Open AccessA Rare Case of Pulmonary Renal Syndrome Secondary to Microscopic Polyangiitis – A Case Report
Author(s) -
Viraj Shah,
Alexandra Lacqua,
Gregory Demirjian,
Akash J. Patel,
Dhvanit I. Shah,
Taaha Mendha
Publication year - 2022
Publication title -
frontiers in medical case reports
Language(s) - English
Resource type - Journals
ISSN - 2582-8142
DOI - 10.47746/fmcr.2022.3102
Subject(s) - medicine , microscopic polyangiitis , pulmonary hemorrhage , plasmapheresis , bronchoalveolar lavage , diffuse alveolar hemorrhage , rapidly progressive glomerulonephritis , granulomatosis with polyangiitis , nephrology , renal biopsy , vasculitis , biopsy , pathology , gastroenterology , kidney , glomerulonephritis , lung , immunology , antibody , disease
Diffuse Alveolar Hemorrhage (DAH) is one of the life-threatening pulmonary complications of AAV (ANCA-Associated Vasculitis). Pulmonary Renal Syndrome (PRS) is defined as concurrent autoimmune-induced DAH and Rapidly progressive glomerulonephritis (RPGN). Major organs involved in MPA (Microscopic Polyangiitis) are the kidneys and the lungs. The gold standard to confirm alveolar hemorrhage is bronchoalveolar lavage (BAL). We hereby describe a case in which our patient presented to the emergency room after being sent by his nephrologist for kidney biopsy in the setting of unexplained worsening renal functions. Patient ultimately developed PRS and was started on remission treatment with pulse dose steroids, plasmapheresis, and cyclophosphamide. Unfortunately, the patient deceased from multiorgan failure secondary to septic shock. PRS, once suspected, appropriate autoimmune workup should be undertaken, usually followed by renal biopsy.