PERIPHERAL EOSINOPHILIA IN GRANULOMATOSIS WITH POLYANGIITIS: A RARE CASE REPORT AND REVIEW OF LITERATURE

Abstract

SESSION TITLE: Pulmonary Manifestations of Systemic Disease 2 SESSION TYPE: Med Student/Res Case Report PRESENTED ON: 10/09/2018 05:00 PM - 06:00 PM INTRODUCTION: Granulomatosis with polyangiitis (GPA, formerly Wegener's granulomatosis) and eosinophilic granulomatosis with polyangiitis (EGPA, formerly Churg-Strauss syndrome) are both ANCA- associated vasculitis of the small and medium sized vessels with involvement of the upper and lower respiratory tracts as well as the kidneys. GPA is typically characterized by sinusitis, alveolar hemorrhage, arthritis, and high titers of proteinase-3 antibodies (PR3-ANCA), whereas EGPA prominently showcases a history of atopy, asthma, and myeloperoxidase antibodies (MPO-ANCA) 1. EGPA typically features significant peripheral eosinophilia; however eosinophilia can also rarely be a feature of GPA. CASE PRESENTATION: 21-year-old Southeastern Asian male with PMH of latent tuberculosis status post treatment with Isoniazid, who presented with one week of hemoptysis and symmetrical polyarthralgias in bilateral knees and elbows. Patient denied a history of asthma, atopic disease, or sinusitis. On physical exam, patient had a temperature of 102.5 F, an effusion of the right elbow, petechial lesions on the extensor surfaces of both elbows and anterior aspects of interphalangeal joints, and vesicular breath sounds. Laboratory testing significant for leukocytosis, eosinophilia, elevated inflammatory markers and positive autoimmune antibodies, IgE of 773.3 IU/mL (normal range 0-100), sedimentation rate of 80 mm/h (normal range 0-15), RF of 46 IU/mL (normal <14) and positive PR3-ANCA (>150 units). Testing was negative for MPO-ANCA, strongyloides antibody, H. brazilienses antibody, stool Giardia antigen, stool ova and parasites. BMP revealed normal serum creatinine, and UA showed 1+ protein, 2+ blood. CXR showed bibasilar infiltrates. Chest CT showed patchy regions of ground glass opacities bilaterally (Fig 1). Renal biopsy showed crescentic and necrotizing glomerulonephritis of the pauci-immune subtype consistent with GPA (Fig 2-4). Patient received pulse steroids, then he was switched to Rituximab and oral prednisone. At 3 month follow up after discharge, his eosinophilia resolved and PR3-ANCA levels dropped to 4 mm/h. DISCUSSION: Eosinophils usually comprise 1 to 3% peripheral WBCs. Eosinophilia is found in many conditions, including atopic disorders, neoplasms, helminthic infections, various hematologic and autoimmune rheumatic disorders as well as secondary effects of medications 2. Peripheral eosinophilia is rare in GPA with only five cases of GPA with peripheral eosinophilia reported 3-6. When peripheral eosinophilia is present, alternative diagnoses should be considered as was done in our case. Treatment options are based on severity of the presentation. Severe disease are often treated with glucocorticoid plus cyclophosphamide or rituximab. Plasmapheresis is indicated in patients with rapidly declining kidney function, positive anti-GBM, or pulmonary hemorrhage CONCLUSIONS: GPA is a differential diagnosis of eosinophilia Reference #1: Masi AT, Hunder GG, Lie JT, et al. The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome. Arthritis Rheum 33: 1094-1107, 1990 Reference #2: Rothenberg ME. Eosinophilia. N Eng J Med. 1998; 338:1592-1600 Reference #3: Potter MB, Fincher RK, Finger DR. Eosinophilia in Wegener's Granulomatosis. Chest 116: 1480-1483, 1999 DISCLOSURES: No relevant relationships by Melody Chin, source=Web Response No relevant relationships by Elie Jean, source=Web Response No relevant relationships by Roshan Patel, source=Web Response