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 hydronephrosis 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 ureter producing severe obstructive uropathy of the right kidney 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 urology 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.