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Hospitalization Risks for Neurological Disorders in Primary Sjogren's Syndrome Patients.

ABSTRACT: Primary Sjögren's syndrome (pSS) can be associated with neurological and cognitive involvement, negatively affecting patients' quality of life. The aim of this study was to assess whether pSS patients are at higher risk of hospitalization for neurological diseases. Through a nationwide retrospective study using the French Health insurance database (based on International Classification for Disease codes, ICD-10), we selected patients hospitalized with new-onset pSS between 2011 and 2018. We compared the incidence of hospitalization for dementia, multiple sclerosis (MS), encephalitis, and peripheral neuropathy with an age- and sex-matched (1:10) hospitalized control group. Adjusted Hazard Ratios (aHR) considered confounding factors, particularly socio-economic status and cardiovascular diseases. We analyzed 25,661 patients hospitalized for pSS, compared with 252,543 matched patients. The incidence of hospitalization for dementia was significantly higher in pSS patients (aHR = 1.27 (1.04-1.55); p = 0.018), as well as the incidence of hospitalization for MS, encephalitis, and inflammatory polyneuropathies (aHR = 3.66 (2.35-5.68), p < 0.001; aHR = 2.66 (1.22-5.80), p = 0.014; and aHR = 23.2 (12.2-44.5), p < 0.001, respectively). According to ICD-10 codes, pSS patients exhibited a higher incidence of hospitalization for dementia, encephalitis, MS, and peripheral neuropathies than controls. Physicians must be aware of these neurological risks to choose the most appropriate diagnostic work-up.

SUBMITTER: Goulabchand R

PROVIDER: S-EPMC9000167 | biostudies-literature | 2022 Apr

REPOSITORIES: biostudies-literature

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Hospitalization Risks for Neurological Disorders in Primary Sjögren's Syndrome Patients.

Goulabchand Radjiv R Gabelle Audrey A Ayrignac Xavier X Malafaye Nicolas N Labauge Pierre P Noël Danièle D Morel Jacques J Roubille Camille C Barateau Lucie L Guilpain Philippe P Mura Thibault T

Journal of clinical medicine 20220401 7

Primary Sjögren’s syndrome (pSS) can be associated with neurological and cognitive involvement, negatively affecting patients’ quality of life. The aim of this study was to assess whether pSS patients are at higher risk of hospitalization for neurological diseases. Through a nationwide retrospective study using the French Health insurance database (based on International Classification for Disease codes, ICD-10), we selected patients hospitalized with new-onset pSS between 2011 and 2018. We comp ...[more]

PMID: 35407586

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Project description:Opinion statement: Purpose of review:Neurologic disease is a common extraglandular manifestation of Sjögren's syndrome (SS), the study of which has been hampered both by the lack of uniform definitions for specific neurologic complications and by the imprecision of the tools used to diagnose SS. There is a great need to develop consensus criteria for classifying these varied neurologic manifestations, as has been done in systemic lupus erythematosus (SLE) "Arthritis and rheumatism 42:599-608, 1999". SS patients with certain forms of neurologic involvement, such as small fiber neuropathy and sensory ataxic ganglionopathy, frequently lack anti-SSA and anti-SSB antibodies and other serologic abnormalities. In these patients, neurologic disease is often their presenting manifestation, triggering a search for underlying SS. Given the frequent seronegativity of such patients, their diagnosis of SS rests heavily on the interpretation of a labial gland biopsy. However, these biopsies are prone to misinterpretation "Vivino etal. J Rheumatol 29:938-44, 2002", and "positive" ones are found in up to 15% of healthy volunteers "Radfar et al. Arthrit Rheumatu 47:520-4, 2002". Better diagnostic tools are needed to determine if the frequent seronegative status of these SS patients may be related to a unique disease pathogenesis. Recent findings:Recent advances in diagnostic techniques have served to define a likely pathogenetic basis for certain neurologic manifestations of SS. The advent of punch skin biopsies to analyze intraepidermal nerve fiber density and morphology has helped define pure sensory small fiber neuropathy as common in SS and the basis for both length- and non-length-dependent patterns of neuropathic pain. New protocols for magnetic resonance imaging (MRI) have enabled the recognition of dorsal root ganglionitis, a finding originally detected in pathologic studies. The advent of the anti-aquaporin-4 (AQP4) antibody test in 2004 has Led to the appreciation that demyelinating disease in SS is often related to the presence of neuromyelitis optica spectrum disorder. The anti-AQP4 antibody is considered to be directly pathogenic in the brain, targeting the primary water channel proteins in the brain, expressed prominently on astrocytic foot processes. Summary:There are no clinical trials evaluating the efficacy of systemic immune suppressive therapy for peripheral or central nervous system involvement. With the recent increase in clinical trials of biologic agents for SS, which utilize systemic disease manifestations as standardized outcome measures, there is an urgency to deveLop appropriate definitions of neuroLogic compLications of SS and cLear parameters for clinical improvement.

Dataset Information

Hospitalization Risks for Neurological Disorders in Primary Sjogren's Syndrome Patients.

Publications

Hospitalization Risks for Neurological Disorders in Primary Sjögren's Syndrome Patients.

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