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Collapsing glomerulopathy in a patient with mixed connective tissue disease
Mohammad Atari1, Josephine M Ambruzs2, Osaid Saqqa3
1Division of Nephrology and Hypertension, School of Medicine, Tulane University, New Orleans, LA, USA.
Insights
Collapsing glomerulopathy (CG) in mixed connective tissue disease (MCTD) is rare. Steroids and mycophenolate showed promise in one challenging case, highlighting the need for treatment guidelines.
Area of Science:
- Nephrology
- Rheumatology
- Immunology
Background:
- Collapsing glomerulopathy (CG) is a severe podocytopathy with challenging management.
- CG can be idiopathic or linked to autoimmune connective tissue diseases (ACTDs).
- Therapeutic guidelines for CG in ACTDs are lacking.
Observation:
- A unique case of CG with mixed connective tissue disease (MCTD) is presented.
- The patient's CG responded to initial steroid therapy followed by mycophenolate.
- A literature review identified three prior cases of CG with MCTD and other autoimmune diseases.
Findings:
- CG associated with ACTDs disproportionately affects females and Black patients.
- Treatment responses in CG associated with ACTDs are variable and often inconsistent.
- Despite various treatments, many patients with CG in ACTDs progress to end-stage renal disease requiring dialysis.
Implications:
- This case and review underscore the complexity of managing CG in the context of MCTD.
- Further research is needed to establish effective therapeutic strategies for CG in ACTDs.
- Developing clear treatment guidelines is crucial for improving outcomes in these patients.
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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