SAT0291 PULMONARY INVOLVEMENT AND FUNCTIONAL LIMITATION IN SYSTEMIC SCLEROSIS

Background: Pulmonary involvement is the main cause of death in Systemic Sclerosis patients (SS). However, there is little information whether its presence is capable of affecting the functional capacity of patients and if this influences the quality of life perceived by them. Objectives: To determine whether the presence of pulmonary involvement in patients with SS (Interstitial Lung Disease and or Pulmonary Arterial Hypertension) is related to greater functional disability. Methods: Observational and cross-sectional study, with a prospectively performed protocol, of patients diagnosed of SS according to ACR/EULAR 2013 criteria. Demographic, clinical, analytical, activity (EUSTAR index), severity (Medsger scale and modified Rodnan index), health perception (SF36) and disability (HAQ and Cochin test) variables were collected. Moreover, Videocapillaroscopy (VCL) and Respiratory Function Test were made. All the patients had pulmonary TCMD and echocardiography in order to describe pulmonary features. Results: 42 patients were included (95.4% women), with an average age of 59.2 (S.D 12.9) years. The median of years from diagnosis was 4, and 6 from the first not Raynaud symptom. Out of them, 20 were SS limited, 20 patients SS diffuse; and 2 patients SS sine scleroderma. Pulmonary hypertension (HTP) was found in 11.9% of patients, as well as EPID in 33.3% (UIP 16.7%, NSIP 14.3%, bronchiolitis 2.3%). Analyzing the subgroup of patients with EPID, we can highlight higher score of HAQ, Cochin and activity index EUSTAR, with less influence in Rodnan index and without differences in SF36. Regarding the subset of patients with PAH, we found higher score on HAQ, EUSTAR activity index, index Rodnan, Medsger gravity scale and the SF36, than in non-PAH patients, with no differences in Cochin test. Conclusion: We can highlight the presence of greater functional limitation that is especially reflected in rates of disability (HAQ and Cochin), as well as a increased activity of disease and a lower self-perception of health in patients with Systemic Sclerosis pulmonary involvement. Disclosure of Interests: None declared