Introduction: Sjögren’s syndrome (SS) is a chronic autoimmune disease characterized by lymphocytic infiltrates in the exocrine glands. Carpal tunnel syndrome (CTS) is suggested to be more frequent among SS patients than in the general population. The aim of this study was to seek associations between the CTS and the laboratory and clinical findings of SS patients. Methods: Fifty patients diagnosed with primary SS (pSS) were examined. Clinical evaluation by a rheumatologist and electrophysiological studies were conducted. Data on laboratory tests results was collected. Control group consisted of 50 sex and age-matched individuals with osteoarthritis (OA). Results: Out of 50 patients in the study group 27 (54%) were diagnosed with CTS. The prevalence of CTS among 50 individuals in the control group was 8%. Among pSS patients with CTS the joint involvement was not more common than in those from the non-CTS group [15 vs. 13 (p = 0.945)]. There was an expected difference in sleep disorders [18 vs. 9 (p = 0.012)] and paresthesia [23 vs. 13 (p = 0.024)]. The major finding was a significant difference in elevated beta2-microglobulin (B2MG) [23 vs. 13 (p = 0.024)]. Other studied factors, suggested in the literature as significant in the pSS-related neuropathy, were not statistically different between the groups. Conclusion: Our study confirms that CTS is more prevalent among pSS patients than in the general population and suggests that a new approach is required towards the pathogenesis of this phenomenon. We hypothesize that CTS is more associated with an overall disease activity than joint involvement as such.
Even though primary Sjögren disease (pSjD) is mainly associated with sicca symptoms, there are extraglandular manifestations of the disease which affect the quality of life of patients the most and may even be life-threatening. Among the most severe, polyneuropathy and myopathy are worth mentioning. Additionally, clinical observations suggest a higher prevalence of fibromyalgia (FM) in this group of patients, clouding physicians’ assessment and potentially leading to unsuccessful therapeutic decisions. The aim of our study was to evaluate the frequency of pSjD and FM co-occurrence as well as to find the most effective screening tools and markers of such overlap. A total of 97 consecutive patients with diagnosed pSjD were incorporated in the study after obtaining their informed consent. Participants completed a set of broadly available questionnaires, including Fibromyalgia Survey Questionnaire, SF-36 and EULAR Sjögren’s Syndrome Patient-Reported Index (ESSPRI). Data on their laboratory results was collected in the dedicated database. Moreover, patients underwent electroneurographic (ENG) and electromyographic (EMG) testing. Central nervous system (CNS) abnormalities were detected using MRI. Objective disease activity was evaluated based on EULAR Sjögren’s Syndrome Disease Activity Index (ESSDAI). The mean age was 55.3 (range 19.0–78.0 years, SD = 13.9). The disease duration ranged from 2 to 42 years (M = 9.03 years, SD = 7.1 years). Nearly half of the participants (n = 44, 45%) met diagnostic criteria of FM. Interestingly, the diagnosis of FM correlated with CNS involvement. There was no significant correlation between FM and either polyneuropathy/myopathy nor laboratory findings (however, C3c and folic acid concentrations were near the level of significance—mean 1.2 vs. 1.29; p = 0.075 and mean 11.35 vs. 9.21; p = 0.071, respectively). Within the subcategories of SF-36 and ESSPRI scales, significant positive correlation was noted with ESSPRI total score and ESSPRI pain score (neuropathic subcategory), while a negative correlation was found with SF-36 vitality score, physical functioning score, and the SF-36 total score. FM is common among pSjD patients and should be considered rather a comorbidity requiring different therapeutic approaches. At the fast-paced clinical environment, a concise ESSPRI assessment may be helpful in the initial screening of patients at risk of FM. Even though the origin of this phenomenon is unknown, the concepts of central sensitization and microglia polarization may be potential explanations and more molecular research in this direction could benefit the pSjD patients.
Polyneuropathy is a common condition that limits the quality of life among patients with primary Sjögren disease (pSjD). Somatic sensory fiber neuropathy involving small myelinated (A-δ) and unmyelinated C fibers may precede the development of sicca syndrome. The cutaneous silent period (CSP) is an inhibitory spinal reflex that can be used as a tool for evaluating the dysfunction of A-δ fibers. This study sought to examine CSP parameters, and their correlates, in patients with pSjD vs. healthy controls. We recruited 134 consecutive patients with a diagnosis of pSjD, of whom 109 subjects were included in the analysis. Electrodiagnostic tests comprised a nerve conduction study (NCS) and CSP analysis, alongside laboratory tests and questionnaires (the ESSPRI and SF-36). The examination of the healthy control (HC) group consisted of 113 NCSs and CSP studies. NCS tests of the median nerve in both groups were within the normal range. Statistical analysis revealed a significant difference in CSP duration (p < 0.001), S1 latency (p < 0.001) and S2 latency (p < 0.001) between the pSjD and HC groups. We observed prolonged CSP duration in approximately 38% of patients with pSjD and prolonged S2 latency in 18.35%. Small A-delta fiber neuropathy was diagnosed in 38% (41 subjects) patients. A regression analysis of CSP parameters indicated an association between the age of patients and PM Scl-75 antibodies (ab) levels in the pSjD cohort. As a new, noninvasive method of assessing A-δ nerve fibers, CSP was found to have a relation to the age and PM Scl-75 antibodies in patients with pSjD. The utility and sensitivity of CSP as a test for screening A-δ fiber function require further investigation in large cohorts of the pSjD population.
The impact of vitamin B complex has long been studied and utilized in clinical practice, both for healthy and diseased individuals. Its beneficial effects on physical and mental performance have been proven by multiple studies leading to widespread supplementation among the general population. As of today’s knowledge, vitamin B6 (pyridoxine) in excessive doses may lead to symptoms of neuropathy. Unfortunately, many of those supplementing vitamin B complex are unaware of such a risk. This case-based review presents three patients who initially searched for help due to neuropathic symptoms with the rheumatological diagnostic process following. All of them had a history of excessive vitamin B6 consumption, though through different sources. Cessation of supplementation led to prompt improvement in symptoms. These cases stress the importance of in-depth history taking and specifying instructions regarding prescribed supplements. The novelty of this article lies in the description of a patient who reported symptoms associated with central nervous system with concomitant abnormalities of the complement system, all of which resolved spontaneously upon normalization of pyridoxine concentration.
Musculo-skeletal complaints in a patient suffering from systemic lupus, with co-existing chronic renal failure, undergoing immunosuppressive treatment after kidney transplantation, can have a varied etiology. The aim of this work was to present a case based review of differential diagnosis of knee pain in such a patient. A literature search was carried out using MEDLINE/PubMed, Google Scholar and EBSCO, with no time limit. We undertook a systematic review of the literature published in English, limited to full-text publications of original articles, letters to the editor, and case reports in peer-reviewed journals, for a discussion and analysis of studies reporting arthralgia in patients with lupus after kidney transplantation. We present a case report of a 45-year-old woman with lupus nephritis, after kidney transplantation, who started to complain of increasing pain in the knees, most pronounced at night and after physical activity approximately 2 years after transplantation. Extensive causal diagnostics were carried out, which revealed bilateral extensive regions of bone infarction in the femur and tibia, chondropathy, degenerative changes of medial meniscuses in the body and posterior horn as well as chondromalacia of the patella. Establishing the right diagnosis is crucial for implementing appropriate treatment.
Peripheral neuropathy constitutes a prevalent, albeit underrecognized, extraglandular manifestation of Sjögren’s disease (SjD). Carpal tunnel syndrome (CTS) commonly coexists with SjD; however, the efficacy of standard CTS treatments in this population remains insufficiently characterized. This study evaluated the clinical and biological response to local corticosteroid injection in CTS patients with and without SjD and explored factors potentially associated with treatment outcomes. Twenty patients with SjD and CTS diagnosed based on typical clinical symptoms and supportive ultrasonographic findings and 19 control subjects with idiopathic CTS underwent ultrasound-guided corticosteroid injection. Median nerve cross-sectional area (CSA), the Boston Carpal Tunnel Questionnaire (BCTQ), and the Disabilities of the Arm, Shoulder and Hand (DASH) questionnaire were assessed before treatment and 4 weeks after injection. Patients with SjD additionally underwent comprehensive clinical, laboratory, neurophysiological, and patient-reported outcome assessments. Patients with SjD appeared to demonstrate a significantly greater reduction in median nerve CSA following corticosteroid injection compared with controls (mean reduction: 0.46 vs. 0.31 mm2; p = 0.006, Cohen’s d = 0.96, CI90% [0.51, 1.49]). Despite this favorable biological response, clinical improvement was significantly smaller in the SjD group. Patients without SjD exhibited greater improvement in both the BCTQ Symptom Severity Scale (p = 0.021) and Functional Status Scale (p = 0.033), whereas changes in DASH scores did not differ significantly between groups. Patients with concomitant peripheral neuropathies experienced significantly poorer BCTQ improvement than those with isolated CTS, suggesting that neurological comorbidity may contribute to treatment resistance. No significant associations were identified between treatment response and fibromyalgia status or ESSPRI domains. Among quality-of-life measures, only the SF-36 General Health domain differed significantly between neuropathy types. Patients with SjD and CTS exhibit a dissociation between structural improvement of the median nerve and subjective symptom relief after corticosteroid injection. These findings suggest that mechanisms beyond local nerve compression, including coexisting peripheral neuropathy and potentially altered pain processing, may contribute to persistent symptoms. Comprehensive neurological assessment and multidimensional outcome evaluation may improve identification of SjD patients at risk of suboptimal response to conventional CTS therapies.