Sophie Trujillo, DO
Resident Physician
Department of Medicine, Case Western Reserve University - MetroHealth, Ohio, United States
Disclosure information not submitted.
Marina Duran, MD
Physician
Department of Emergency Medicine, Case Western Reserve University - MetroHealth, Ohio, United States
Disclosure information not submitted.
Ziad Shaman, MD
Physician
Department of Pulmonary, Critical Care and Sleep Medicine, Case Western Reserve University - MetroHealth, Ohio, United States
Disclosure information not submitted.
Title: Diffuse alveolar hemorrhage in debut granulomatosis with polyangiitis after presumptive COVID-19
Case Report Body
Introduction: Granulomatosis with polyangiitis (GPA) is a rare anti-neutrophil cytoplasmic autoantibody (ANCA) (primarily anti-protease 3 [PR3])-associated vasculitis, usually affecting small to medium vessels in the upper and lower respiratory tract and kidneys. COVID-19 can mimic the symptoms of GPA and the ongoing pandemic renders clinicians susceptible to a variety of cognitive biases.
Description: A 72-year-old male patient with a history of recently diagnosed ILD with a 2-liter home oxygen requirement was admitted to ICU for hypoxic respiratory failure after a week of worsening dyspnea. He was diagnosed with ILD after a presumptive COVID-19 admission 3 months prior, during which he had multiple negative antibody and nucleic acid amplification tests (NAAT) though a reactive antibody test after receiving convalescent plasma. Of note, a CT obtained at the end of his admission showed honeycombing, and he was found to have two positive PR3 levels (34 and 47 AU/mL). On arrival, the patient was ventilated noninvasively with bilevel pressure, but required intubation due to worsening hypercapnia. His initial work-up was notable for acute kidney injury (AKI), elevated C-reactive protein and erythrocyte sedimentation rate, negative blood and respiratory cultures, negative COVID-19 NAAT and bilateral airspace opacities on chest X-ray. Due to severe respiratory acidosis the patient underwent interval proning, and his course was further complicated by worsening AKI and an acute hemoglobin drop. He underwent bronchoscopy with lavage which showed alveolar hemorrhage. Additional work-up was notable for a positive ANCA titer of 1:320 and PR3 level of 367 AU/mL. The patient was started on a course of high strength glucocorticoids for possible autoimmune pneumonitis with some improvement in his respiratory status. A kidney biopsy confirmed the diagnosis of GPA and the patient was started on rituximab.
Discussion: There is a growing body of literature describing both COVID-associated ILD and post-COVID autoimmune disease. In this case, the diagnosis of COVID-19 confounded the diagnosis of GPA. This case highlights a number of cognitive biases including anchoring, availability, and confirmation. Not all COVID is COVID and a broad differential diagnosis must prevail.