Management Approach For Microscopic Polyangiitis: A Case Report

Mohammed Al Azzawi
Alfarooq Alshaikhli
Mustafa Altaei
Abbas Alshami
Alsadiq Al-Hillan, Beaumont Health Fellow
Asseel Albayati
Eric Costanzo


Microscopic polyangiitis is a small vessel necrotizing vasculitis. It usually exhibits its manifestation through inflammation on the kidneys, it also presents with an extrarenal systemic involvement such as the lungs, musculoskeletal systems, central or peripheral nervous system, and skin manifestations. Laboratory markers show an elevation in the inflammatory markers and Myeloperoxidase-Antinuclear Cytoplasmic Antibody. Kidney involvement usually manifests in the form of rapidly progressive pauciimmune glomerulonephritis. The Lung present at the range of fleeting local infiltrates to massive hemorrhage is the setting of diffuse alveolar hemorrhage. Patients generally respond appropriately to induction therapy with high IV pulse-dose prednisolone plus an additional immunosuppressive agent followed with maintenance therapy with an oral immunosuppressive drug, the response to the guidelines-recommended regimen is significant in most of the patients and results in symptoms recovery concomitantly. Notwithstanding, we present a case of a female with a recent diagnosis with microscopic polyangiitis confirmed with a kidney biopsy, achieved complete remission with the combination of prednisone and cyclophosphamide. While she was on induction therapy, she developed a major relapse in the form of diffuse alveolar hemorrhage and needed an aggressive form of management in the form of plasmapheresis, prednisone, and rituximab to stabilize her condition.