AB0591 ANALYSIS OF A COHORT OF PATIENTS WITH SYSTEMIC SCLEROSIS AND INTERSTITIAL LUNG DISEASE
Authors: L. Mendez Diaz, R. J. Gil Velez, I. Madroñal García
Journal: Annals of the Rheumatic Diseases (2020)

Background: Interstitial lung disease (ILD) is a common manifestation of systemic sclerosis that is associated with early mortality. The development or progression of ILD can occur at any time, so patients should be monitored regularly, particularly in the first years after diagnosis. Treatment should be considered when the disease is clinically significant, particularly when there is evidence of progression based on a decrease in lung function, progression of fibrosis on the HRCT or worsening of respiratory symptoms. Objectives: -To relate the type of systemic sclerosis (SS) with pulmonary involvement with the radiological pattern. -To study if there is a relationship between the antibodies and the aforementioned affectation. Methods: Retrospective descriptive study of patients treated in our Hospital (2009-2019) by the Rheumatology and Internal Medicine department diagnosed with systemic sclerosis and interstitial lung disease. The data were obtained through the review of medical records. We have included data from patients who have a diagnosis of limited or diffuse systemic sclerosis or overlap with interstitial lung involvement. Results: Of the 213 patients with systemic sclerosis in our database 43 had interstitial lung involvement (20.2%). 79% of the patients with ILD (34) had a non-specific interstitial pneumonia type (NSIP) radiological pattern and 21% of the patients (9) had a pattern of usual interstitial pneumonia (UIP) Among the patients with ILD with a NSIP -type radiological pattern, 19 patients were diagnosed with diffuse SS, 9 patients with overlap syndrome and 6 with limited SS. Of the patients with ILD with radiological pattern type UIP, 5 patients were diagnosed with diffuse SS, 3 patients with overlap syndrome and 1 patient was diagnosed with limited SS. TABLE 1. RELATIONSHIP BETWEEN SS TYPE AND RADIOLOGICAL PATTERN NSIP UIP LIMITED SS 6 (17.6%) 1 (11.1%) DIFFUSE SS 19 (55.8%) 5 (55.5%) OVERLAP 9 (26.4%) 3 (33.3%) Among the patients with the NSIP pattern, 17 had positive SCL70 antibody, 3 positive ANA patients and 1 patient had positive anti-centromere antibody. Of the patients with UIP type interstitial pneumopathy, 8 patients had anti-SCL70 antibody, 3 patients ANA positive antibody and 2 patients anti-centromere positive antibody. TABLE 2. RELATIONSHIP BETWEEN TYPE OF AB AND RADIOLOGICAL PATTERN NSIP UIP Anti SCL70 17 8 Anti centromere 1 2 ANA 3 3 Regarding treatment, 21 patients were taking Mycophenolate, 16 patients required cyclophosphamide and 6 patients rituximab. No patient in our cohort died due to interstitial lung disease. Conclusion: The data obtained are consistent with what is collected in the medical literature. The subtype of scleroderma most related to ILD was diffuse SS. The most frequent antibody was anti-SCL 70. Regarding the treatment,the most used in ILD in our center was the mycophenolate. From our sample analyzed when applying the likelihood ratio (RV) a value of 47,186 is obtained, which has an associated probability of 0, which is less than 0.05, leads to reject the null hypothesis (there is no dependence between antibodies and type of radiological pattern of ILD in SS), concluding that there is dependence between the analyzed variables. After this analysis, we can conclude that in our sample there is a relationship between the type of interstitial pneumopathy pattern and the antibody present in patients with SS. Disclosure of Interests: None declared