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Introduction: Insterstitial lung disease (ILD) is a frequent manifestation in patients with myositis related antibodies (MRA) with impact in the evolution. Prognostic factors have been described but we don’t have information about the behaviour of this disease in Latin Amarica (LA). Methods: Multicentric retrospective study. We included all patients with MRA and ILD, with ≥3 months of follow up, evaluated in 3 specialized centers of LA between 2016 and 2018. We analized variables associated with functional improvement ≥10% in FVC% in an univariate logistic regression model model. Results: 121 patients,79% female with a mean (SD) age of 54 (12) years:62% from Chile, 22% from Argentina and 16% from Mexico. Clinical diagnosis was dematomyositis (DM) in 6%, polimyositis in 4%, amyopathic DM in 6%, antisynthetase syndrome in 55% and IPAF in 29%. The mean (SD) of FVC% was 67 (20.6). In HRCT: 83% had NSIP, 65% OP, and 11% UIP. The most frequent antibody was Jo-1 (23%). Variables that show association with functional improvement in the univariate analysis were: mechanic hands (OR 2.3; p=0.035), ILD from the beginning of the disease (OR=0.3;p=0.013), skin involvement (OR 2.02;p=0.078), sclerodactyly (OR 0.23;p=0.06), Jo-1 (OR 2.57,p Conclusion: This study shows variables associated with functional improvement in patients with ILD and MRA. Prospective studies are necessary to confirm this results.

Introduction: ILD is a common manifestation of patients with myositis related antibodies (MRA). The aim of our study is to describe the clinical and radiological features of a group of patients with ILD and MRA, and their association with the initial pulmonary function (PF). Methods: Descriptive study of a multicentric cohort of patients evaluated between 2016-2018 in 3 ILD clinics in Argentina, Chile and Mexico. Descriptive statistics, univariate and multivariate analysis were performed. Results: 184 patients presented initial ILD diagnosis or simultaneous with connective tissue disease (CTD). The majority were women, with a mean age of 57±12 years. Anti-Synthetase (AS) antibodies were the most frequent (Jo-1, PL-12, PL-7). Main CTD diagnoses were AS syndrome and ILD with autoimmune features (IPAF). Main extra-thoracic symptoms, more frequent HRCT patterns and PFTs are described in Table 1. Worse PF was defined as FVC Conclusions: AS antibodies, NSIP and NSIP/OP patterns were the most frequent data, as reported in other cohorts. Worse PF could be related to the absence of extra-thoracic symptoms and “classic” antibodies of CTD, causing a delay in ILD diagnosis.

Pulmonary fibrosis is a progressive disease with a bad prognosis. This situation makes rise lung transplant as a therapeutic option among carefully selected patients. Objective: Evaluate the results and survival rates of patients with pulmonary fibrosis that were transplanted through an 8 years period of follow-up, from the beginning of our transplant program. Methods: Descriptive study of the transplanted patients diagnosed with pulmonary fibrosis from august 2010 to july 2018. Results: Out of 76 transplants, 68.4% were due to pulmonary fibrosis, among these, the main diagnosis was idiopathic pulmonary fibrosis (75%). The average lung allocation score (LAS) was 53 and 32% of them had urgency criteria. Patients’ age averaged 55 years-old and 98% of them underwent a single lung transplant. Early medical complications were seen in 26 patients with infectious episodes and 6 with acute rejection. The main late complication was chronic allograft dysfunction. The main surgical complication was bronchial stenosis (7.6%). In comparison to its base line reference values FVC means pre transplant and 1 and 3 years post-transplant were 49%, 70% and 71% respectively. A 40% of patients died during follow up period. Infections were the main cause of mortality during the first year. Survival rates at 1st 3rd and 5th year were 86,2%; 65.2% and 59.8% respectively. Conclusions: Single lung transplant is a therapeutic option for patients with interstitial lung disease with a 59% survival rate in 5 years, 1/3 fulfilled urgency criteria at the transplant time. The infections were the main early complication and chronic graft dysfunction was the main late complication.