*Purpose: To evaluate the post-lung transplant comorbidities of patients with connective tissue disease (CTD) compared with an age-matched cohort of patients transplanted for idiopathic pulmonary fibrosis (IPF). Mortality has previously been established to be similar in these patient populations, but development of life-altering morbidity has been under-recognized.

*Methods: Patients who underwent lung transplant at The Johns Hopkins Hospital from 5/2014 to 11/2019 were retrospectively screened if they had a diagnosis of CTD, interstitial lung disease ILD, or IPF. CTD-ILD included patients with scleroderma, systemic sclerosis, rheumatoid arthritis, systemic lupus erythematosus, dermatomyositis/polymyositis or mixed connective tissue disease. Pretransplant, baseline comparators included age; lung allocations score (LAS), digital ischemia; venous thromboembolism (VTE); esophageal dysmotility; mean pulmonary artery pressure (mPAP); cardiac index (CI); 24-hour urine CrCl; and presence of Raynaud’s. Post-transplant comparators included death from any cause; PEA arrest; index length of stay (LOS); presence of VTE; esophageal dysmotility; digital ischemia; dialysis at any point; CKD staging; presence of primary graft dysfunction (PGD) stage 3 at 72 hours; chronic lung allograft dysfunction (CLAD) staging; and life-threatening infections from causes other than donor pathogens.

*Results: 18 patients met criteria for CTD-ILD, and they were compared to 16 age-matched patients with IPF. At baseline, the populations had no significant differences in age (54 vs. 58.5 years), LAS (42.5 vs. 48.2), digital ischemia (6% vs. 0%), VTE (6% vs 6%), esophageal dysmotility (50% vs. 75%), mPAP (30 vs. 24mmHg), CI (2.99 vs. 2.81L/min/m²), CrCl (94 vs. 113 mg/dL). Patients with CTD-ILD had significantly more Raynaud’s (50% vs.12.5%, p=0.04), and pre-transplant immunosuppression; prednisone (94% vs.56%, p<0.001), mycophenolate mofetil/azathioprine (83% vs. 31%, p <0.001), 3^rd immunosuppressive agent (50% vs.6%, p<0.001). Post-transplant, there was a trend toward increased PGD (39% vs. 12.5%, p=0.09) and significantly more digital ischemia (39% vs. 0%, p=0.04); dialysis (33% vs.6%, p=0.03), and life-threatening infections (50% vs.12%, p=0.008). There was no significant difference in 1-year mortality (14% vs. 15%), death from any cause (4 vs. 4), PEA arrest (4 vs. 2), LOS (22.5 days vs. 12 days), CLAD Stage 3 or greater (4 vs. 3), swallow function (39% vs. 50%) or VTE (18%).

*Conclusions: In an age- matched cohort of patients undergoing lung transplantation for CTD-ILD compared with IPF at The Johns Hopkins Hospital, there was significantly more morbidity in the form of digital ischemia, dialysis, and life-threatening infection, without any increased mortality. Morbidity outcomes in this high-risk patient population warrants further investigation.

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