Collapsing glomerulopathy in a patient with mixed connective tissue disease
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CASE REPORT
Collapsing glomerulopathy in a patient with mixed connective tissue disease Gajapathiraju Chamarthi1 · William L. Clapp1 · Saraswathi Gopal1 Received: 31 March 2020 / Accepted: 30 September 2020 © Japanese Society of Nephrology 2020
Abstract Collapsing glomerulopathy (CG) is a distinct podocytopathy characterized by the global or segmental collapse of glomerular capillary tuft with overlying podocyte hypertrophy and hyperplasia. CG has been associated with numerous etiologies, including infections, autoimmune disorders, drugs, and malignancies. Anecdotal reports of CG in patients with mixed connective tissue disease (MCTD) have been reported in the literature. We report a case of a 53-year-old female who presented to us with acute kidney injury and proteinuria. The patient underwent renal biopsy for further evaluation of her proteinuria, and was diagnosed to have collapsing glomerulopathy. The patient was subsequently diagnosed with MCTD, given her constellation of symptoms and serology titers. The patient was started on prednisone with subsequent stabilization of renal function and reduction of proteinuria and continues to be in remission. We report our case to highlight the association between collapsing glomerulopathy and MCTD and the potential role of steroids as first-line therapy in such cases. Keywords Collapsing glomerulopathy · Collapsing FSGS · Mixed connective tissue disease · Autoimmune disorder
Introduction
Case presentation
Collapsing glomerulopathy (CG) is a podocytopathy which manifests as massive proteinuria with a rapid clinical deterioration of renal function and is often resistant to the standard treatments [1]. CG is an important cause of ESRD and is now recognized as a distinct disease characterized by the global or segmental collapse of glomerular capillary tuft along with epithelial cell proliferation in Bowman’s space [1, 2]. CG has been strongly associated with HIV (Human immunodeficiency virus) in the past. Overtime CG has been reported in non-HIV patients and has been associated with various etiologies, including infections, autoimmune disorders, hereditary disorders, drugs, and malignancies. The overall prognosis of patients with CG is poor, and currently, no evidence-based guidelines exist in the management of this disease. We present a case of CG associated with mixed connective tissue disease (MCTD) who responded to prednisone treatment.
A 53-year-old African American woman with a history of hypothyroidism and ulcerative colitis saw her primary care physician for complaints of facial puffiness and itching. She was found to be in acute renal failure and was sent to the emergency room for further evaluation. She has a history that is significant for joint pains, puffiness of the fingers, and photosensitivity for 20 years. Evaluation by rheumatology before this event showed positive antinuclear antibody (ANA) titers, but her presentation did not meet the criteria for any specific autoimmune disorder. Her joint pain responded to treatment with methotrexate. She was dia
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