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

Author:

Kunpara Janmejay Ashvinkumar1,Darji Bhavya Prakash2,Patel Himanshu Arvindbhai1,Patwari Devang1,Patel Sujit Shaileshbhai1,Darji Prakash Ishwarlal1

Affiliation:

1. Department of Nephrology, Zydus Hospitals, Gujarat, India

2. GCS Medical College, Ahmedabad, Gujarat, India

Abstract

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.

Publisher

Scientific Scholar

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