Neuro‐Sjögren: Uncommon or underestimated problem?

Abstract

Objectives Sjögren's syndrome (SS) is a chronic inflammatory disease with an autoimmune background with possible complications from peripheral (PNS) and central nervous system (CNS). The aim of this study was to assess the prevalence and to describe the phenotype of peripheral neuropathies in patients with SS. Materials & Methods We studied fifty patients with primary Sjögren's syndrome for peripheral nervous system involvement. All patients underwent neurological and rheumatological examination followed by nerve conduction studies (NCS) of nine peripheral nerves. Results Thirty‐six patients (72%) fulfilled the criteria for the diagnosis of neuropathy. Carpal tunnel syndrome (54%) and axonal sensorimotor neuropathy (22%) were the most common. Neurological symptoms preceded the diagnosis of SS in eight patients. Conclusions Peripheral neuropathies are frequent in SS patients. Neurologists should be aware of possible autoimmune causes of neuropathies because clinical manifestations of neuropathy may precede the development of other symptoms of the autoimmune disease.

Clinical Manifestations, Pathogenesis, Diagnosis and Treatment of Peripheral Neuropathies in Connective Tissue Diseases: More Diverse and Frequent in Different Subtypes than Expected

“…Only one crosssectional study from China in 2018 compared pSS with sSS and reported that sSS patients had a higher prevalence of PN events than pSS patients (31.1% vs. 19%) [17]. Several previous studies found a higher frequency of symmetric sensorimotor polyneuropathy and symmetric sensory polyneuropathy [24,25], but mononeuropathy or mononeuritis multiplex was the most common pattern in these studies [21][22][23]. It is difficult to estimate the precise determination of the prevalence of these manifestations, partly due to the criteria-related variations for inclusion of patients and those related to the recruitment of patients studied, and the manifestations considered.…”

“…SS is the second most common chronic autoimmune rheumatic disease, including primary Sjögren's syndrome (pSS) and secondary Sjögren's syndrome (sSS). We found six valid cross-sectional studies and one cohort study that discussed the prevalence and clinical features of PN in SS [17][18][19][20][21][22][23]. In the studies on pSS, the reported prevalence of PN events ranged from 19% to 72% and could be the initial manifestation [17][18][19][20][21][22][23].…”

“…We found six valid cross-sectional studies and one cohort study that discussed the prevalence and clinical features of PN in SS [17][18][19][20][21][22][23]. In the studies on pSS, the reported prevalence of PN events ranged from 19% to 72% and could be the initial manifestation [17][18][19][20][21][22][23]. Only one crosssectional study from China in 2018 compared pSS with sSS and reported that sSS patients had a higher prevalence of PN events than pSS patients (31.1% vs. 19%) [17].…”

“…Older age at SLE diagnosis [10,12,13] Higher SLE Disease Activity Index 2000 scores [11][12][13][14] Cutaneous vasculitis [12,14] Sjögren's syndrome Salivary gland enlargement [22,26] Hypocomplementemia [17,22] Eosinophilic granulomatosis with polyangiitis Myeloperoxidase-ANCA positivity [38,39] Skin, musculoskeletal, cardiovascular involvement [38] Rheumatoid arthritis DAS-28 [49] Inflammatory markers of disease activity [49,78] Increased platelet numbers [78] In SLE, Saigal et al [11] reported that PN-SLE patients had a statistically significant higher occurrence of pyuria, pleuritis and leukopenia. Xianbin et al [8] reported that complement 3 (C3) hypocomplementemia and anti-Sm antibody (anti-Sm) positivity occurred more frequently, and remarkably elevated concentrations of serum immunoglobulin G (IgG) were measured in PN-SLE patients compared to non-PN-SLE patients.…”

Integrated Network Pharmacology and Mice Model to Investigate Qing Zao Fang for Treating Sjögren’s Syndrome

“…Sjögren's syndrome (SS) is well-recognized as a systemic autoimmune disease, and it is characterized by manifestations of sicca (dryness) of the oral, ocular mucosa, and genital taking diverse exocrine glands as targets [ 1 ]. This disorder is categorized into primary SS existing as an independent entity and secondary SS with a coexisting entity [ 2 ]. Symptoms of secondary SS are aggravated accompanying with additional autoimmune diseases including systemic lupus erythematosus, systemic sclerosis, or rheumatoid arthritis [ 3 ].…”