Is there specific neurological disorders of primary Sjögren's syndrome?

Alegria, Guillermo Carvajal; Guellec, Dewi; Devauchelle‐Pensec, Valérie; Saraux, Alain

doi:10.1016/j.jbspin.2014.04.002

Cited by 21 publications

(7 citation statements)

References 23 publications

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“…The first studies of neurological manifestations in pSS were reported in the 1980s. 3 Although neurological manifestations seem common, our recent review 4 shows wide variations in reported prevalences. Nevertheless, the most recent data suggest a prevalence of about 20%.…”

Section: Introductionmentioning

confidence: 98%

Epidemiology of neurological manifestations in Sjögren's syndrome: data from the French ASSESS Cohort

et al. 2016

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ObjectivesNeurological manifestations seem common in primary Sjögren's syndrome (pSS) but their reported prevalences vary. We investigated the prevalence and epidemiology of neurological manifestations in a French nationwide multicentre prospective cohort of patients with pSS, the Assessment of Systemic Signs and Evolution in Sjögren's syndrome (ASSESS) cohort.MethodsThe ASSESS cohort, established in 2006, includes 395 patients fulfilling American–European Consensus Group criteria for pSS. Demographic and clinical data were compared between patient groups with and without neurological manifestations, and across patient groups with peripheral nervous system (PNS) manifestations, central nervous system (CNS) manifestations and no neurological manifestations.ResultsData at inclusion were available for 392 patients, whose mean age was 58±12 years. Mean follow-up was 33.9 months. Neurological manifestations were present in 74/392 (18.9%) patients, including 63 (16%) with PNS manifestations and 14 (3.6%) with CNS manifestations. Prevalences were 9.2% for pure sensory neuropathy, 5.3% for sensorimotor neuropathy, 1.3% for cerebral vasculitis and 1.0% for myelitis. Neurological manifestations were associated with greater pSS activity as assessed using the ESSDAI (9.4±6.8 vs 4.3±4.8; p<0.001) and proportion of patients taking immunomodulatory/immunosuppressive drugs (32.4% (24/74) versus 13.8% (44/318), p=0003). New neurological symptoms were more common in patients with than without prior neurological manifestations (RR=3.918 (95% CI 1.91 to 8.05); p<0.001).ConclusionsPrevalences of peripheral and central neurological manifestations in pSS are about 15% and 5%, respectively. Neurological manifestations are associated with greater pSS activity. New neurological manifestations are more common in patients with prior neurological involvement.

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Section: Introductionmentioning

confidence: 98%

Epidemiology of neurological manifestations in Sjögren's syndrome: data from the French ASSESS Cohort

et al. 2016

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“…Its main pathologic feature is a lymphocytic infiltrate of the exocrine glands (mainly salivary and lachrymal), but this inflammatory process often develops beyond these principal targets and may affect other organs, such as the joints, skin, lungs, kidneys, nerves, and small vessels. Thus, almost all patients report the same disabling cardinal symptoms associated with the glandular involvement (mouth and eyes dryness, fatigue, and diffuse musculoskeletal pain), and a subset of patients experience systemic involvement (such as polysynovitis, inflammatory lung disease, or neurologic involvement) , which can sometimes be severe and is associated with an increased risk of lymphoma development . These 2 facets of the disease have major implications for both daily patient care and the design of clinical trials.…”

Section: Introductionmentioning

confidence: 99%

Severe Health‐Related Quality of Life Impairment in Active Primary Sjögren's Syndrome and Patient‐Reported Outcomes: Data From a Large Therapeutic Trial

Cornec

Devauchelle‐Pensec

Mariette

et al. 2017

Arthritis Care & Research

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“…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.…”

Section: Sjögren's Syndrome (Ss)mentioning

confidence: 99%

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

Jin

Liu

2021

Diagnostics

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Purpose of review: To discuss and summarize recent findings in peripheral neuropathy (PN) related to connective tissue diseases (CTD) including its prevalence, clinical manifestations, pathogenesis, diagnosis and treatment. Recent findings: Although PN is a common complication in CTD and has been well studied, recent research has shown that PN is more diverse and frequent in different subtypes of CTD than was expected. The incidence of PN in Sjögren’s syndrome and rheumatoid arthritis (RA) varies according to different disease subtypes, and the pathogenesis of neuropathic pain in different subtypes of eosinophilic granulomatosis with polyangiitis (EGPA) may also differ. Neurogenic inflammation, autoantibody-mediated changes, ischemia of the vascular wall and metabolic mechanisms have been shown to contribute to the pathogenesis of PN in CTD. Moreover, allergic inflammation has been recently identified as a possible new mechanism producing peripheral neuropathic pain associated with MPO-ANCA negative EGPA patients. Glucocorticoids are routinely used to relieve pain caused by PN. However, these steroids may cause hyperalgesia, exacerbate neuropathic pain, and activate the early phase of pain induction and produce hyperalgesia. Recently, neuroactive steroids, such as progesterone, tetrahydroprogesterone and testosterone, have been shown to exert protective effects for several PN symptoms, and in particular neuropathic pain. Neuroactive steroids will be an interesting topic for future research into PN in CTD. Summary: It is essential for the diagnosis and treatment of PN in CTD to be updated. Timely diagnosis, appropriate treatments, and multidisciplinary care are essential to minimize morbidity and decrease the risk of permanent neurologic deficits. Further studies are needed to guide diagnosis and treatment.

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Is there specific neurological disorders of primary Sjögren's syndrome?

Cited by 21 publications

References 23 publications

Epidemiology of neurological manifestations in Sjögren's syndrome: data from the French ASSESS Cohort

Epidemiology of neurological manifestations in Sjögren's syndrome: data from the French ASSESS Cohort

Severe Health‐Related Quality of Life Impairment in Active Primary Sjögren's Syndrome and Patient‐Reported Outcomes: Data From a Large Therapeutic Trial

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

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