GRANULOMATOSIS WITH POLYANGIITIS PRESENTING AS BILATERAL DIFFUSE ALVEOLAR HEMORRHAGE

Abstract

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) most commonly occurs in older adults with multi-organ involvement including skin, kidneys and respiratory tract. Some of the pulmonary complications include dyspnea, small airway obstruction, pulmonary infiltrates, cavitary lesions and rarely, pulmonary hemorrhage. We present a case diffuse alveolar hemorrhage (DAH) as the initial presentation of GPA. CASE PRESENTATION: 57-year-old African American female with history of hypertension, diabetes mellitus, ductal carcinoma in situ, ischemic stroke, tobacco abuse and obesity presented with cough, progressive dyspnea and mild hemoptysis. On pulmonary examination, she had labored breathing and expiratory wheezes. Initial laboratory examinations were all within normal limits. A chest radiograph showed diffuse bilateral infiltrates. A high-resolution computed tomography of her chest revealed diffuse bilateral alveolar infiltrates with sparing of the lung peripheries and bases with confluent ground glass nodularity. She was empirically started on intravenous methylprednisolone and oral levofloxacin. Due to worsening respiratory symptoms and increased oxygen requirements, she was intubated. Bronchoscopy revealed bilateral alveolar hemorrhage across four dilutions. Bronchoalveolar lavage was negative for bacterial growth. Video-assisted thoracoscopic surgery (VATS) wedge biopsy showed diffuse neutrophilic inflammation, without evidence of granuloma or vasculitis. Plasmapheresis was initiated. She was successfully extubated after three sessions of plasmapheresis. Rheumatological workup returned positive for PR3 antibody. She was sarted on Rituximab for presumed GPA. She was discharged to a nursing facility. On a subsequent admission 10 days later, she was diagnosed with rapidly progressive glomerulonephritis, confirmed on renal biopsy. A skin lesion biopsy showed retiform purpura with ulcerations, consistent with medium and small vessel vasculitis. She was transitioned from rituximab to induction with cyclophosphamide and plasmapheresis was continued until she was stable and discharged back to nursing facility. DISCUSSION: This is an unfortunate but interesting initial presentation of GPA with mild hemoptysis and dyspnea resulting in alveolar hemorrhage, a rare complication of GPA. In addition, her renal involvement contributes to a worse prognosis with an estimated 10-year survival rate of 40%. CONCLUSIONS: GPA is a necrotizing systemic vasculitis that involves multiple organs, most commonly affecting skin, kidneys and lungs. However, these organs can have an array of symptoms that pose a difficulty in recognizing the disease, therefore, requiring a high clinical suspicion. DAH is a rare and life-threatening complication of GPA. Some clues to further explore DAH would include hemoptysis, dyspnea, anemia and hypoxia. Early diagnosis and initiation of treatment is important to preventing morbidity and mortality. Reference #1: Greco A, Marinelli C, Fusconi M, et al. Clinic manifestations in granulomatosis with polyangiitis. Int J Immunopathol Pharmacol. 2015;29(2):151-9. Reference #2: West S, Arulkumaran N, Ind PW, Pusey CD: Diffuse alveolar haemorrhage in ANCAassociated vasculitis. Intern Med 52: 5–13, 2013 DISCLOSURES: No relevant relationships by Kylee Dawson, source=Web Response No relevant relationships by Navneet Kaur, source=Web Response No relevant relationships by Sowjanya Yenigalla, source=Web Response