Neuropathy and myopathy in primary Sjögren's syndrome: neurophysiological, immunological and muscle biopsy results

Abstract

Seventeen consecutive patients with primary Sjögren's syndrome (PSS) received neurophysiological examination, analysis of antibodies against peripheral nerve‐myelin (PNM) and muscle biopsy, to show the prevalence and characteristics of peripheral neuropathy (PN) and myopathy; 3 PSS cases showed a clinical mild sensorimotor polyneuropathy, 1 of them had been treated with cytostatic drugs; 1 had mononeuropathia multiplex; and 1 clinical carpal tunnel syndrome. In these 5 patients neurophysiological investigation verified the clinical diagnosis of peripheral nerve involvement; 2 with PN had serum‐antibodies against PNM; 1 of IgM‐, and 1 of IgA‐isotype. Muscle biopsies were taken from 15 PSS patients; 11 showed inflammatory myositis or inflammatory perivascular infiltrates and 3 showed signs of denervation. A combination of inflammation and morphological signs of myopathy, compatible with the biopsy diagnosis of polymyositis, was seen in 4, 1 of whom showed clinical signs of polymyositis. We conclude that the peripheral nervous and muscular systems are often involved in PSS, but commonly with relatively mild symptoms and laboratory findings. The common findings of inflammatory myopathy indicate an immune reaction in muscles in addition to other autoimmune manifestations of the disease.

The value of animal models to study immunopathology of primary human Sjögren's syndrome symptoms

“…Additionally, extraglandular manifestations are often observed in SjS patients including vasculitis [10], fatigue [11,12], abnormal organ function [13], and various neuropathies. Prior to the past few years, the recognized neural symptoms of SjS included peripheral neuropathy [10,14] and loss of concentration or memory [15] however, more recently, reports have been published focusing on other novel neural symptoms. Similar to the SLE headache, evidence is surfacing that there is a chronic tension-type headache associated with SjS independent of autoantibodies or any other neurological symptoms (fatigue, depression, etc.)…”

Inflammatory myopathies and overlap syndromes: Update on histological and serological profile

“…In previous reports, the occurrence of IMs in SjS patients was estimated in about 3% of cases [56]. According to different reports, it seems that the possibility of a subclinical myositis in SJS patients can be estimated in a wide range of 5e73% [57,58]. Although muscular involvement is usually detected over the course of SjS, it may also appear at disease onset in some cases.…”

Neurological manifestations of primary Sjogren's syndrome

“…For example, in one series, muscle biopsy was abnormal in 13 of 15 PSS patients, only one of whom had symptoms and signs of myopathy. The major histological abnormality was perivascular inflammation in 11 biopsies, typically with perimysial or endomysial infiltrates [26]. In a series of 48 PSS patients from a secondary and tertiary referral center [25], muscle pain was noted in 21 patients (44%), 13 of whom (27%) met criteria for fibromyalgia.…”