI showed destruction in the intervertebral disc between and bladder outlet obstruction, bacteremia and abscesses [1]. 1. Van Ophoven A, deKernion JB. Clinical management of foreign bodies of the genito-urinary tract. Urology. 2000;164(2):274–287. T12 and L1 and paraspinal soft tissue enhancement. He eventually • Most patients are hesitant to admit urethral sounding or the act of inserting an 10.1097/00005392-200008000-00003 required a T12 & L1 corpectomy and posterior instrumented fusion of 2. Aliabadi H, Cass AS, Gleich P, Johnson CF: Self-inflicted foreign bodies involving lower urinary tract and male genitals. Urology. 1985, object for autoerotic, psychiatric, therapeutic, or any other reasons [2]. Patients 26(1):12–16. 10.1016/0090-4295(85)90245-6 T9-L3. 3. Guvel S, Tekin MI, Kilinc F, Pekircioglu L, Ozkardes H: Bladder stones around a migrated and missed intrauterine contraceptive present when symptoms develop or there are complications. device. Int J Urol. 2001, 8(2):78–79. 10.1046/j.1442-2042.2001.00249.x 4. Dermici D, Ekmekcioglu O, Demitras A, Gulmez I: Big bladder stones around an intravesical migrated intrauterine contraceptive • Hematuria may occur from trauma due to self-manipulation or rough objects device. Int Urol Nephrol. 2003, 35(4):495–496. 10.1023/b:urol.0000025624.15799.8d 5. Kim ED, Moty A, Wilson DD, Zeagler D: Treatment of lower urinary tract obstruction secondary to an expandable foam that injure the bladder wall. sealant. Urology. 2002, 60(1):164. 10.1016/s0090-4295(02)01657-6 Concomitant systemic lupus erythematosus and ankylosing spondylitis Carissa S. Klarich1 BA, Laura M. Nichols1 MD University of North Dakota School of Medicine and Health Sciences1

Introduction Case Report Discussion • Systemic lupus erythematosus (SLE) • A 48-year-old female with a history of SLE controlled on • Concomitant SLE and AS rarely reported in the literature 1 – "the great imitator” azathioprine presented with subacute intermittent low back • SLE can cause a polyarticular synovitis, yet abnormalities within pain radiating to her groin and left flank pain 2 – frequently associated with overlap syndromes with other synovial lined sacroiliac joints are reported in only a few cases rheumatologic illnesses such as rheumatoid arthritis • Saw improvement with chiropractic intervention but • Diagnosis of AS includes radiological changes in the sacroiliac experienced subsequent worsening of her pain • Overlap with ankylosing spondylitis (AS) rarely reported joints and joint pain >3 months that improves with exercise • No red flag symptoms • We present a case of a patient with a known diagnosis of • Still unknown if genetic factors like HLA-B27 with DR3 and/or SLE who presented with and was ultimately – weakness, numbness, tingling, bowel or bladder DR5 play a critical role or whether the sacroiliitis is simply an diagnosed with an SLE-AS overlap syndrome dysfunction independent and infrequent manifestation of SLE 3 • Left flank tenderness to palpation • Genetic testing showing HLA-B27+ not necessary for diagnosis • Given pain on palpation and lack of red flag symptoms, it was • AS is an uncommon (0.3% of all cases) cause of low back pain felt the most likely cause of her pain was muscle strain • Our case illustrates the importance of considering inflammatory • Concern for a renal stone with radiation of flank pain to the back pain in the differential diagnosis, particularly in patients with groin, thus CT kidney stone protocol and UA were performed positive histories and rheumatologic conditions like SLE • UA was negative • CT was negative for renal stones but showed chronic Figure 2. Ankylosing sclerotic and erosive changes on either side of both SI joints spondylitis. Axial view with typical sequela of sacroiliitis and a chronic left-sided pars from CT showing interarticularis defect at L5 (Figure 1 and 2) bilateral SI joint • Discussed with her rheumatologist given sacroiliitis on chronic sclerotic and imaging erosive changes with typical sequela of • At rheumatology follow up, she reported intermittent low back sacroiliitis. pain since her 20s in a pattern of stiffness at night with improvement with movement • Diagnosed with ankylosing spondylitis in addition to her SLE – Based on inflammatory back pain in the presence of SI joint inflammation on imaging Figure 1. Ankylosing spondylitis. Coronal view from CT kidney References • HLA-B27 measurement was not required for diagnosis 1. Tarhan F, Argın M, Can G, Özmen M, Keser G. Coexistence of systemic lupus erythematosus and stone protocol showing chronic sclerotic and erosive changes ankylosing spondylitis: another case report and review of the literature. Eur J Rheumatol. 2014;1(1):39-43. of bilateral SI joint. • Pain was treated successfully with celecoxib doi:10.5152/eurjrheum.2014.008 2. Chandrasekhara PK, Jayachandran NV, Thomas J, Narsimulu G. Systemic lupus erythematosus and dermatomyositis with symptomatic bilateral sacroiliitis: an unusual and interesting association. Mod Rheumatol. 2009;19(1):84-86. doi:10.1007/s10165-008-0120-6 3. Kohli M, Bennett RM. Sacroiliitis in systemic lupus erythematosus. J Rheumatol. 1994;21(1):170-171. Right Central Retinal Artery Occlusion requiring focal thrombolysis with tPA Pranish A. Kantak MPH1, Carissa S. Klarich BA1, Alexander Drofa MD2 Department of Surgery, University of North Dakota School of Medicine and Health Sciences, Grand Forks, ND1 Department of Neurosurgery, Sanford Health, Fargo, ND2

Introduction Case Report Discussion • Central Retinal Artery Occlusion (CRAO) constitutes an • A 66-year-old female, with a past medical history of • The clinical findings of CRAO are often easily recognized ophthalmic emergency hypertension, dyslipidemia, and 50-pack-year smoking making the diagnosis straightforward • CRAO most commonly occurs through embolic occlusion of history presented to the ED with three to five hours of • While visual improvement can spontaneously occur following the central retinal artery from atherosclerotic plaques transient, painless, monocular vision loss in her right eye CRAO, improvement largely depends upon both the duration embolizing from the internal carotid artery • Since symptom onset, she had experienced several episodes and type of CRAO • Retinal ischemia can cause an acute, painless vision loss of vision loss, with intermittent return of normal vision • Additionally, recent discussion has focused on the length of • CRAO is a relatively rare condition, with an incidence of 1-2 • On physical exam retinal survival time as a crucial factor in prognosis per 100,000, 1-2% of which are bilateral cases – she had a blood pressure of 223/105 • Furthermore, much has been written about the diagnosis • Conservative management includes anterior chamber – Clonus was noted, but vision was intact in both eyes itself, however, uncertainty and disagreement exist in management and treatment paracentesis, CO and oxygen inhalation therapy, ocular – No corneal abrasions or ulcers were seen massage, topical beta-blockers, and IV acetazolamide • Non-arteritic transient CRAO (transient monocular blindness) • Intraocular pressure was measured at 20 mmHg, bedside accounts for 15–17% of CRAOs, which is what was seen in • Unfortunately, these methods appear to offer minimal to no ultrasound did not show any obvious retinal detachment; and this patient benefit in altering the natural course of the disease slit lamp exam of the anterior eye, lid, and orbit did not reveal • Intra-arterial thrombolysis (IAT) with tissue-plasminogen any abnormal findings other than cataracts in her right eye • Interestingly, while this patient did have history that would suggest atherosclerotic disease, she did not have significant activator (TPA) has shown promising outcomes but is • At that point, inflammatory markers were tested, and a CTA stenosis of her right carotid or ophthalmic arteries, when controversial due to limited randomized controlled studies Head and Neck was performed to rule out temporal arteritis viewed during cerebral angiography, which would explain her and stroke, respectively symptoms • While these tests were being run, she had another episode of • Hence, work-up was underway for hypercoagulable states vision loss, which coincided approximately three-and-a-half and cardio-embolic origins hours after her last-known well time • Of note, as of October 2020, no abnormalities were seen via • Angiography took place approximately 4 hours and 39 loop recorder or on any of her hypercoagulable labs minutes from the last known well time • No stenosis was seen at the origin of the right internal carotid artery and its intracranial course was within normal • Selective microangiography was then performed, which also showed decreased flow in the right central retinal artery, with significantly delayed choroidal blush Figure 1. Angiogram of CRAO. Before (delayed choroidal • We injected 5 mg of intra-arterial TPA over 15 minutes blush) and After tPA (restored choroidal blush.) • Following injection, normal flow was seen in the central retinal artery, with a normal choroidal blush (Figure 1) • On follow-up no visual abnormalities were seen Maggot Wound Therapy Associated with Wohlfahrtiimonas chitiniclastica Blood Infection: Case Report and Review of Literature. Peter Bueide1,2, Jeff Hunt, DO1,2, Dubert Guerrero, MD1,2 1Sanford Health; 2University of North Dakota School of Medicine and Health Sciences Introduction • He was discharged on levofloxacin. • Maggot therapy was made popular during the First World War • He underwent left facial, head and neck excision. and is still occasionally used to treat infections. • Review of previous human cases of W. chitiniclastica including this case • Wohlfahrtiimonas chitiniclastica is an aerobic, nonmotile, report is summarized in Table 1. gram-negative rod first isolated in 2008 from Wohlfahrtia magnifica maggot. Discussion • 23 previous human cases of W. chitiniclastica have been • Previous human cases of W. chitiniclastica have occurred globally. reported so far. • This is the first reported case of W. chitiniclastica bacteremia • We add this case of W. chitiniclastica bacteremia which is the associated with medical maggot therapy. first to be associated with maggot therapy. • Maggots have been demonstrated to secrete substances, defensins, Figure 1. Myiasis of the left temple by an unidentified species of that may result in the beneficial antimicrobial properties of maggot maggot (left) and appearance of the wound following debridement Case Report therapy. • 70-year old male with a PMH of stage I large B cell non-Hodgkin in the emergency room (right). lymphoma diagnosed in 2012 treated with 3 cycles of R-CHOP • Bacteria of the maggot microbiome may still be a source of infection. now in remission. Characteristics of human N=24 • Sterilization precautions are typically followed throughout the processing and utilization of maggots for wound debridement. • Noticed a slow growing ulcerating mass in the left temple for the Wohlfahrtiimonas chitiniclastica past year. infection • Wohlfahrtiimonas chitiniclastica was first isolated in Wohlfahrtia magnifica but has been identified in the gut of L. sericata, Musca • Sought alternative medicine therapies with a naturopathic Male 16 (67%) domestica, and Hermetia illucens. provider with no improvement. Age, years 64 (17-87) • L. Sericata is commonly used for medicinal purposes in maggot • Attempted maggot therapy. Risk Factors therapy. • Mechanical fall resulted in an emergency department visit at an • Chronic wounds 23 (96%) • Chronic wounds, myiasis, and underlying medical and social outside facility. • Myiasis 11 (46%) conditions impacting personal sanitation are risk factors for infection. • Noted large ulcerative and infiltrative soft tissue lesion in the left • Alcohol abuse 7 (29%) • A total of 6 out of 24 patients were deceased. temple with evidence of secondary infection and parasitic infection (Figure 1). • Homelessness 4 (17%) Conclusions • Subsequently admitted to Sanford Medical Center for further • Poor sanitary condition 4 (17%) • This is the first case of W. chitiniclastica bacteremia associated with evaluation and management and was started on empiric • Loss of consciousness 3 (13%) antibiotics. medical maggot therapy. • Obesity 2 (8%) • Tissue biopsy revealed invasive squamous cell carcinoma. • Review of literature suggested chronic wounds and myiasis as well • Medical maggot therapy 1 (4%) as medical and social conditions are risk factors for the infection. • Secondary bacterial infection of the wound: Bacteremia with W. chitiniclastica 13 (54%) • Infection associated mortality is up to 25%. – Local cultures: Staphylococcus aureus and Proteus mirabilis. – Blood cultures : Wohlfahrtiimonas chitiniclastica. Mortality 6 (25%) Acknowledgements Table 1. Demographic information and outcomes data associated We would like to thanks the staff at Sanford Health for help throughout with reported Cases of W. chitiniclasitca reported globally. this process. Tale of a Twisted PICC Line in Pandemic Times Madeline DeFrance, MS4; Siddharth Singhal, MD; Yuri Nakasato, MD

Introduction Case Report Cont. Discussion Thanks

Peripherally inserted central catheter (PICC) • Continued low grade fevers after eleven days of Tip position of a PICC line is critically important to • Fargo VA lines are commonly used for safe and antibiotics avoid complications, such as thrombosis and • UND School of Medicine and Health Sciences prolonged administration of drugs and fluids in • Transferred to hospice due to ongoing fevers and catheter failure. It is standard practice to check the • Siddharth Singhal, MD, Hospitalist Fargo VA, hospitalized patients. One complication of PICC lines COVID infection position of the tip after every placement of a PICC Assistant Professor UND SMHS is malposition, with a reported incidence of 5- line and to reposition if necessary 31%. Further complications of • Discussion regarding the best process for removal • Yuri Nakasato, MD, Rheumatologist Fargo VA of the coiled PICC line since the patient was The commonality between many case reports is malposition are patient discomfort and loss of line that malpositioned PICC lines were repositioned to usage. Due to these possible hospitalized at a smaller facility without a vascular surgeon avoid complications. We report a case where complications, malpositioned lines are usually the malpositioned PICC line was unable to be • Interventional radiology from an outside facility References repositioned or removed and replaced [1]. We repositioned due to severe patient agitation was consulted and felt that the PICC line should present a case of a malpositioned PICC line that and complications of the COVID-19 pandemic. The come out easily with no need for transfer • Song L, Li H. Malposition of peripherally inserted was unable to be repositioned and continued to be PICC line was able to be used safely to deliver central catheter: Experience from 3,012 patients used for IV antibiotics and lab draws. • General surgery removed the PICC line successfully antibiotics for an 11-day period. Additionally, the with cancer. Exp Ther Med. 2013;6(4):891-893. • No further complications related to the PICC line coiled PICC line was able to be removed safely by a doi:10.3892/etm.2013.1267 removal general surgeon without the assistance of • Cotogni P, Pittiruti M. Focus on peripherally Case Report • Patient passed away after 7 days in hospice specialty vascular care. This case suggests that as a last resort, it is possible for PICC lines to be safely inserted central catheters in critically ill used in a malpositioned state and could provide patients. World J Crit Care Med. 2014;3(4):80-94. • 94-year old male admitted to the hospital with Published 2014 Nov 4. doi:10.5492/wjccm.v3.i4.80 delirium, fever, and hypotension guidance in similar rare situations. • Danckers M, Mukherjee V, Pradhan D. • Diagnosed with a urinary tract infection (UTI) and Spontaneous coiling of a peripherally inserted found to be COVID-19 positive central venous catheter. BMJ Case Rep. • Significantly agitated and combative due to acute 2014;2014:bcr2014206644. Published 2014 Oct 28. delirium with a history of Alzheimer’s doi:10.1136/bcr-2014-206644 • Placed on IV antibiotics for UTI • Franklin I, Gilmore C. Placement of a peripherally • Patient was confused and pulled out IV on hospital inserted central catheter into the azygous vein. J day three Med Radiat Sci. 2015;62(2):160-162. doi:10.1002/jmrs.98 • PICC line was inserted for antibiotics and lab draws • No reported complications and PICC line easily drew back blood and flushed • Chest x-ray following procedure showed PICC line Disclaimer curved back on itself with the tip in the left axillary vein (Figure 1) This material is the result of work supported with • PICC line continued to function well for IV fluids resources and the use of facilities at the Fargo VA and lab draws and did not appear to be causing Health Care System. The contents do not represent any discomfort the views of the U.S. Department of Veterans Affairs or the United States Government.

Figure 1. Chest X-rays following PICC line placement showing curvature of line and line tip located in the axillary vein