OBJECTIVE: To evaluate systemic involvement in primary SS in a large cohort of Spanish patients using the EULAR-SS disease activity index (ESSDAI) definitions. METHODS: Systemic involvement was characterized using ESSDAI definitions for the 10 clinical domains (constitutional, lymphadenopathy, glandular, articular, cutaneous, pulmonary, renal, peripheral nervous system, central nervous system and muscular). ESSDAI scores at diagnosis, during follow-up and cumulated at the last visit were calculated. RESULTS: The cohort consisted of 921 patients. After a mean follow-up of 75 months, 77 (8%) patients still had an ESSDAI score of zero at the last visit. Organ by organ, the percentage of patients who developed activity during the follow-up (ESSDAI score ≥ 1 at any time) ranged between 1.4% and 56%, with articular, pulmonary and peripheral neurological involvement being the most common. Logistic multivariate regression analysis showed the following features at diagnosis and had the closest association with systemic activity (statistically significant independent variables in at least two domains): cryoglobulinaemia in five domains; anaemia, lymphopenia and low C3 levels in three domains each and age <35 years in two domains. Sicca features, ANA and RF at diagnosis were not associated with a higher cumulated activity score in any clinical domain. CONCLUSION: Primary SS is undeniably a systemic disease, with the joints, lungs, skin and peripheral nerves being the most frequently involved organs. Cytopenias, hypocomplementaemia and cryoglobulinaemia at diagnosis strongly correlated with higher cumulated ESSDAI scores in the clinical domains. Clinically the ESSDAI provides a reliable picture of systemic involvement in primary SS.
OBJECTIVE: To evaluate systemic involvement in primary SS in a large cohort of Spanish patients using the EULAR-SS disease activity index (ESSDAI) definitions. METHODS: Systemic involvement was characterized using ESSDAI definitions for the 10 clinical domains (constitutional, lymphadenopathy, glandular, articular, cutaneous, pulmonary, renal, peripheral nervous system, central nervous system and muscular). ESSDAI scores at diagnosis, during follow-up and cumulated at the last visit were calculated. RESULTS: The cohort consisted of 921 patients. After a mean follow-up of 75 months, 77 (8%) patients still had an ESSDAI score of zero at the last visit. Organ by organ, the percentage of patients who developed activity during the follow-up (ESSDAI score ≥ 1 at any time) ranged between 1.4% and 56%, with articular, pulmonary and peripheral neurological involvement being the most common. Logistic multivariate regression analysis showed the following features at diagnosis and had the closest association with systemic activity (statistically significant independent variables in at least two domains): cryoglobulinaemia in five domains; anaemia, lymphopenia and low C3 levels in three domains each and age <35 years in two domains. Sicca features, ANA and RF at diagnosis were not associated with a higher cumulated activity score in any clinical domain. CONCLUSION: Primary SS is undeniably a systemic disease, with the joints, lungs, skin and peripheral nerves being the most frequently involved organs. Cytopenias, hypocomplementaemia and cryoglobulinaemia at diagnosis strongly correlated with higher cumulated ESSDAI scores in the clinical domains. Clinically the ESSDAI provides a reliable picture of systemic involvement in primary SS.
Authors: Anastasia Secco; Lucila Marino; Natalia Herscovich; Pedro Aicardi; Lorena Techera; Lorena Takashima; María Lida Santiago; Felix Romanini; Marta Mamani; Antonio C Catalán Pellet Journal: Eur J Rheumatol Date: 2019-12-16
Authors: Clare Oni; Sheryl Mitchell; Katherine James; Wan-Fai Ng; Bridget Griffiths; Victoria Hindmarsh; Elizabeth Price; Colin T Pease; Paul Emery; Peter Lanyon; Adrian Jones; Michele Bombardieri; Nurhan Sutcliffe; Costantino Pitzalis; John Hunter; Monica Gupta; John McLaren; Annie Cooper; Marian Regan; Ian Giles; David Isenberg; Vadivelu Saravanan; David Coady; Bhaskar Dasgupta; Neil McHugh; Steven Young-Min; Robert Moots; Nagui Gendi; Mohammed Akil; Francesca Barone; Ben Fisher; Saaeha Rauz; Andrea Richards; Simon J Bowman Journal: Rheumatology (Oxford) Date: 2015-10-27 Impact factor: 7.580
Authors: J Demarchi; S Papasidero; M A Medina; D Klajn; R Chaparro Del Moral; O Rillo; V Martiré; G Crespo; A Secco; A Catalan Pellet; C Amitrano; C Crow; C Asnal; P Pucci; F Caeiro; N Benzanquen; J P Pirola; M Mayer; F Zazzetti; S Velez; J Barreira; N Tamborenea; L Santiago; L Raiti Journal: Clin Rheumatol Date: 2017-09-14 Impact factor: 2.980