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

Characterization and outcomes of 414 patients with primary SS who developed haematological malignancies

Objective: To characterize 414 patients with primary SS who developed haematological malignancies and to analyse how the main SS- and lymphoma-related features can modify the presentation patterns and outcomes. Methods: By January 2021, the Big Data Sjögren Project Consortium database included 11 966 patients fulfilling the 2002/2016 classification criteria. Haematological malignancies diagnosed according to the World Health Organization (WHO) classification were retrospectively identified. Results: There were 414 patients (355 women, mean age 57 years) with haematological malignancies (in 43, malignancy preceded at least one year the SS diagnosis). A total of 376 (91%) patients had mature B-cell malignancy, nearly half had extranodal marginal zone lymphoma (MZL) of mucosa-associated lymphoid tissue (MALT lymphoma) (n = 197), followed by diffuse large B-cell lymphoma (DLBCL) (n = 67), nodal MZL lymphoma (n = 29), chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) (n = 19) and follicular lymphoma (FL) (n = 17). Rates of complete response, relapses and death were 80%, 34% and 13%, respectively, with a 5-year survival rate of 86.5% after a mean follow-up of 8 years. There were significant differences in age at diagnosis (younger in MALT, older in CLL/SLL), predominant clinical presentation (glandular enlargement in MALT lymphoma, peripheral lymphadenopathy in nodal MZL and FL, constitutional symptoms in DLBCL, incidental diagnosis in CLL/SLL), therapeutic response (higher in MALT lymphoma, lower in DLBCL) and survival (better in MALT, nodal MZL and FL, worse in DLBCL). Conclusion: In the largest reported study of haematological malignancies complicating primary SS, we confirm the overwhelming predominance of B-cell lymphomas, especially MALT, with the salivary glands being the primary site of involvement. This highly-specific histopathological scenario is linked with the overall good prognosis with a 5-year survival rate of nearly 90%

ULTRASONOGRAPHY OF MAJOR SALIVARY GLANDS IN JUVENILE SJOGREN'S SYNDROME - PRELIMINARY FINDINGS IN A MULTI-CENTER STUDY

Haukeland Hosp, Dept Rheumatol, Bergen, NorwayUniv Fed Espirito Santo, Dept Rheumatol, Med Clin, Vitoria, BrazilUniv Fed Rio de Janeiro, Dept Rheumatol, Rio De Janeiro, BrazilUniv Sao Paulo, Hosp Clin HCFMUSP, Sjogrens Syndrome Outpatient, Sao Paulo, BrazilOslo Univ Hosp, Dept Rheumatol, Oslo, NorwayHosp Gen Univ Gregorio Maranon, Dept Rheumatol, Madrid, SpainUniv Fed Sao Paulo, Dept Pediat Rheumatol, Sao Paulo, BrazilUniv Med Ctr Groningen, Dept Rheumatol & Clin Immunol, Groningen, NetherlandsUniv Sao Paulo, Sch Med, Div Rheumatol, Sao Paulo, BrazilUniv Groningen, Univ Med Ctr Groningen, Dept Rheumatol & Clin Immunol, Groningen, NetherlandsUniv Bergen, Dept Clin Sci, Rheumatol Sect, Bergen, NorwayUniv Bergen, Dept Clin Sci, Broegelmann Res Lab, Bergen, NorwayUniv Bergen, Sect Oral & Maxillofacial Radiol, Dept Clin Dent, Bergen, NorwayUniv Fed Sao Paulo, Dept Pediat Rheumatol, Sao Paulo, BrazilWeb of Scienc