Abstract Granulomatosis with polyangitis (GPA) is an autoimmune disease characterized by necrotizing granulomatous inflammation and vasculitis of small and medium sized vessels, most commonly affecting the upper and lower respiratory tract and kidneys, but virtually any organ can be affected. Diffuse alveolar hemorrhage (DAH) is a well recognized and potentially life threatening complication of GPA. We describe a case of GPA, initially disguised as chronic sinopulmonary infection, ultimately causing catastrophic DAH despite being on immunosuppression. A 48 year old male with history of chronic sinusitis with nasal crusting, occasional epistaxis, pulmonary infections, psoriasis and ulcerative colitis presented with massive hemoptysis and respiratory failure leading to cardiac arrest. Five years earlier, he experienced worsening exertional dyspnea which led to CT chest showing bronchiectasis. Cultures from bronchoscopy grew mycobacterium conspicuum for which he competed 1 year treatment with rifampin, clarithromycin and ethambutol. Further evaluation revealed positive c-ANCA and immunosuppressive therapy was initiated; with prednisone and then methotrexate. He presented with worsening hemoptysis, leading to respiratory failure and cardiac arrest. Initial labs were remarkable for WBC 22,000, hemoglobin 11g/dL (baseline 12-13), lactic acidosis and acute kidney injury. Imaging demonstrated pan sinusitis and multifocal consolidations. Initial bronchoscopy with BAL (bronchoalveolar lavage) and serial aliquots were negative for DAH. He was however started on high dose IV methylprednisone. He had refractory hypoxia and hypercapnia while on the ventilator, necessitating repeat bronchoscopy for further evaluation and clot removal. VV ECMO (Veno Veno Extracorporeal Membrane Oxygenation) was initiated to stabilize him as a bridge for ongoing assessment, given his young age and no significant comorbidities, other than GPA precluding VV ECMO. A subsequent bronchoscopy revealed diffuse bleeding without a focal source, consistent with DAH. Unfortunately, it was discovered he had sustained severe anoxic brain injury, and due to the poor prognosis, ECMO was stopped and family transitioned him to comfort care. This case highlights progression of GPA, currently on treatment with immunosuppression with methotrexate and prednisone, progressing to DAH with concurrent bacterial infection. Chronic sinopulmonary disease can obscure the progression of vasculitis. Despite being the primary diagnostic test used, early bronchoscopy with serial bronchoalveolar lavage may not reveal evolving hemorrhage. ECMO can serve as a bridge for stabilization. In patients with chronic sinus, sinopulmonary disease and bronchiectasis, we should have a high index of suspicion for GPA as early recognition and immunosuppression may prevent fatal pulmonary hemorrhage. We should remain vigilant for flares even in patients on treatment. This abstract is funded by: None
Onumajuru et al. (Fri,) studied this question.