AN UNUSUAL PRESENTATION OF INITIAL AND RELAPSED GRANULOMATOSIS WITH POLYANGIITIS IN AN ASIAN MALE

SESSION TITLE: Tuesday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/22/2019 01:00 PM - 02:00 PM INTRODUCTION: Granulomatosis with Polyangiitis (GPA) is a rare systemic vasculitis with a predilection for the upper airways, lungs and kidneys. However, there is a broad range of ambiguous clinical presentations which often result in a diagnostic dilemma. CASE PRESENTATION: A 47-year-old male of Pakistani origin, farmer by occupation, was admitted in the ward with a 3-week history of progressive deltoid swelling with concurrent signs and symptoms of systemic disease. Physical examination revealed a fluctuant, erythematous and tender swelling measuring 10cm x 7cm on the right deltoid. The initial chest X-ray showed two nodules in the right upper lung zone and thick-walled cavitary lesions in the left mid and lower lung zones (Figure 1). After a lack of response to treatment for an infective etiology, a diagnosis of GPA was confirmed by ANCA testing. With the administration of corticosteroids and cyclophosphamide, the deltoid abscess and cavitary lung lesions regressed completely (Figure 2). The patient remained in remission for a period of 2 years on maintenance therapy of azathioprine and prednisolone. He then developed hematuria with supra-pubic tenderness and respiratory symptoms. Urinalysis revealed sterile pyuria. Subsequently, a cystoscopic biopsy was done which revealed necrotizing granulomatous inflammation (Figure 3). With the reappearance of cavitary lung lesions and bladder vasculitis, he was treated as a relapsed GPA with rituximab and corticosteroids. DISCUSSION: Our case describes an atypical presentation of GPA presenting as a deltoid abscess, followed by another rare entity of urinary bladder involvement in relapsed disease after several years of remission on immunosuppressive therapy. GPA can potentially involve any organ or system, however, in the urogenital tract it is often limited to the kidneys or (rarely) the prostate.1 Urinary bladder involvement, as an initial or a relapsed presentation, has not been listed in multiple large scale studies.2 In our review of literature, there is only one case implicating GPA in causing isolated vasculitis of the bladder, this was a manifestation in an initial presentation.3 Interestingly, our patient developed a rapidly progressive relapse involving a new organ, despite being on a maintenance therapy. CONCLUSIONS: This case highlights the importance of clinicians to be aware of and vigilant towards the cryptic presentations of GPA. This will result in a timely diagnosis and treatment, and eventually a better prognosis. We conclude that an active vasculitis should be included among the differentials along with neoplasia and infection for a similar clinical presentation. Reference #1: Davenport A, Downey SE, Goel S, MacIvert AG. Wegener’s granulomatosis involving the urogenital tract. Br J Urol. 1996;78(3):354-357 Reference #2: Reinhold-Keller E, Beuge N, Latza U, et al. An interdisciplinary approach to the care of patients with Wegener’s granulomatosis: Long-term outcome in 155 patients. Arthritis Rheum. 2000; 43(5):1021–1032 Reference #3: Kassir R, Mouracade P, Barabino G, Peoc’H M, Cuilleron M, Gigante M. Vasculitis of the bladder: An extremely rare case report. Int J Surg Case Rep. 2013;4(9):782-784 DISCLOSURES: No relevant relationships by Maaha Ayub, source=Web Response No relevant relationships by Usman Bin Hameed, source=Web Response No relevant relationships by Ali Zubairi, source=Web Response No relevant relationships by Mustafa Zubairi, source=Web Response