C47-08 Characteristics of Patients With Myositis Specific and Myositis Associated Antibodies in Lung Transplantation

Abstract Rationale Lung transplantation (LTx) can extend life for patients with myositis interstitial lung disease (myositis-ILD). Studies have shown that post-transplant mortality in this population is comparable to that observed in recipients with idiopathic pulmonary fibrosis (IPF). We aim to describe clinical characteristics of patients with myositis specific and myositis associated antibodies (MSA/MAA) who receive LTx. Methods We conducted a single-center, retrospective study of patients who underwent LTx between 2010-2025 using electronic health records in the context of a positive MSA/MAA including Jo1, PL7, Pl12, EJ, OJ, MDA5, Mi2, SRP, TIF1, NXP2, SAE, PM-Scl, RNP, Ku, SSA, SSB, Ro52, Ro60, and TH/TO. Demographic variables including age, sex, body mass index (BMI), smoking history, and race were evaluated. Diagnosis and outcome including mortality was also assessed. Results We identified 18 patients with positive MSA/MAA who underwent LTx. The most prevalent antibody present was RNP (16/18), followed by SSA52 (14/18), SSA60 (7/18), Jo-1 (5/18), SRP (1/18), and MDA-5 (1/18). There was a total of 827 patients at our center who underwent LTx from 2010-2025, and patients with MSA/MAA account for 2.2% of patients who were transplanted. Of these patients transplanted, 61.1% were male, and 38.9% were female. Average age at transplantation was 61.3 years, and average BMI was 25.0 kg/ m². There were 44.4% of patients who had never smoked, 44.4% were former smokers, and 11.1% lacked documentation. Racial distribution included White (61.1%), followed by Hispanic (27.8%), and Black (11.1%). The most prevalent diagnosis was ILD (66.7%), followed by pulmonary arterial hypertension (PAH) (11.1%), idiopathic pulmonary fibrosis (IPF) (11.1%), chronic obstructive pulmonary disease (COPD) (5.6%), sarcoidosis (5.6%), and acute respiratory distress syndrome (ARDS) (5.6%). All-cause mortality was 11.1%. The cause of mortality in both patients was respiratory failure, one in the setting of antibody mediated rejection (AMR) and other with enlarging aortic aneurysm. Conclusion Clinicians should be aware of the characteristics and outcomes in patients with MSA/MAA who undergo LTx. While the anti-synthetase and MDA-5 antibodies have the highest risk for ILD, they accounted for only 6 out of 18 patients undergoing LTx. Our cohort of patients had mortality comparable to prior studies of patients with IPF. By identifying risk factors in these patients, we can prevent post-transplant complications that may occur. Patients with MSA/MAA who undergo LTx constitute a small proportion of patients undergoing LTx, and further research with multi-center cohorts is needed to further identify their defining characteristics for better risk stratification. This abstract is funded by: None