Neurophysiological Features of Peripheral Nervous System Involvement and Immunological Profile of Patients with Primary Sjögren Syndrome

Perzyńska-Mazan, Joanna; Maślińska, Maria; Gasik, Robert

doi:10.3899/jrheum.181464

Cited by 13 publications

(16 citation statements)

References 29 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…The nervous system is one of the targets of systemic damage in pSS patients ( 6 ). Neurologic manifestations of pSS are diverse, and may involve the peripheral nervous system and/or central nervous system ( 7 ). The coexistence of MG and pSS is limited to reports of one to two cases ( 8 – 21 ).…”

Section: Introductionmentioning

confidence: 99%

Myasthenia Gravis Coexisting With Primary Sjögren's Syndrome: Report of Three Cases and Literature Review

Zhao

Liao

et al. 2020

Front. Neurol.

View full text Add to dashboard Cite

Objective: The coexistence of myasthenia gravis (MG) and primary Sjögren's syndrome (pSS) is rarely reported. This study aims to describe the clinical features, treatment and outcome of MG coexisting with pSS. Materials and Methods: Herein we reported three cases with the two coexisting diseases, and also searched the PubMed, Medline databases, and China Wanfang databases for the relevant case reports written in English, Chinese, or Japanese with detailed data. Results: We reviewed a total of 17 patients with both diseases. Fifteen patients were female. The median age at onset was 48 years (range 28–78 years). MG was the initial disease in nine of 17 cases. The median interval between the onsets of the two diseases was 30 months (range 7 months to 20 years). The symptoms of MG included fatigable ptosis (64.7%), bulbar symptoms (58.8%), muscle fatigability (64.7%), diplopia (64.7%), dyspnea (23.5%), and facial paralysis (5.9%). Anti-acetylcholine receptor antibody was positive in 70.6% patients. All the patients had sicca symptoms. Manifestations of pSS also included swollen exocrine glands (23.5%), joint pain (23.5%), hair loss (11.8%), leukopenia (11.8%), recurrent oral ulcers (5.9%), Raynaud phenomenon (5.9%), and fever (5.9%). ANA positivity was present in 70.6% patients, anti-SSA positivity in 47.1%, and double positivity of anti-SSA and anti-SSB in 17.6%. There were 12 patients (70.6%) with two autoimmune diseases (pSS and MG), and five patients with more than two autoimmune diseases. Cholinesterase inhibitors were the most commonly prescribed drugs (82.4%). Seven patients received thymectomy and one patient improved after the operation. Two patients were given intravenous methylprednisolone pulse therapy, and four patients oral steroids combined with immunosuppressants initially. Intravenous immunoglobulin and plasma exchange were used in two patients, respectively, for the respiratory failure. All the patients improved following treatment except one patient who died of MG crisis due to medication withdrawal. Conclusion: The coexistence of SS with MG is quite rare. The onset of MG may occur before or after the diagnosis of SS. Co-morbidity with MG does not seem to adversely affect the course of SS. Thus, controlling the progress of MG is the critical aspect of treatment.

show abstract

Section: Introductionmentioning

confidence: 99%

Myasthenia Gravis Coexisting With Primary Sjögren's Syndrome: Report of Three Cases and Literature Review

Zhao

Liao

et al. 2020

Front. Neurol.

View full text Add to dashboard Cite

show abstract

“…Further testing of clinically diagnosed peripheral neuropathy patients revealed that the majority of the test group had sensory neuropathy (8/13, 61%), while a small proportion qualified as both sensory and motor [22]. Conversely, smaller cohorts studies proved significantly increased positivity for anti-Ro/SSA and anti-La/SSB antibodies in pSS patients diagnosed with peripheral neuropathy [23,24]. B-cell proliferation markers were generally higher in PN patients as reported by multiple authors, with a consistent higher incidence of hypergammaglobulinemia in non-PN patients [21,25].…”

Section: Discussionmentioning

confidence: 99%

Decreased Protein Levels and Vitamin B12 Plasma Concentration in Primary Sjögren Syndrome are Associated with Peripheral Neuropathy: A Case-Control Study

Ancuta¹,

Maria²,

Mihnea³

et al. 2022

J Musculoskelet Disord Treat

View full text Add to dashboard Cite

Background: Prognostic factors for peripheral nervous system (PNS) manifestations in primary Sjögren Syndrome (pSS) are essential for predicting and preventing diseaserelated disability. Given the established relationship between vitamin B12 deficiency and pSS, we aimed to investigate the predictive value of low B12 concentration for Sjögren's induced peripheral neuropathy (PN). Methods:We conducted a case-control study which included 96 patients with pSS, 13 of which were diagnosed with PN. We measured multiple nutrition-related biomarkers, including total protein levels, vitamin B12, folic acid, and iron concentrations.Results: Total protein serum concentration was lower in PN diagnosed patients compared to the control group (7.15 vs. 7.64 g/dL, p < 0.05). In addition, the mean concentration of plasma B12 was decreased in the PN group with regard to control (431.92 vs. 588.43 ng/L, p < 0.05). However, a significant predictive pattern was not established for either of the biomarkers. Conclusions:Peripheral neuropathy in pSS is associated with reduced serum protein and vitamin B12 levels. Although a predictive correlation was not demonstrated, the screening for early deficit is justified and recommended.

show abstract

“…The frequency, prevalence, and diagnostic criteria for peripheral nervous system involvement in SS have been the object of several studies as well as the underlying pathogenetic mechanisms. The reported prevalence of PN varies from 19 to 72% and may represent the first clinical manifestation (61)(62)(63)(64)(65). A cross-sectional study from China in 2018 reported a higher prevalence of PN in sSS than in pSS patients (31.1 vs. 19%) (61).…”

Section: Sjögren's Syndromementioning

confidence: 99%

High-resolution ultrasound of peripheral neuropathies in rheumatological patients: An overview of clinical applications and imaging findings

Picasso¹,

Pistoia

Sanguinetti

et al. 2022

Front. Med.

View full text Add to dashboard Cite

Peripheral neuropathies are surprisingly common and can be associated with a number of conditions, including rheumatological diseases. Whether the co-existence of peripheral neuropathies with rheumatological disorders is coincidental or related to a common pathogenic mechanism, these disabling conditions can affect the outcome of rheumatological patients and should be targeted with specific treatment. The clinical presentation of peripheral neuropathy can be multifaceted and difficult to recognize in polysymptomatic patients. However, physicians adopting state-of-art diagnostic strategies, including nerve imaging, may improve the detection rate and management of neuropathies. In particular, a diagnostic approach relying exclusively on clinical history and nerve conduction studies may not be sufficient to disclose the etiology of the nerve damage and its anatomical location and thus requires integration with morphological studies. High-Resolution Ultrasound (HRUS) is increasingly adopted to support the diagnosis and follow-up of both joint disorders in rheumatology and peripheral neuropathies of different etiologies. In this review, the different types of nerve disorders associated with the most common syndromes of rheumatological interest are discussed, focusing on the distinctive sonographic features.

show abstract

Neurophysiological Features of Peripheral Nervous System Involvement and Immunological Profile of Patients with Primary Sjögren Syndrome

Cited by 13 publications

References 29 publications

Myasthenia Gravis Coexisting With Primary Sjögren's Syndrome: Report of Three Cases and Literature Review

Myasthenia Gravis Coexisting With Primary Sjögren's Syndrome: Report of Three Cases and Literature Review

Decreased Protein Levels and Vitamin B12 Plasma Concentration in Primary Sjögren Syndrome are Associated with Peripheral Neuropathy: A Case-Control Study

High-resolution ultrasound of peripheral neuropathies in rheumatological patients: An overview of clinical applications and imaging findings

Contact Info

Product

Resources

About