SAT0334 PERICARDIAL INVOLVEMENT IN SYSTEMIC SCLEROSIS
Authors: P. Morán Álvarez, C. De la Puente Bujidos
Journal: Annals of the Rheumatic Diseases (2020)

Background: Systemic sclerosis (SSc) is an autoimmune rheumatic disease characterized by visceral and skin fibrosis, vascular dysfunction, and immune dysregulation. Regarding cardiac manifestations, pericardial disorder is one of the most frequent but often asymptomatic. Objectives: To analyze clinical manifestations, diagnostic tools and treatments of a patient cohort with SSc and pericardial involvement associated. Methods: A descriptive, observational, cross-sectional study was carried out. We included all patients between 1975 and 2019 with diagnosis of SSc. Demographic, clinical and analytical data; imaging tests; treatments; and mortality rate were collected. Results: 158 patients were included, 142 (89.9%) women and 16 (10.1%) men. 144 (91.1%) were Caucasians. Mean age at diagnosis was 57 years (range: 17-86). Type of scleroderma, clinical manifestations and autoimmunity profile were shown in table 1 and 2, respectively. Table 1. Clinical manifestations Clinical manifestations n (%) Cutaneous - Limited 104 (66.5%) - Diffuse 40 (25.3%) - Sine 13 (8.2%) Raynaud’s phenomenon 156 (98.7%) Digital tip ulcers 63 (39.9%) Telangiectasia 111 (70.3%) Calcinosis 29 (18.4%) Tendon friction rubs 3 (1.9%) Joint involvement 44 (27.6%) Musle involvement 13 (8.2%) Interstitial Lung Disease 46 (28.9%) Gastrointestinal 100 (62.9%) Pulmonary hypertension 22 (13.8%) Renal 1 (0.6%) 15 (9.4%) patients developed a pericardial disorder, 3 of them (1.8%) an acute pericarditis. In the majority of the patients (11, 73.3%) pericardial involvement appeared later than diagnosis of SSc with a mean time of 8 years (maximum period of time: 20 years), and the remaining 4 (2.5%) presented the pericardial involvement prior to SSc onset (up to 4 years before). Regarding to cutaneous involvement, limited SSc was the most frequent (13 patients, 86.6%), followed by diffuse SSc (3 patients, 13.4%). In relation to antibody profile, 11 (73.3%) patients were AC positive and 4 (26.6%) AT positive. Seven (46.6%) of the 15 patients with pericardial involvement had a prior pulmonary hypertension associated. Eleven (73.3%) were asymptomatic, 4 (26.6%) referred chest pain and 4 (26.6%) dyspnea. 100% of the pericardial effusions were detected through the performance of an echocardiogram, 3 of them (13.4%) with signs of diastolic dysfunction. Regarding to electrocardiographical findings, 2 (13.3%) showed low voltages. The size of the pericardial effusion was described as: mild in 9 (60%) patients, moderate in 5 (33.3%) and severe in 1 (6.7%). One Cardiac- Magnetic Resonance Imaging was performed which showed constriction data. None of the patients suffered cardiac tamponade or hemodynamic compromise, due to invasive therapies were not necessary. A spontaneous resolution of the pericardial effusion was registered in 6 (40%) patients, with a mean time of 8 months (range: 2-36 months); and 1 (6.7%) patient after an increment in immunosuppressive dose, without improvement of pulmonary hypertension. No renal crisis or deaths were recorded in these patients. Conclusion: In our cohort patient with SSc, 9.5% developed a pericardial involvement. This kind of disorder could be the first manifestation of the disease. Pulmonary hypertension is the most frequent underlying pathway, however serositis must also be taken into account. Most part of the pericardial effusions were mild. No hemodynamic compromise was registered, due to an invasive treatment was not required. Table 2. Antibody profile Antibodies n (%) Anticentromere (AC) + 92 (58.2%) Anti- topoisomerase I (AT) + 36 (22.8%) Antinuclear (ANA) + 155 (97.5 %) References: [1]Fernández Morales A et al. Cardiac tamponade and severe pericardial effusion in systemic sclerosis: report of nine patients and review of the literature. Int J Rheum Dis. 2017 Oct [2]Hosoya H, et al. Clinically Symptomatic Pericardial Effusions in Hospitalized Systemic Sclerosis Patients: Demographics and Management. Biomed Res Int. 2018 Jun 4 Disclosure of Interests: None declared