Background and objective Antifibrotic drugs are the standard treatments for patients with idiopathic pulmonary fibrosis (IPF). This study aims to assess the safety of antifibrotic treatment in IPF patients undergoing lung transplantation. Methods Patients with a diagnosis of IPF who received a lung transplant between January 2015 and June 2019 at four Spanish hospitals specialized in lung transplantation were retrospectively recruited. Cases were defined as patients receiving antifibrotic treatments at time of transplant. Each case was matched with a control who did not receive antifibrotic treatment. Results A total of 164 patients were included in the study cohort (103 cases and 61 controls). There were no statistically significant differences between the cases and controls in any of the items studied related to transplantation except the time until the appearance of chest wall dehiscence: although there were no differences in the incidence of wall dehiscence in either group (12.3% vs. 13.7%; p = 0.318), the patients on antifibrotic drugs experienced it earlier (21 days [IQR = 12.5–41.5] vs. 63 days [IQR = 46.75–152.25]; p = 0.012). There were no differences in overall post‐transplant survival between the two groups (p = 0.698) or in conditional survival at 30 days, 90 days, 3 years or 5 years. However, 1 year survival was significantly greater among controls (80.6% vs. 93.3%; p = 0.028). Conclusion There was evidence that chest wall dehiscences appeared earlier post‐transplant in patients using antifibrotics, even though this factor did not significantly impact survival.

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Klinefelter syndrome is one of the most frequent congenital chromosome disorders. Autoimmune diseases are not uncommon in patients with Klinefelter syndrome and associations between them have long been described. However, there is a paucity of literature regarding the effect of hypogonadism on respiratory system in these patients. Herein, we described a 28-year-old patient who presented with a constellation of unexplained respiratory signs and symptoms, such as tachypnea, hypoxemia, crackles recurrent pulmonary infections since childhood and progressive worsening of his pulmonary function, was diagnosed with Klinefelter syndrome in adulthood. CT chest with diffuse interstitial lung involvement and multiple cystic areas. Based on the radiological findings, an underlying autoimmune process was suspected and investigations showed findings compatible with Sjögren's syndrome. Double lung transplantation was thus indicated considering the irreversible interstitial changes in both lungs. Explant biopsy showed up histological pattern suggestive of interstitial lung disease with autoimmune features. Therefore, the importance of suspecting connective tissue diseases in patients with Klinefelter syndrome, perhaps due to the double X chromosome and a low androgen-toestrogen ratio that characterizes them.

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