Immune-mediated demyelinating polyradiculoneuropathies-acute inflammatory demyelinating polyneuropathy (AIDP)/Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating polyneuropathy (CIDP)-are rare but potentially life-threatening manifestations reported in association with systemic lupus erythematosus (SLE). Evidence is scattered, and optimal diagnostic and therapeutic strategies remain uncertain. We performed a Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA)-guided systematic review of PubMed, Embase, Cochrane Library, Scopus, and Google Scholar to identify English-language studies describing AIDP/GBS or CIDP occurring in patients with SLE and reporting clinical features, diagnostic findings, treatments, and outcomes. A total of 2,121 articles were yielded. After removal of duplicates, titles/abstracts were screened, full texts were assessed using predefined eligibility criteria, and data were extracted using a standardized form. Four studies met the inclusion criteria for qualitative synthesis. Across AIDP/GBS cohorts, there was a marked female predominance and a frequent “neuropathy-first” pattern, with AIDP/GBS preceding formal SLE diagnosis in more than half of reported cases. Clinically, acute limb weakness was the most common presentation, often accompanied by sensory symptoms. Cranial nerve involvement and respiratory involvement were also seen in many patients. Similarly, CIDP demonstrated strong female predominance and had variable temporal association with SLE, presenting as an initial, concomitant, or delayed manifestation during the course of SLE. Diagnostic evaluations consisted of cerebrospinal fluid studies and nerve conduction study; albuminocytologic dissociation and demyelinating features were supportive but not uniformly present across CIDP reports. Antiganglioside antibodies were usually negative in the tested patients. Treatments consisted of combined standard immunomodulatory approaches such as intravenous immunoglobulin (IVIg) and/or plasma exchange (PLEX) with lupus-directed immunosuppression (high-dose corticosteroids and cyclophosphamide). Outcomes were generally favorable in AIDP/GBS but had heterogeneous functional recovery in CIDP, particularly when therapy was delayed.
Vadher et al. (Wed,) studied this question.