Lung Transplantation Outcomes of Patients with Interstitial Pneumonia with Autoimmune Features

Objectives Interstitial pneumonia with autoimmune features (IPAF) is a designation proposed in 2015 by the European Respiratory Society/American Thoracic Society (ERS/ATS) to describe patients who have interstitial lung disease (ILD) and combinations of clinical, serologic, and/or pulmonary morphologic features of an underlying systemic autoimmune condition, but do not meet rheumatologic criteria for a characterized connective tissue disease (CTD).[1] Previous studies have suggested that patients with IPAF have worse clinical outcomes and survival compared to those with CTD-associated ILD, but better than those with idiopathic pulmonary fibrosis (IPF).[2] Survival after lung transplantation is similar between CTD-associated ILD and IPF.[3] Limited data are available regarding post-lung transplant outcomes in patients with IPAF. The objective of our study is to compare post-transplant survival, lung function, and other complications in IPAF with IPF. Methods We reviewed the data of all patients who underwent lung transplantation in British Columbia, Canada between January 1, 2014, and April 30, 2024. Diagnoses of IPAF were made through a multidisciplinary approach in conjunction with ILD respirologists, chest radiologists, rheumatologists, and pulmonary pathologists. All IPAF cases met the 2015 ERS/ATS criteria and had compatible explant pathology. Continuous variables were assessed with Mann-Whitney U tests and categorical variables with χ2 tests. Results We identified 16 patients with IPAF and compared them to 32 randomly selected patients with IPF. Patient baseline characteristics before lung transplant and post-transplant outcomes are summarized in Table 1. Before transplant, patients with IPAF were more likely to be on immunosuppression (63% vs 6%, p<0.001), and less likely to be on antifibrotics (19% vs 69%, p<0.001). Age, smoking status, sex, Charleson comorbidity index, and pre-transplant forced vital capacity (FVC), diffusing capacity for carbon monoxide (DLCO), and pulmonary artery systolic pressure (PASP) were similar between the 2 groups. Post lung transplant, no difference was seen in survival at 1 year or cumulative survival at the last follow-up. At 1-year follow-up, no difference was seen in FVC and DLCO, rates of acute rejection, or hospitalization for infection. At last follow-up, no difference was seen in the rates of chronic lung allograft dysfunction (CLAD) or post-transplant malignancy. Table 1. Patient baseline characteristics before lung transplant and post-transplant outcome. Conclusion We found no significant difference in post-transplant survival, lung function, infectious or rejection complications in patients who underwent lung transplantation for IPAF compared with IPF at our center. This study is the first of its kind to report both short-term and long-term outcomes of lung transplantation in patients with IPAF. [1.] Fischer A. Eur Respir J 2015;46:976-87. [2.] Dai J. Clin Rheumatol 2018;37:2125-32. [3.] Zhang N. Arthritis Care Res (Hoboken) 2023;75:2389-98. Best Abstract by a Rheumatology Resident Award

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