Epidemiological profile and north-south gradient driving baseline systemic involvement of primary Sjögren's syndrome

OBJECTIVE To characterize the systemic phenotype of primary Sjögren’s syndrome at diagnosis by analysing the EULAR-SS disease activity index (ESSDAI) scores. METHODS The Sjögren Big Data Consortium is an international, multicentre registry based on worldwide data-sharing cooperative merging of pre-existing databases from leading centres in clinical research in Sjögren’s syndrome from the five continents. RESULTS The cohort included 10 007 patients (9352 female, mean 53 years) with recorded ESSDAI scores available. At diagnosis, the mean total ESSDAI score was 6.1; 81.8% of patients had systemic activity (ESSDAI score ≥1). Males had a higher mean ESSDAI (8.1 vs 6.0, P 65 years, P < 0.001). The highest global ESSDAI score was reported in Black/African Americans, followed by White, Asian and Hispanic patients (6.7, 6.5, 5.4 and 4.8, respectively; P < 0.001). The frequency of involvement of each systemic organ also differed between ethnic groups, with Black/African American patients showing the highest frequencies in the lymphadenopathy, articular, peripheral nervous system, CNS and biological domains, White patients in the glandular, cutaneous and muscular domains, Asian patients in the pulmonary, renal and haematological domains and Hispanic patients in the constitutional domain. Systemic activity measured by the ESSDAI, clinical ESSDAI (clinESSDAI) and disease activity states was higher in patients from southern countries (P < 0.001). CONCLUSION The systemic phenotype of primary Sjögren’s syndrome is strongly influenced by personal determinants such as age, gender, ethnicity and place of residence, which are key geoepidemiological players in driving the expression of systemic disease at diagnosis

Autoantibodies specificity in acute anterior uveitis according to the presence of the HLA-B27 allele

Purpose: To study HLA-B27circle plus and HLA-B27O patients with AAU with respect to the frequency and specificity of organ-specific and non-organ-specific autoantibodies. Methods: Fifty-seven consecutive patients with AAU were subjected to ophthalmologic and rheumatologic evaluation as well as to autoantibody determination: antinuclear antibodies (ANA), rheumatoid factor (RF), anticardiolipin, anti-smooth muscle, and anti-parietal cell antibodies, and immunoblot for antibodies to HeLa cells and to bovine iris extract. HLA-B27 was determined by a microlymphocytotoxicity assay. Statistical analysis employed chi-square test, Fisher's exact test, and McNemar test. Results: Thirty-four patients had the HLA-B27 allele (B27circle plus/AAU) and 23 did not (B27O/AAU). ANA, RF, anticardiolipin, and anti-parietal cell antibodies appeared in low frequency. No patient presented anti-smooth muscle antibody. Immunoblot showed a high frequency of antibodies to HeLa cell proteins in B27O/AAU patients with predominant reactivity at 46 kDa and 56kDa. in contrast, sera from B27circle plus/AAU patients reacted poorly against HeLa cell antigens. Immunoblot with bovine iris extract showed a significant frequency of antibodies in both groups, with a predominant response to antigens with an estimated mobility of 35, 52, and 54 kDa. Conclusion: Antibodies specific to iris antigens were equally frequent in both acute uveitis groups, whereas non-organ-specific autoantibodies, especially those to HeLa cell proteins, were far less frequent in B27circle plus/AAU than in B27O/AAU patients.Universidade Federal de São Paulo, Div Rheumatol, São Paulo, BrazilUniversidade Federal de São Paulo, Div Ophthalmol, São Paulo, BrazilUniversidade Federal de São Paulo, Div Rheumatol, São Paulo, BrazilUniversidade Federal de São Paulo, Div Ophthalmol, São Paulo, BrazilWeb of Scienc

Bilateral deep keratitis caused by systemic lupus erythematosus

We report the optical and ultrasonic biomicroscopy and confocal microscopy findings in bilateral stromal keratitis (keratoendotheliitis), a rare ocular manifestation of systemic lupus erythematosus (SLE). Examination revealed deposits with polyrefringent crystals. Topical corticosteroid produced regression of the corneal edema, but there was an increase in corneal opacity. Ultrasound biomicroscopy images confirmed the deep location of the corneal opacities, and confocal microscopy showed a disruption of the corneal stroma and crystal-like bodies.Universidade Federal de São Paulo, Dept Ophthalmol, São Paulo, BrazilUniversidade Federal de São Paulo, Dept Reumatol, São Paulo, BrazilUniv Santo Amaro, São Paulo, BrazilUniversidade Federal de São Paulo, Dept Dermatol, São Paulo, BrazilUniv Calif Davis, Dept Ophthalmol, Davis, CA 95616 USAUniversidade Federal de São Paulo, Dept Ophthalmol, São Paulo, BrazilUniversidade Federal de São Paulo, Dept Reumatol, São Paulo, BrazilUniversidade Federal de São Paulo, Dept Dermatol, São Paulo, BrazilWeb of Scienc

Effectiveness of resistance exercise in functional fitness in women with primary Sjögren’s syndrome: randomized clinical trial

Objective: The purpose of this study was to analyse the effectiveness of resistance exercise in functional fitness in women with primary Sjögren’s syndrome (pSS). Method: This is a randomized controlled clinical trial with 51 volunteers: 26 allocated to the exercise group (GEX) and 25 to the control group. The GEX underwent a supervised resistance-training programme for 16 weeks, with two sessions per week. The outcomes measured were: functional capacity (FC), by the Fullerton Functional Fitness Test; Daily Motor Activity Index (DMAI), evaluated by an actigraph; disease activity, by the ESSDAI; and quality of life, by the 36-item Short Form Health Survey (SF-36). The evaluations were performed by a blind evaluator at baseline (TØ) and after 16 weeks (T16wk). Results: In the GEX, all FC parameters demonstrated improvement, except for the upper limb flexibility test (p = 0.866): upper and lower limb strength, flexibility, aerobic capacity, and agility (all p Conclusion: The supervised resistance exercise programme did not worsen the DMAI or disease activity, demonstrating the safety of the intervention, and was effective in improving FC and quality of life in women with pSS. Registry identifier (clinical trials.gov): NCT03130062.</p