Sjögren syndrome

Background
Sjogren’s syndrome is one of the more common inflammatory rheumatological diseases, with a prevalence of at least 0.4% in Germany.

The Diagnosis and Treatment of Sjögren's Syndrome.

Sjogren's syndrome (SjS) is a systemic autoimmune disease that mainly affects the exocrine glands, leading to generalized mucosal dryness. However, primary SjS may initially present with non-sicca (systemic) manifestations. When these features appear before the onset of an overt sicca syndrome, we may talk of an underlying 'occult' SjS. The European League Against Rheumatism (EULAR) has promoted and supported an international collaborative study group (EULAR-SS Task Force) aimed at developing consensual recommendations to provide a homogeneous approach to the patient with primary SjS presenting with systemic involvement. This review summarizes the key factors that should be taken into account in the diagnostic approach in a patient with suspected SjS according to the main clinical patterns of presentation, and is especially focused on organ-specific systemic disease presentations, including a consensus set of recommendations in order to reach an early diagnosis. Close collaboration with the different specialties involved through a comprehensive multidisciplinary approach is essential in SjS patients presenting with systemic involvements.

Early diagnosis of primary Sjögren’s syndrome: EULAR-SS task force clinical recommendations

Objectives Neurological manifestations seem common in primary Sjogren9s 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 Sjogren9s syndrome (ASSESS) cohort. Methods The 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. Results Data 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 Conclusions Prevalences 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.

https://rmdopen.bmj.com/content/rmdopen/2/1/e000179.full.pdf

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

To identify the principal determinants of health-related quality-of-life [HR-QoL] impairment in patients with active primary Sjogren's syndrome [pSS] participating in a large therapeutic trial [TEARS]. At the inclusion visit for the TEARS trial, 120 patients with active pSS completed the Short Form survey 36 [SF36], a validated HR-QoL assessment tool. Univariate then multivariate linear regression analyses were used to assess associations linking SF36 physical and mental components to demographic data, patient-reported outcomes [symptom intensity assessments for dryness, pain and fatigue, including the EULAR SS Patient Reported Index (ESSPRI)], objective measures of dryness and autoimmunity, and physician evaluation of systemic activity [using the EULAR SS Disease Activity Index (ESSDAI)]. SF36 scores indicated marked HR-QoL impairments in our population with active pSS. Approximately 1/3 of the patients had low, moderate and high systemic activity according to the ESSDAI. ESSPRI and ESSDAI scores were moderately but significantly correlated. The factors most strongly associated with HR-QoL impairment were patient-reported symptoms, best assessed using the ESSPRI, with pain and ocular dryness intensity showing independent associations with HR-QoL. Conversely, systemic activity level was not associated with HR-QoL impairment in multivariate analyses, even in the patient subset with ESSDAI values indicating moderate-to-high systemic activity. The cardinal symptoms of pSS (dryness, pain and fatigue, best assessed using the ESSPRI) are stronger predictors of HR-QoL impairment than systemic involvement (assessed by the ESSDAI), and should be used as endpoints in future therapeutic trials focusing on patients' well-being. New consensual and data-driven response criteria are needed for pSS studies. This article is protected by copyright. All rights reserved.

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

The EULAR Sjogren's syndrome (SS) disease activity index (ESSDAI) is a systemic disease activity index that was designed to measure disease activity in patients with primary SS. With the growing use of the ESSDAI, some domains appear to be more challenging to rate than others. The ESSDAI is now in use as a gold standard to measure disease activity in clinical studies, and as an outcome measure, even a primary outcome measure, in current randomised clinical trials. Therefore, ensuring an accurate and reproducible rating of each domain, by providing a more detailed definition of each domain, has emerged as an urgent need. The purpose of the present article is to provide a user guide for the ESSDAI. This guide provides definitions and precisions on the rating of each domain. It also includes some minor improvement of the score to integrate advance in knowledge of disease manifestations. This user guide may help clinicians to use the ESSDAI, and increase the reliability of rating and consequently of the ability to detect true changes over time. This better appraisal of ESSDAI items, along with the recent definition of disease activity levels and minimal clinically important change, will improve the assessment of patients with primary SS and facilitate the demonstration of effectiveness of treatment for patients with primary SS.

/pdf/eular-sjogren-s-syndrome-disease-activity-index-essdai-a-45hdlp3cev.pdf

Elena Bartoloni

Papers

EULAR Sjögren's syndrome disease activity index (ESSDAI): a user guide.