INTRODUCTION: Fibrotic interstitial lung diseases (ILD) are associated with significant morbidity and poor prognosis. Infections, including chronic pulmonary aspergillosis (CPA), may further worsen outcomes but remain understudied in this population. This study aimed to characterize the clinical features and outcomes of ILD patients who develop CPA. METHODS: We conducted a nationwide retrospective study across expert centers of the French rare pulmonary diseases Orphalung network, including patients with idiopathic ILD who met diagnostic criteria for CPA. RESULTS: Twenty-six patients were identified, predominantly male (96%) and smokers (88%). The most common underlying diagnosis was idiopathic pulmonary fibrosis. At CPA diagnosis, median forced vital capacity was 63% 49-87 predicted and DLCO 29% 21-39 predicted. The median interval between ILD diagnosis and CPA onset was 39 months 0-72. All patients had CT scans showing CPA-compatible features, and 96% tested positive for Aspergillus IgG. Chest computed tomography revealed emphysema in 92% of patients, with CPA lesions predominantly in emphysematous areas (73%) and upper zones (69%). Over a median follow-up of 21 months 10-33, median survival from CPA diagnosis was 24 months 15-NA. Neither prior corticosteroid nor antifungal treatment was associated with improved survival. DISCUSSION: CPA is a rare but clinically significant complication in patients with fibrotic ILD. Lesions are frequently localized in emphysematous regions, and serum IgG testing is a key diagnostic tool. Further research is needed to better characterize this patient population and optimize management strategies.
Bermudez et al. (Thu,) studied this question.