Background: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is one of the most common autoimmune peripheral neuropathies in adults. Membranous nephropathy (MN), focal segmental glomerulosclerosis (FSGS), and other nephropathy have been reported in CIDP patients and are possibly correlated to CIDP pathogenesis. This study reviewed the previously described cases of patients with CIDP and nephropathy in order to provide comprehensive evidence on the diagnosis and treatment regarding CIDP patients in the context of renal diseases.
Method: We reviewed our database to identify patients with CIDP and nephropathy. Online database including PubMed, EMBASE, and OVID were searched for relevant cases.
Results: We identified a total of 18 cases with CIDP and nephropathy, including 2 cases from our database and 16 ones from online searching. A predominance of male was observed [14 (77.8%)] with the mean age of 53.3 (standard deviation, SD: 16.6) years old. Almost all patients complained paresthesia in distal limbs (94.4%), except one only presented weakness of four extremities. Corticosteroids were prescribed for 14 (77.8%) patients, and 10 showed responsiveness. Three patients experienced relapses during the gradual tapering of steroids.
Conclusion: The same immune-mediated pathogenesis may be involved in CIDP and concomitant nephropathy. Male and sensory-predominant CIDP are red flags for complications of renal diseases in CIDP patients. Corticosteroids remain the first-line treatment for CIDP when complicated with renal diseases. Slower tapering or long-term maintenance of steroids may be beneficial for the prognosis of patients with CIDP and nephropathy.
Keywords: Association; Chronic inflammatory demyelinating polyradiculoneuropathy; Nephropathy; Review.
© 2022. Fondazione Società Italiana di Neurologia.