Clinical Features of Chronic Inflammatory Demyelinating Polyneuropathy With Autoimmune Hepatitis

Abstract

Chronic inflammatory demyelinating polyneuropathy (CIDP) with autoimmune hepatitis is rare in clinical practice. Here we present a case to describe the relevant clinical features and provide case data for the diagnosis and treatment of the disease. The diagnosis was supported by medical history, physical examination, laboratory and imaging examination, nerve conduction velocity and cerebrospinal fluid examination. The combined disease was identified by autoimmune antibodies and ultrasonography. Combined with the patient’s history of chronic progression, clinical manifestations of peripheral nerve damage, cerebrospinal fluid showing protein-cell separation and nerve conduction velocity showing decreased amplitude and conduction velocity, CIDP was confirmed. Combined with the patient’s examination result of abdominal bulging, color Doppler ultrasound examination showed cirrhosis, positive (+) anti-liver/kidney microsomal type 1 antibody (anti-LKM-1), considering combination of autoimmune hepatitis type II (AIH-II). The clinical symptoms of both were significantly improved by hormone therapy. CIDP is clinically uncommon in elderly male patients, and it is also rare to have AIH-II. But according to the typical pathogenesis, clinical manifestations, detailed physical examination, laboratory and imaging examination, and neuro-immunological data, clinical diagnosis can be made. Pathological data are needed to confirm the diagnosis. Both have poor natural prognosis. However, the use of hormone therapy according to its pathogenesis can effectively alleviate clinical symptoms and prevent progression. J Neurol Res. 2020;10(1):25-29 doi: https://doi.org/10.14740/jnr542