AB0674 RETROSPECTIVE STUDY OF A COHORT OF PATIENTS WITH SYSTEMIC SCLEROSIS IN A TERTIARY CARE HOSPITAL
Authors: David Moyano Bueno, Luis Gómez-Lechón, Olga Martínez González, Ana Isabel Turrión, Carlos a. Montilla-Morales, Cristina Hidalgo
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background:</h3> Systemic sclerosis (SS) is an autoimmune disease of unknown etiology, characterized by the presence of fibrosis and vasculopathy in skin and multiple internal organs such as the lungs, the kidneys and the digestive tract. The course of the disease is unpredictable and could remain relatively stable or have a rapid evolution. Multiple studies have been carried out to determine the clinical characteristics and survival prognosis on SS patients. <h3>Objectives:</h3> To analyze the demographic characteristics, clinical features, treatment and prognosis in a SS disease cohort. <h3>Methods:</h3> We performed an observational and retrospective study of patients whit SS. The patients had been attended by the Rheumatology department of a tertiary care hospital. We collected demographic, clinical and analytical variables, as well as treatment and prognosis. We classified the disease employing the LeRoy and Medsger, VEDOSS criteria and 2013 aCR/EULAR criteria. <h3>Results:</h3> Of our 43 patients, 36 (83.7%) were female and only 7 (16.3%) were male. The average age was 60.4 years (SD 15.6), the average age at diagnosis was 53.3 years (SD 17.6) and the mean time of evolution of the disease was 7.9 years (SD 6.3). Of all the patients, 3 patients (6.9%) died, the mean age at death being 53.6 years (SD 23.7) and the mean time from diagnosis to death of 19 years (SD 10.1). The most frequently ocurring presentation was limited SS (with 18 patients (41.9%), followed by preescleroderma with 14 patients (32.6%), diffuse SS with 6 patients (14%) and escleroderma sine escleroderma with 2 patients (4.7%). Three patients (7%) were labeled with MCTD. SS was associated with other autoimmune diseases in 20% of patients. Five (11.6%) patients developed neoplasms throughout the course of the study. The rest of the clinical characteristics are listed in Table 1 and 2, as well as the strength of association of these with the type of SS, calculated using chi square and Fischer test. <h3>Conclusion:</h3> Worthy of noting in our cohort is the absence of scleroderma in more than 40% of our patients, probably because the new criteria have allowed us to diagnose the disease at an earlier stage and also due to the scarce frequency of puffy fingers with respect to other larger series. Digestive involvement was the most frequent visceral manifestation, followed by pulmonary manifestations, specifically interstitial lung disease (ILD). Despite the small sample size, lung disease was significantly associated with the two forms of systemic sclerosis, but not with the antibody pattern. Both the ILD and the pulmonary arterial hypertension (PAH) were more frequent in patients with SSd. Mortality in all cases was due to interstitial lung involvement. As is frequently described, SS is associated with other systemic autoimmune diseases, constituting an overlap syndrome. <h3>References</h3> [1] Cañete Crespillo, J. (2008). Manual SER de las enfermedades reumáticas. Madrid: Editorial Médica Panamericana [2] Dougherty, D., Kwakkenbos, L., Carrier, M., Salazar, G., assassi, S., Baron, M., Bartlett, S., Furst, D., Gottesman, K., van den Hoogen, F., Malcarne, V., Mouthon, L., Nielson, W., Poiraudeau, S., Sauvé, M., Boire, G., Bruns, a., Chung, L., Denton, C., Dunne, J., fortin, P., Frech, T., Gill, a., Gordon, J., Herrick, a., Hinchcliff, M., Hudson, M., Johnson, S., Jones, N., Kafaja, S., Larché, M., Manning, J., Pope, J., Spiera, R., Steen, V., Sutton, E., Thorne, C., Wilcox, P., Thombs, B. And Mayes, M. (2018). The Scleroderma Patient-Centered intervention Network Cohort: baseline clinical features and comparison with other large scleroderma cohorts. Rheumatology, 57(9), pp.1623-1631. [3] CLINICAL FEATURES aND PROGNOSTIC FACTORS for SYSTEMIC SCLEROSIS RELATED inTERSTITIAL LUNG DISEASE. (2018). Respirology, 23, pp.155-155. <h3>Disclosure of interests:</h3> None declared