PULMONARY-RENAL SYNDROME: URINE TROUBLE WHEN YOU DEVELOP ALVEOLAR HEMORRHAGE

Abstract

SESSION TITLE: Wednesday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/23/2019 09:45 AM - 10:45 AM INTRODUCTION: Acute ANCA vasculitis with in-hospital development of alveolar hemorrhage results in significant mortality of up to 24%. Generally, diffuse alveolar hemorrhage has worsening prognostic outcomes in patients age > 60 years, particularly when associated with GFR < 60, tobacco abuse, cardiovascular disease, shock, and mechanical ventilation. Furthermore, ANCA-associated vasculitis has a 90% 2-year mortality if left untreated, which significantly decreases to 74% 5-year mortality in patients receiving prompt corticosteroid therapy. It is important to promptly recognize and treat late onset ANCA vasculitis, especially in patients requiring critical care admission. CASE PRESENTATION: We present an 80-year-old woman with medical history of hypothyroidism, diabetes mellitus type II, and hypertension. She was initially admitted for treatment of Clostridium difficile colitis. Despite clinical improvement of diarrheal illness after treatment with oral vancomycin, she was noted to have worsening acute kidney injury on hospital day 2. On hospital day 4, she had an acute drop in hemoglobin. On day 7, the patient's respiratory status began to decompensate with increasing hypoxia and work of breathing. She was transitioned to an intermediate level of care for high flow nasal cannula, and ultimately was placed on non-invasive positive pressure ventilation for refractory hypoxemia. Computed tomography (CT) imaging was notable for bilateral infiltrates and the patient was treated with broad spectrum antibiotics with concern for pneumonia, though she remained afebrile. She never experienced hemoptysis. On hospital day 10, the patient further declined requiring urgent intubation. Upon intubation, significant bloody secretions were noted. This was further evaluated with bronchoscopy with serial BAL aliquots, which proved to be progressively bloody, consistent with diffuse alveolar hemorrhage. Treatment with pulse dose steroids and plasma exchange was initiated. Serologic workup was consistent with granulomatosis with polyangiitis (GPA) as demonstrated by positive c-ANCA titer 1:640. The patient was started on rituximab, successfully extubated and discharged from intensive care in stable condition on hospital day 22. DISCUSSION: This case is especially interesting considering the new diagnosis of GPA in the inpatient setting following an admission for C. difficile colitis. She had no hemoptysis, no bloody diarrhea, prior diagnosis of CKD, nor other manifestations concerning for GPA. Her presentation was acute and atypical, and her mortality was ameliorated due to urgent intubation and immediate treatment. CONCLUSIONS: By initiating prompt steroid therapy and plasma exchange, this 80-year-old woman with significantly impaired GFR, shock requiring multiple vasopressors, and invasive ventilatory support, defied the expected outcome and significant mortality associated with late-onset granulomatosis with polyangiitis. Reference #1: de Prost N, Parrot A, Picard C, Ancel PY, Mayaud C, Fartoukh M, et al. Diffuse alveolar haemorrhage: Factors associated with in-hospital and long-term mortality. Eur Respir J. 2010;35:1303–11 Reference #2: Biscetti F, Carbonella A, Parisi F, et al. The prognostic significance of the Birmingham Vasculitis Activity Score (BVAS) with systemic vasculitis patients transferred to the intensive care unit (ICU). Medicine (Baltimore). 2016;95(48):e5506 DISCLOSURES: No relevant relationships by Meagan Marshburn, source=Web Response No relevant relationships by Sarah Ellen Stephens, source=Web Response No relevant relationships by Amanda Westbrook, source=Web Response