Background Systemic sclerosis (SS) is an autoimmune disease with generalized vascular dysfunction and a myriad of clinical and laboratory manifestations. Spreading of thickening of the skin, serological characteristics and the pattern of compromise of the internal organs help to classify them as diffuse scleroderma, localized sclerosis and systemic sinus scleroderma. Reports of Latin American cohorts published to date are scarce.
Objectives To describe the clinical and serological data for a single center cohort and to compare those with national and international cohorts.
Methods Descriptive, observational, cross-sectional study. We analyzed our SS database. Patients were evaluated since 01/15 to 05/2016 and fulfilled SS classification criteria (ACR 1980/ACR-EULAR 2013).Patients were classified according to Le Roy criteria in Limited Systemic Sclerosis (lSS), Diffuse Systemic Sclerosis (dSS), Systemic Sclerosis sine scleroderma (SsnS) and pre-systemic sclerosis (pSS). Organic compromise was defined as follows: Gastrointestinal (GI): esophagic dismotility by manometry or esophagitis by endoscopy; Lung: Pulmonary hypertension (PH): PSAP>25 mmHg by right heart catheterization; Intersticial Lung Disease (ILD): compatible HRCT or <70% predicted FVC or<80% predicted DLCO; Heart: Left ventricle dysfunction without PH or arterial hypertension, pericardial effusion; Digital ulcers and pitting scars; History of sclerodermic renal crisis (RSC); calcinosis. Anti-Scl-70 was determined by ELISA and anti-Centromere (ACA) by IIF.
Results 123 patients were included.74% with lSS,24% dSS and 2% SsnS; age at diagnosis (years): 49.7 (18–79 DS: 12.27) and 48.7 (27–79 DS: 13.01) in lSS and dSS respectively. Raynaud's phenomenon previous to diagnosis (years) 7,7 (0–54 DS: 12.81) in lSS and 4.03 (0–22 DS: 5.5) in dSS. 49.5% lSS were ACA positive vs 17% in dSS as well also 41,4% had Scl-70 positive (p<0.05). Lung:58.6% of dSS presented ILD and 3,4% RSC, according to published reports. No statistically significant differences were found about the presence of calcinosis, digital tip ulcers or PH between dSS and lSS. GI: was studied in only 39/123 patients with 84,6% affected among those.
Conclusions Our data showed similar results to the local and european cohorts. We found fewer calcinosis and digital tip ulcers in both lSS and dSS than international cohorts. A lower prevalence of PH was reported in lSS respect to other series, and it might be atributed to the method wich it was measured. (right heart catheterism).
Disclosure of Interest None declared