Long-Term Outcomes in Lung Transplant for Anti-Melanoma Differentiation-Associated Gene 5 Antibody Positive Interstitial Lung Disease: A Case Series

Objectives To evaluate the long-term outcomes of patients who survived the first year after lung transplant for interstitial lung disease (ILD) secondary to anti-melanoma differentiation-associated gene 5 antibody (MDA5). Methods A retrospective chart review was conducted for patients who survived lung transplant (with transplant date between 2017 and 2020) for anti-MDA5 associated ILD, with a minimum of 48 months follow-up, in British Columbia, Canada. This project received ethical approval from the University of British Columbia. Results Five patients were identified, 3 females (60%) and 2 males, mean age at diagnosis of idiopathic inflammatory myopathy (IIM) was 43.2 years old, with a mean follow-up time of 73 months. Two patients were Chinese (40%), 1 patient was White (20%), 1 patient was Filipino (20%), 1 patient’s ethnicity was not recorded (20%). Four patients presented with rapidly progressive interstitial lung disease (RP-ILD) (decline within 3 months of ILD onset), the other had progressive ILD over 12 months and pneumomediastinum. All patients were immunosuppressed prior to transplant; with glucocorticoids (GC) (n=5), cyclophosphamide (n=3), rituximab (n=3), IVIG (n=3), mycophenolate mofetil (MMF) (n=2), tacrolimus (n=1), azathioprine (n=1), ciclosporin (n=1), and hydroxychloroquine (n=1). All patients remain on immunosuppression; GC, tacrolimus and MMF triple therapy in all patients (n=5) and 2 patients treated with the addition of monthly IVIG (n=2). The most common complications were infections; 9 incidences of lung allograft infection were reported. Other infections included COVID19 (n=3), shingles (n=2), CMV viraemia (n=1), EBV viraemia (n=1), bacteremia (n=2), UTI (n=1) and parapneumonic effusion (n=1). There were no fungal infections. Two patients had acute cellular rejections (resolved), 1 patient had chronic allograft dysfunction causing airflow obstruction, and 2 patients had restrictive lung defects from baseline after transplant. Heart failure with preserved ejection fraction (HEFPEF) occurred in 2 patients within the first year after transplant. There have been no flares of underlying auto-immune disease. None of the patients have required long-term oxygen therapy and all patients are able to perform usual activities of daily living. Detailed case review is displayed in Table 1. Table 1: MDA5 lung transplant case details Conclusion This is the first study of long-term follow-up in this patient group. Infections were common but all resolved. HEFPEF was seen in 40% of patients, despite it being uncommon in the general population. This case series provides evidence that patients who survive lung transplant can have good long-term respiratory and functional outcomes.

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