AN UNUSUAL CAUSE OF RECURRENT PNEUMONIAS

Abstract

TOPIC: Pulmonary Manifestations of Systemic Disease TYPE: Medical Student/Resident Case Reports INTRODUCTION: Granulomatosis with polyangiitis (GPA) is a necrotizing small vessel vasculitis classically involving the upper and lower respiratory tract and kidneys. We present a patient with GPA which initially presented as "recurrent pneumonias" unresponsive to antibiotics ultimately culminating in a presentation with alveolar hemorrhage and respiratory failure. CASE PRESENTATION: A 70-year-old man presented to the Emergency Department with dyspnea and hemoptysis. He had been hospitalized several times over the preceding month for recurrent pneumonia, and recently had been started on systemic anticoagulation for a pulmonary embolism. On arrival he was afebrile, tachycardic, hypoxemic and tachypneic. Exam revealed coarse bilateral breath sounds. Laboratory studies were significant for anemia, leukocytosis, acutely elevated creatinine and microscopic hematuria. CT Chest showed diffuse consolidation and ground glass opacities markedly worse than 1 week prior. He was initiated on broad spectrum antibiotics and admitted to the ICU. The patient was intubated for progressive respiratory failure and hemoptysis; bronchoalveolar lavage demonstrated a progressively bloody return. C-ANCA and PR3 antibodies were positive; broad infectious evaluation and other autoimmune serologies were negative. A presumptive diagnosis of ANCA associated vasculitis was made and treatment with methylprednisolone 1,000 mg daily was initiated then transitioned to prednisone 1 mg/kg daily after 3 days. Renal biopsy confirmed GPA; rituximab was added with rapid improvement. DISCUSSION: GPA is a systemic vasculitis with variable pulmonary manifestations, ranging from asymptomatic disease to respiratory failure. Specific manifestations include tracheobronchial stenosis, inflammatory lesions, interstitial lung disease, and diffuse alveolar hemorrhage (DAH). Image findings include pulmonary nodules with or without cavitation, ground glass and consolidation. Delayed diagnosis is common, necessitating a high index of suspicion. Involvement and disruption of the alveolar capillaries leads to alveolar hemorrhage, and early bronchoscopy can facilitate identification of DAH, exclusion of other potential etiologies, and lead to early diagnosis and treatment. Induction therapy for severe GPA, defined as life or organ threatening disease, includes high dose intravenous corticosteroids in combination with cyclophosphamide or rituximab. While DAH may be a life-threatening complication of GPA, most patients achieve remission and survive to hospital discharge. CONCLUSIONS: Severe presentations of GPA include diffuse alveolar hemorrhage and respiratory failure. Image findings may be non-specific, and early bronchoscopy in the setting of persistent symptoms and pulmonary findings can accelerate identification and initiation of life saving treatment. REFERENCE #1: Chung MP, Yi CA, Lee HY, Han J, Lee KS. Imaging of Pulmonary Vasculitis. Radiology. 2010;255(2):322-341. doi:10.1148/radiol.10090105 REFERENCE #2: Polychronopoulos VS, Prakash UBS, Golbin JM, Edell ES, Specks U. Airway Involvement in Wegener's Granulomatosis. Rheumatic Disease Clinics of North America. 2007;33(4):755-775. doi:10.1016/j.rdc.2007.09.004 REFERENCE #3: Cartin-Ceba R, Diaz-Caballero L, Al-Qadi MO, et al. Diffuse Alveolar Hemorrhage Secondary to Antineutrophil Cytoplasmic Antibody-Associated Vasculitis: Predictors of Respiratory Failure and Clinical Outcomes. Arthritis & Rheumatology. 2016;68(6):1467-1476. doi:10.1002/art.39562 DISCLOSURES: No relevant relationships by Scott Canepa, source=Web Response No relevant relationships by Bryan Kelly, source=Web Response No relevant relationships by Alexander Niven, source=Web Response