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Patient-Centered Focused Review| Volume 364, ISSUE 1, P99-105, July 2022

Collapsing glomerulopathy in a patient with mixed connective tissue disease

  • Author Footnotes
    # Present address: Mohammad Atari, MD, University of Mississippi Medical Center, Division of Nephrology and Hypertension, 2500 North State Street, Office L520, Jackson, MS 39110, USA.
    Mohammad Atari
    Correspondence
    Corresponding author at: Mohammad Atari, MD, University of Mississippi Medical Center, 2500 North State Street, Office L520, Jackson, MS, 39216 USA
    Footnotes
    # Present address: Mohammad Atari, MD, University of Mississippi Medical Center, Division of Nephrology and Hypertension, 2500 North State Street, Office L520, Jackson, MS 39110, USA.
    Affiliations
    Division of Nephrology and Hypertension, School of Medicine, Tulane University, New Orleans, LA, USA
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  • Josephine M. Ambruzs
    Affiliations
    Arkana Laboratories, Little Rock, AK, USA
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  • Osaid Saqqa
    Affiliations
    Internal Medicine Department, School of Medicine, Tulane University, New Orleans, LA, USA
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  • Eric E. Simon
    Affiliations
    Division of Nephrology and Hypertension, School of Medicine, Tulane University, New Orleans, LA, USA
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  • Author Footnotes
    # Present address: Mohammad Atari, MD, University of Mississippi Medical Center, Division of Nephrology and Hypertension, 2500 North State Street, Office L520, Jackson, MS 39110, USA.

      Abstract

      Collapsing 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.

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