A Case of ANCA-Associated Vasculitis With Devastating Complications

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INTRODUCTION: Granulomatosis with polyangiitis (GPA) is an inflammatory process which can affect the small vessels of any organ or tissue. Typical organs affected include the respiratory tract and kidneys, and less commonly: skin, brain, eyes, heart, liver, and lower GU tract. [1] In addition, inflammatory changes of GPA can sometimes mimic tumors or masses, causing delay in diagnosis and therapy. CASE PRESENTATION: A 60-year-old female with no significant medical history presented to the hospital with fatigue for 1 week. Initial lab was significant for acute renal failure with urinalysis showing microscopic hematuria. Day 1 chest x-ray was unremarkable (Image 1). Additional imaging demonstrated a 21 cm heterogenous pelvic mass causing mass effect and right hydronephrosis (Image 2). Despite ureteral stent placement, patient's renal failure progressed requiring hemodialysis. Hospital course was further complicated by hemoptysis a week after admission, severe hypoxemic respiratory failure requiring mechanical ventilation. Repeat chest x-ray showed extensive bilateral airspace opacities. CT chest demonstrates diffuse ground-glass opacities and superimposed dense bilateral patchy consolidations (Image 3). There was concern for pulmonary-renal syndrome and bronchoscopy was performed; BAL findings were consistent with DAH and pulmonary capillaritis. Patient had an unrevealing infectious and cardiac workup. She was subsequently started on pulse-dose steroids, followed by plasma exchange. Autoimmune workup showed elevated PR-3 antibody and c-ANCA titer, suggestive of GPA. Kidney biopsy confirmed pauci-immune ANCAassociated crescentic glomerulonephritis. After a multidisciplinary discussion, the decision was made to start Rituximab due to persistent hemoptysis, severe hypoxemia, and the need for renal replacement therapy. Patient's hypoxemia improved, however she was unable to be liberated from mechanical ventilation due to Posterior Reversible Encephalopathy Syndrome. She underwent tracheostomy 3 weeks after intubation and hospital course was further complicated by recurrent multiorgan infections including acute cholangitis s/p ERCP and invasive candidiasis. She suffered from septic shock and a brief cardiopulmonary arrest. The patient's family ultimately elected to pursue comfort measures. DISCUSSION: Patient in this case initially presented with obstructive uropathy and acute kidney injury secondary to a large pelvic mass. Differentials for pelvic mass with fatty component include malignancy, teratoma and vasculitic mass. Imaging had reassuring features that suggest against malignancy with negative CA-125 and CA 19-9. CONCLUSIONS: This case presented a diagnostic dilemma as there has been rare cases of GPA vasculitis whose first manifestation was obstructive uropathy causing kidney injury. Due to initial presumption of pelvic mass being malignant in nature, autoimmune work up was not obtained until patient had developed hemoptysis, which demonstrates elevated c-ANCA and PR-3 Antibody and eventual renal biopsy confirmed GPA. There has been a reported case of biopsy proven vasculitis involving a large ovarian mass with compressive uropathy as an initial presentation of GPA. [2] In retrospect, perhaps clinical course could have been changed with early diagnosis of GPA in the setting of a large pelvis mass and initiation of therapy to avoid the devastating complications mentioned above.




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Chest Annual Meeting 2023, October 8-11, 2023, Honolulu, HI