Granulomatosis With Polyangiitis in a Patient With Incidental COVID Infection

Document Type

Conference Proceeding

Publication Date

10-2023

Publication Title

Chest

Abstract

INTRODUCTION: Granulomatosis with polyangiitis (GPA) is a systemic disorder that involves necrotizing granulomatous vasculitis of small arteries and veins. It is associated with antineutrophilic cytoplasmic autoantibodies (ANCA). Lower respiratory tract and kidneys are usually affected. Patients commonly present with dyspnea, cough and hemoptysis. CASE PRESENTATION: A 60-year-old female presented to the Emergency Room with worsening dyspnea, fatigue, and anemia. Initial testing revealed infection with COVID-19. Complete blood count revealed anemia with hemoglobin of 7, mild leukocytosis, and thrombocytosis. Urine analysis showed hematuria and proteinuria. The metabolic panel was significant for renal insufficiency with a creatinine of 3.59 and BUN of 40. The initial Chest X-Ray at the time of presentation was unremarkable (see image 1). Treatment with Remdesivir and Decadron was initiated for presumed COVID pneumonia. However, her symptoms worsened, and she developed hypoxic respiratory failure, prompting admission to the Medical Intensive Care Unit where she was intubated and placed on mechanical ventilation. Examination of the endotracheal tube revealed persistent blood. Her hemoglobin dropped to 6.7, requiring multiple blood transfusions. Intermittent hemodialysis was started on account of declining renal function. Repeat Chest X-Ray showed bilateral interstitial and airspace opacities with consolidations, worse in the left lung (see image 2). Serum testing was positive for PR3-ANCA (anti-proteinase 3 ANCA), confirming the diagnosis of Graulomatosis with polyangiitis. Our patient received a total of seven cycles of plasmapheresis, as well as four doses of rituximab. Her respiratory and renal function improved gradually, and she was shifted out of intensive care. DISCUSSION: COVID pneumonia and Granulomatosis with Polyangiitis (GPA) have overlapping clinical presentation. While COVID pneumonia would explain her symptoms, her lack of response to appropriate treatment and progressively worsening renal function hinted towards an alternative diagnosis. Granulomatosis with polyangiitis (GPA) is a systemic disorder that involves necrotizing granulomatous vasculitis of small arteries and veins. It is associated with antineutrophilic cytoplasmic autoantibodies (ANCA). Lower respiratory tract and kidneys are usually affected. Patients commonly present with dyspnea, cough and hemoptysis. Pauci-immune crescentic pattern of glomerulonephritis is observed in histopathology. Diagnostic criteria for GPA includes clinical evidence of disease in at least two of three areas (upper airways, lung and kidney), as well as biopsy proven disease in at least one of these organ systems. GPA is a potentially life-threatening condition if left untreated. (1) Management of severe disease such as in the aforementioned patient includes immunosuppression with corticosteroids in addition to either rituximab or cyclophosphamide. (2) CONCLUSIONS: Differentiating new-onset GPA from COVID pneumonia can be challenging, especially if the non-pulmonary symptoms are overlooked. Nephritic picture on urine analysis should prompt for getting serum ANCA and anti-PR3 titers. Early diagnosis of GPA requires a high degree of suspicion and tissue biopsy is warranted due to mortality rate approaching 90% if left untreated. (3)

Volume

164

Issue

4 Suppl

First Page

A5612

Comments

Chest Annual Meeting 2023, October 8-11, 2023, Honolulu, HI

Last Page

A5613

DOI

10.1016/j.chest.2023.07.3626

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