Collapsing glomerulopathy in a patient with mixed connective tissue disease

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.

Automated summary

Collapsing glomerulopathy (CG) is a challenging form of podocytopathy that may be associated with autoimmune connective tissue diseases. There are no current guidelines for the management of CG in the setting of these diseases. It is more common in females and black patients, and the response to therapy is inconsistent, with many patients progressing to dialysis despite various treatment modalities.