Focal Crescentic Glomerulonephritis Superimposed on Myeloproliferative Disease-Related Glomerulopathy in a Case of Myelofibrosis.

Autor: Kunpara JA; Department of Nephrology, Zydus Hospitals, Gujarat, India., Darji BP; GCS Medical College, Ahmedabad, Gujarat, India., Patel HA; Department of Nephrology, Zydus Hospitals, Gujarat, India., Patwari D; Department of Nephrology, Zydus Hospitals, Gujarat, India., Patel SS; Department of Nephrology, Zydus Hospitals, Gujarat, India., Darji PI; Department of Nephrology, Zydus Hospitals, Gujarat, India.
Jazyk: angličtina
Zdroj: Indian journal of nephrology [Indian J Nephrol] 2024 Jan-Feb; Vol. 34 (1), pp. 64-66. Date of Electronic Publication: 2023 Feb 21.
DOI: 10.4103/ijn.ijn_218_22
Abstrakt: Proliferative glomerulonephritis in myelofibrosis is a very rare. Mesangial proliferation and sclerosis with changes of chronic thrombotic microangiopathy have been reported, but pauci-immune focal crescentic glomerulonephritis has not been described so far. Herein, we present a 68-year-old male who was a known case of myelofibrosis and presented with rapidly progressive glomerulonephritis and nephrotic range proteinuria. He was diagnosed as anti-neutrophil cytoplasmic antibody (ANCA)-negative focal crescentic glomerulonephritis, and he responded well to a course of intravenous methylprednisolone and cyclophosphamide. Pauci-immune focal crescentic glomerulonephritis may occur in myelofibrosis without ANCA and may be related to unknown pathogenetic mechanisms in myeloproliferative disorders or suggest any superimposed pathology that might respond well to immunosuppressants.
Competing Interests: There are no conflicts of interest.
(© 2024 Indian Journal of Nephrology | Published by Scientific Scholar.)
Databáze: MEDLINE