Collapsing glomerulopathy in a patient with mixed connective tissue disease

Am J Med Sci. 2022 Jul;364(1):99-105. doi: 10.1016/j.amjms.2022.04.011. Epub 2022 Apr 14.

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.

Keywords: Autoimmune; Collapsing glomerulopathy; Lupus; MCTD; Proteinuria.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Autoimmune Diseases*
  • Female
  • Humans
  • Immunosuppressive Agents
  • Kidney Diseases*
  • Mixed Connective Tissue Disease* / complications
  • Mixed Connective Tissue Disease* / drug therapy
  • Renal Dialysis

Substances

  • Immunosuppressive Agents