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CIDP/autoimmune nodopathies with nephropathy: a case series study
Yuwei Tang1, Jing Liu1, Feng Gao1
1Department of Neurology, Peking University First Hospital, 8 Xishiku Street, Xicheng District, Beijing, 100034, China.
Annals of Clinical and Translational Neurology
|March 18, 2023
Summary
This study found anti-CNTN1 antibody is common in patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and kidney disease. Antibody-positive patients showed distinct clinical and pathological features, suggesting differences in disease presentation.
Area of Science:
- Neurology
- Nephrology
- Immunology
Background:
- Co-morbidity of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP)/autoimmune nodopathies with nephropathy is increasingly recognized.
- Understanding the clinical, serological, and neuropathological features of this co-morbidity is crucial for diagnosis and treatment.
Purpose of the Study:
- To explore the clinical, serological, and neuropathological characteristics of seven patients with co-existing CIDP/autoimmune nodopathies and nephropathy.
- To investigate potential differences between anti-CNTN1 antibody-positive and negative patients within this cohort.
Main Methods:
- Retrospective analysis of clinical, electrophysiological, and laboratory data from seven CIDP patients with nephropathy.
- Nodal/paranodal antibody testing, including anti-CNTN1 antibody.
- Sural nerve biopsies and renal biopsies (in 6 patients) for neuropathological and histopathological examination.
Main Results:
- Seven patients with CIDP and nephropathy were identified; membranous nephropathy was observed in renal biopsies.
- Anti-CNTN1 antibody was detected in four patients, who showed higher rates of ataxia, autonomic dysfunction, higher CSF protein, and more frequent conduction blocks.
- All patients responded to immunotherapy, with some showing good response to corticosteroids alone.
Conclusions:
- Anti-CNTN1 antibody is a frequent finding in patients with CIDP and nephropathy.
- Clinical and pathological differences exist between anti-CNTN1 antibody-positive and negative patients, highlighting the importance of serological testing.
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