Chronic inflammatory demyelinating polyradiculoneuropathy associated with systemic lupus erythematosus

Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP) is an uncommon subtype of peripheral neuropathy (PN) and especially when associated with systemic lupus erythematosus (SLE). There are few reports characterizing PN-associated to SLE, in particular CIDP. This study reviewed the frequency and profile of SLE-related CIDP in our cohort and in the literature and propose a treatment scheme for CIDP associated with SLE.

We reviewed our database to identify patients with CIDP and SLE. The literature was also reviewed following the guidelines of PRISMA and using the terms "Polyradiculoneuropathy", "Chronic inflammatory demyelinating polyradiculoneuropathy", "CIDP", "Systemic lupus erythematosus", "SLE", "Autoimmune diseases of the nervous system" until December 2019. Selected articles were published in English.

We identified 3 patients with SLE and CIDP in our cohort of 1,349 patients with SLE (0.2%). All patients were female, aged between 30 and 44 years and 2 (66.7%) had active disease in other organs. In the literature, we identified additional 16 patients. A predominance of women with disease activity, specially nephritis and hematological involvement, was observed. Treatment schemes are diverse, including corticosteroids and immunosuppressive drugs.

Although rare, CIDP has increased frequency in SLE. Women and younger age should rise suspicion of an underlying autoimmune disease. We suggest that CIDP should be included as a possible neuropsychiatric manifestation in SLE.