• Mohammad Atari, Josephine M Ambruzs, Osaid Saqqa, and Eric E Simon.
• Division of Nephrology and Hypertension, School of Medicine, Tulane University, New Orleans, LA, USA. Electronic address: matari@umc.edu.
• Am. J. Med. Sci. 2022 Jul 1; 364 (1): 99-105.

AbstractCollapsing glomerulopathy (CG) is a form of podocytopathy that is challenging to manage. CG can be idiopathic or associated with other conditions including autoimmune connective tissue diseases. In the setting of autoimmune connective tissue diseases, there are no current guidelines to guide therapy. Here we report a unique and challenging case of CG with mixed connective tissue disease (MCTD) that responded to steroids followed by mycophenolate. In PubMed, we identified three previously reported cases of CG with MCTD in addition to other forms of autoimmune diseases, including Sjogren syndrome, adult-onset still's disease, and vasculitis, etc. We are providing a literature review of collapsing glomerulopathy cases in the setting of autoimmune connective tissue diseases and with MCTD. CG in the setting of autoimmune connective tissue diseases is more common in females and black patients. Response to therapy was inconsistent. Many patients progressed to dialysis despite use of various treatment modalities.Copyright © 2022 Southern Society for Clinical Investigation. Published by Elsevier Inc. All rights reserved.

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