Assessment of Chronic Inflammatory Demyelinating Polyneuropathy in Systemic Lupus Erythematosus

Background: The neurological signs of Systemic Lupus Erythematosus (SLE) vary, but the central nervous system (CNS) is more typically affected than the peripheral nervous system. The prevalence of neuropsychiatric or CNS manifestations can range from 24-54 percent, while involvement of the peripheral nervous system is less common, at around 5-27 percent. One of the three most prevalent peripheral nerve system involvements in SLE patients is Chronic Inflammatory Demyelinating Polyradiculopathy (CIDP), which has significant debilitating effects.

Aims: We review all SLE patients that presented with CIDP and report on their clinical manifestations, treatment and progress, and a brief review of the literature on CIDP in SLE patients.

Methods: A retrospective review of all SLE patients that were diagnosed with CIDP between 2000 and 2015 was done under follow up at our center that were diagnosed with CIDP between 2000 and 2015. We reviewed their medical records and analyzed their clinical presentation, investigations, treatment instituted, response to therapy and any neurological sequealae.

Results: A total of 512 case notes were reviewed. Of these 4 patients presented with CIDP (3 females, 1 male) aged between 26 to 46 years old. Three presented with transverse myelitis and the other one with acute motor and sensory axonal neuropathy. All patients were treated with high dose corticosteroids, three patients received cyclophosphamide whilst the other patient was induced with mycophenolate mofetil. Complete recovery was seen in one patient, two had persistent but improving numbness and the other one had a residual weakness. Two of our patients developed CIDP around the time of SLE diagnosis whereas in the other two, it developed many years later during the course of their disease.

Conclusion: Peripheral nervous system involvement in SLE can result in serious debilitating effects. Early diagnosis and treatment are crucial in limiting the neurological sequealae.