Sleep Apnea Management in “Possible IPF” and “Idiopathic NSIP”: A Case- Series

Purpose: The clinical course of patients with possible idiopathic pulmonary fibrosis (IPF) and idiopathic nonspecific interstitial pneumonia (NSIP) is not well understood. While these patients are clinically followed for disease progression before rendering therapy, the import of sleep-disordered breathing on the disease course is unknown. Methods: Retrospective analysis of possible IPF and idiopathic NSIP patients seen at a single center with review of clinical data pertinent to therapy for co-morbid sleep-disordered breathing was done. Results: 7 patients with possible IPF based upon radiologic possible UIP patterns (5 male and 2 female) and 6 patients with idiopathic NSIP (5 female and 1 male) were followed for an average duration of 40 and 30 months respectively. 6/7 patients with possible IPF required continuous positive airway pressure (CPAP) therapy for sleepdisordered breathing and out of this 5/6 were compliant with therapy. Of idiopathic NSIP patients, 2/6 presented with acute exacerbations. All 6 patients required therapy for OSA out of which 5/6 were treated with CPAP and 1 treated with supplemental oxygen. Patients with possible IPF appeared to stabilize their disease following treatment of OSA and none of the study patients experienced any recurrence of acute exacerbations on CPAP therapy. Conclusions: OSA can be an important problem in patients with possible IPF and idiopathic NSIP. Treatment with nocturnal CPAP therapy in these patients with concomitant OSA may result in stability of underlying interstitial lung disease.