Microscopic Polyangiitis Presenting with Splenic Infarction: a Case Report

medicina

Case Report Microscopic Polyangiitis Presenting with Splenic Infarction: A Case Report

Sang Wan Chung

Division of Rheumatology, Department of Internal Medicine, School of Medicine, Kyung Hee University, 26 Kyungheedae-ro, Dongdaemun-gu, Seoul 02447, Korea; [email protected]; Tel.: +82-2-958-8200

Abstract: Microscopic polyangiitis (MPA) is an anti-neutrophil cytoplasmic antibody (ANCA)- associated vasculitis (AAV). The splenic involvement in AAV is known to be rare, and that in MPA has not been reported to date. A 74-year-old woman was admitted owing to left arm numbness and weakness. The patient was diagnosed as MPA with vasculitic neuropathy. Her abdominal computed tomography (CT) revealed splenic infarction incidentally. The splenic infarction had been resolved at follow-up CT after treatment. If splenic involvement of MPA was not considered, treatment may have been delayed in order to differentiate other diseases. Herein, I report the ﬁrst case of splenic involvement of MPA.

Keywords: ANCA-associated vasculitis; splenic infarction; MPA

1. Introduction Microscopic polyangiitis (MPA) is a type of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) that predominantly affects small-sized vessels. The most commonly affected organs are the kidney and lung. Necrotizing and crescentic Citation: Chung, S.W. Microscopic glomerulonephritis and diffuse alveolar hemorrhage are the main clinical presentations. Polyangiitis Presenting with Splenic General signs such as fever, weight loss, peripheral neuropathy, cutaneous manifestations, Infarction: A Case Report. Medicina and upper respiratory tract involvement may be present [1]. In AAV numerous atypical 2021, 57, 157. https://doi.org/ manifestations may also occur, among which, though rarely reported, is splenic involve- 10.3390/medicina57020157 ment, particularly in patients with granulomatous polyangiitis (GPA) [2]. In MPA, splenic involvement has been reported very rarely. Herein, I report a case of splenic involvement Academic Editor: Emanuel of MPA. Della-Torre Received: 14 December 2020 2. Case Presentation Accepted: 5 February 2021 Published: 9 February 2021 A 74-year-old female patient presented to the emergency department of Kyung Hee University Medical Center because of dizziness and generalized weakness that started

Publisher’s Note: MDPI stays neutral 2 days prior. The patient had a history of old cerebral infarction and was taking oral medica- with regard to jurisdictional claims in tions for hypertension and dyslipidemia. Brain magnetic resonance imaging (MRI) revealed published maps and institutional affil- an acute focal infarction in the right basal ganglia. At that time, in the laboratory examina- iations. tions, the erythrocyte sedimentation rate (ESR) was 74 mm/h (normal range, <20 mm/h) and C-reactive protein (CRP) level was 6.61 mg/dL (<0.5 mg/dL) Aspirin was prescribed to the patient. One month later, the patient was admitted to our neurology clinic because of weakness in her left arm and numbness in both hands. MRI revealed no diffusion-restricted lesion in the brain. Physical examination revealed no remarkable findings except for pares- Copyright: © 2021 by the author. Licensee MDPI, Basel, Switzerland. thesia in the left hand. The patient’s motor power of four limbs were normal (grade 5). This article is an open access article Her initial chest radiograph was normal. Her laboratory examination results showed a distributed under the terms and white blood cell count of 9.79 × 109/L (76% neutrophils), ESR of 120 mm/h, and CRP conditions of the Creative Commons level of 18.44 mg/dL. However, she had no fever. Her serum creatinine level (0.92 mg/dL) 2 Attribution (CC BY) license (https:// and estimated glomerular filtration rate (GFR; 63.59 mL/min per 1.73 m ) were within creativecommons.org/licenses/by/ the normal ranges. No proteinuria was identified. Chest computed tomography (CT) 4.0/). and abdominal CT were performed to exclude hidden malignancy. Chest CT revealed a

Medicina 2021, 57, 157. https://doi.org/10.3390/medicina57020157 https://www.mdpi.com/journal/medicina Medicina2021, 57, x FOR PEER REVIEW 2 of 4

1.73 m2) were within the normal ranges. No proteinuria was identified. Chest computed Medicina 2021, 57, 157 tomography (CT) and abdominal CT were performed to exclude hidden malignancy. 2 of 4 Chest CT revealed a subpleural nodule measuring approximately 1.5 cm in the right lower lobe. Abdominal CT revealed multiple small ill-defined low-attenuated lesions in the spleensubpleural nodules (Figure nodule 1). measuring Her thrombophilia approximately screening 1.5 cm test in the result right was lower negative. lobe. Abdominal The CT Padua predictionrevealed score multiple was small 2, in ill-defined the low risk.Her low-attenuated echocardiography lesions in the was spleen normal nodules and (Figure1). 24-hour HolterHer thrombophilia monitoring revealed screening normal test result sinus was rhythm. negative. Nerve The Padua conduction prediction tests score re- was 2, in vealed sensorimotorthe low risk.Her polyneuropathy. echocardiography The myeloperoxidase was normal and 24-hour(MPO)-ANCA Holter monitoringtest result revealed was positivenormal (5.10 sinus index). rhythm. Percutaneous Nerve conduction needle biopsy tests revealed of the sensorimotorpulmonary nodule polyneuropathy. was The performedmyeloperoxidase first; however, the (MPO)-ANCA pathological testresults result showed was positive only focal (5.10 acute index). inflammatory Percutaneous needle cell infiltration,biopsy with of the no pulmonary evidence of nodule malignancy. was performed The second first; biopsy however, of the the sural pathological nerve results demonstratedshowed perivascular only focal inflammatory acute inflammatory cell infiltration cell infiltration, and withnecrotizing no evidence vasculitis. of malignancy. These findingsThe second were biopsyconsistent of the with sural vascul nerveitic demonstrated peripheral neuropathy. perivascular inflammatoryBy considering cell infiltra- the resultstion of the and MPO-ANCA necrotizing vasculitis. test and the These pathological findings were profile consistent of the sural with vasculiticnerve, the peripheral patient wasneuropathy. diagnosed By as considering having MPA-associat the results ofed theneuropathy MPO-ANCA with test a splenic and the infarction pathological profile and pulmonaryof the suralinflammatory nerve, the nodule. patient wasShe diagnosedreceived steroid as having pulse MPA-associated therapy (methylpred- neuropathy with a nisolone, 125splenic mg/day infarction for 3 and days) pulmonary on day 20 inflammatory of hospitalization, nodule. followed She received by steroidcyclophos- pulse therapy phamide (500(methylprednisolone, mg/m2every 4 weeks 125 for mg/day six doses) for 3with days) steroid on day maintenance 20 of hospitalization, therapy. She followed by showed improvementcyclophosphamide after the (500 steroid mg/m therap2 everyy. Her 4 weeks ESR level for sixdecreased doses) withto 49 steroidmm/h and maintenance the CRP leveltherapy. decreased She showed to 0.5 mg/dL. improvement The peripheral after the numbness steroid therapy. subsided Her slowly. ESR levelAfter decreased 1 month, theto 49patient’s mm/h pancreatic and the CRP enzyme level levels decreased (amylase, to 0.5 192 mg/dL. U/L (23–85 The peripheral U/L) and lipase numbness sub- 346 U/L (0–160sided slowly.U/L)) were After elevated 1 month, without the patient’s abdominal pancreatic pain. Abdominal enzyme levels CT (amylase,was per- 192 U/L formed to(23–85 evaluate U/L) for andpancreatitis lipase 346 and U/L reve (0–160aled improvement U/L)) were of elevated the splenic without lesion abdominal such pain. as the infarction.Abdominal The CT patient was performed had an uneventful to evaluate course for pancreatitis for 5 months and revealedafter the improvementtreat- of ment.Thisthe study splenic was lesion approved such asby the the infarction. Institutional The Review patient hadBoard an uneventful(IRB) of Kyung course Hee for 5 months Medical Center,after the treatment.Thisand the requirement study was for approved informed by theconsent Institutional was waived Review Board(IRB (IRB) of 2020-12-083).Kyung Hee Medical Center, and the requirement for informed consent was waived (IRB 2020-12-083).

(A)

Figure 1. Cont.

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(B) Figure 1. AbdominalFigure 1. Abdominal computed computedtomography tomography (CT) image. (CT) (A) image.Axial CT (A image) Axial on CT day image 1, illustrating on day 1, illustrating wedge-shapedwedge-shaped splenic infarcts splenic (white infarcts arrow). (white (B) Axial arrow). CT image (B) Axial 2 months CT image later, 2showing months an later, im- showing an proved splenicimproved infarction. splenic infarction.

3. Discussion3. Discussion MPA is oneMPA of isthe one main of thesubgroups main subgroups of AAV. AAV of AAV. is a AAV group is of a groupsystemic of systemicvasculitis vasculitis characterizedcharacterized by inflammation by inflammation of small- of to small- medium-sized to medium-sized vessels. vessels. AAV can AAV be can divided be divided into into threethree entities, entities, namely namely MPA, MPA, GPA, GPA, and and eosinophilic eosinophilic GPA, GPA, according according to to histological histological findings findings andand clinical manifestationsmanifestations [3[3,4].,4]. Its Its histology histology is is characterized characterized by by necrotizing necrotizing small vessel small vesselvasculitis vasculitis with with little little or no or immuneno immune deposits deposits [5]. In[5]. MPA, In MPA, renal renal and pulmonaryand pulmo- manifesta- nary manifestationstions are frequent, are frequent, but various but various other other symptoms symptoms also occur, also occur, such as,such fever, as, fever, arthralgia, and arthralgia,peripheral and peripheral numbness numbness [1]. ANCA [1]. ANCA appears appears to be closely to be relatedclosely torelated AAV, andto AAV, the anti-MPO and the anti-MPOspecificity specificity of ANCAs of is ANCAs observed is inobserved almost 80%in almost of patients 80% of with patients MPA with [6]. Life-threatening MPA [6]. Life-threateningmanifestations manifestations are treated are with tr glucocorticoidseated with glucocorticoids and cyclophosphamide. and cyclophospha- Rituximab has mide. Rituximabbeen shown has tobeen be effectiveshown to as be a remission-inducingeffective as a remission-inducing therapy. Azathioprine, therapy. methotrexate Aza- or thioprine,mycophenolate methotrexate or mofetil mycophenolat may be recommendede mofetil may for be maintenance recommended therapy for [mainte-6]. nance therapy Splenic[6]. involvement is rarely reported in AAV and tends to be undervalued. This Splenicis because involvement most is patients rarely reported are asymptomatic. in AAV and Gercik tends etto al.be undervalued. investigated the This frequency is of splenic lesions in patients with AAV. In their study, splenic pathologies were observed in because most patients are asymptomatic. Gercik et al. investigated the frequency of 19 patients (28%), of whom 7 (37%) had splenic infarction. All the patients with splenic splenic lesions in patients with AAV. In their study, splenic pathologies were observed infarction were diagnosed as having GPA [2]. No splenic infarction by MPA has been in 19 patients (28%), of whom 7 (37%) had splenic infarction. All the patients with splen- reported until recently. ic infarction were diagnosed as having GPA [2]. No splenic infarction by MPA has been I report a case of MPA with an asymptomatic splenic involvement. High-dose steroid reported until recently. and cyclophosphamide therapies successfully controlled the splenic infarction. Moreover, I report a case of MPA with an asymptomatic splenic involvement. High-dose ster- the patient had no symptoms, so infection and other causes could be ruled out. However, oid and cyclophosphamide therapies successfully controlled the splenic infarction. the lack of awareness of the possibility of splenic involvement of MPA could delay the Moreover, the patient had no symptoms, so infection and other causes could be ruled diagnosis of vasculitis until after infection or malignancy has been ruled out. out. However, the lack of awareness of the possibility of splenic involvement of MPA could delay4. Conclusionsthe diagnosis of vasculitis until after infection or malignancy has been ruled out. Here, I describe the case of MPA presenting with splenic infarction. If practitioners overlook the splenic involvement of AAV, the diagnosis and treatment of vasculitis may be delayed until infection or other causes have been identified. Therefore, this should be kept

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in mind when evaluating patients with the possibility of splenic involvement in AAV, and even MPA.

Funding: This research received no external funding. Institutional Review Board Statement: The study was conducted according to the guidelines of the Declaration of Helsinki, and approved by the Institutional Review Board of Kyung Hee Medical Center (IRB 2020-12-083). Informed Consent Statement: The requirement for informed consent was waived due to the nature of retrospective case study. Conﬂicts of Interest: The author declares no conﬂict of interest.

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