Double Lung Transplantation for Idiopathic Pulmonary Fibrosis in a Patient with a History of Liver Transplantation and Prolonged Journey for Disease-Specific Antifibrotic Therapy.

Idiopathic pulmonary fibrosis (IPF) is characterized by uncontrolled progressive lung fibrosis with a median survival of 3 to 5 years. Although currently available pharmacotherapy cannot cure the disease, antifibrotics including pirfenidone and nintedanib were shown to slow disease progression and improve survival in IPF. Nevertheless, there is a knowledge gap on the safety of antifibrotics in patients after liver transplantation receiving concomitant immunosuppressive therapy. This case report of a 68-year-old male patient with IPF illustrates how a complex medical history has led to diagnostic and therapeutic challenges considerably affecting clinical decisions and impacting the patient's journey. The increasing severity of lung function impairment due to the progressive natural history of IPF ultimately led to severe respiratory failure. Double lung transplantation (LTx) was performed as the only therapeutic option in end-stage disease with the potential to improve quality of life and survival. To the best of our knowledge, this is the first case report describing the feasibility and safety of antifibrotic therapy with pirfenidone for IPF in a 68-year-old patient with a history of liver transplantation receiving concomitant immunosuppressive therapy with tacrolimus who underwent successful double lung transplantation when alternative medical interventions had been exhausted.

Emphysema as a possible complication of infant respiratory distress syndrome leading to lung transplantation.

Infant respiratory distress syndrome (IRDS) develops among premature infants due to structural immaturity of the lungs and insufficient production of pulmonary surfactant. Nowadays, treatment takes place under conditions of intensive care and includes oxygen therapy, mechanical ventilation, exogenous supplementation of pulmonary surfactant and antenatal corticosteroid therapy. The treatment of IRDS, especially mechanical ventilation, may lead to complications which can contribute to developing a severe dysfunction of the respiratory system. Unavailability of pharmacological treatment of IRDS and development of pulmonary barotrauma due to mechanical ventilation in our patient led to the forming of severe pulmonary interstitial emphysema. In this case report, lung transplantation was performed as an only successful therapeutic option.