Lung Transplantation in Connective Tissue Disease-Associated Interstitial Lung Disease (CTD-ILD)

AbstractPurpose of ReviewConnective tissue disease-related interstitial lung disease (CTD-ILD) previously was a relative contraindication to lung transplantation primarily due to extra-pulmonary involvement of the disease. Recent published information focusing on the diagnosis and management of the extra-pulmonary complexities associated with CTD-ILD that challenge the success of lung transplantation indicate similar outcomes when compared to idiopathic causes of interstitial lung disease. Recent literature examining appropriate wait-listing criteria, disease management, and outcomes after lung transplantation are discussed.Recent FindingsEsophageal dysmotility with or without gastroesophageal reflux disease (GERD) is the most common reason for denying lung transplant candidacy. Recent multiple single-center studies and registry data now show that carefully selected scleroderma and non-scleroderma connective tissue disease patients have acceptable outcomes when compared to matched interstitial lung disease recipients.SummaryConnective tissue disease patients have extra-pulmonary manifestations that must be considered and properly managed in the setting of lung transplantation. With appropriate candidate selection and proper management, lung transplantation is an acceptable treatment option for these individuals and should not be immediately abandoned based on extra-pulmonary complexities.

Source: Current Respiratory Care Reports - September 20, 2018 Category: Respiratory Medicine Source Type: research