Abstract
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.
Highlights
Idiopathic interstitial pneumonias represent a difficult group of diseases where the disease evolution is unpredictable and there are no good treatment options
Sleep quality is in idiopathic pulmonary fibrosis (IPF) patients is affected by nocturnal hypoxemia, the severity of which correlates with daytime measures of physical and social functioning [3]
While initial reports focused on the high prevalence of obstructive sleep apnea (OSA) in IPF patients [5,6], recent reports indicate that OSA is prevalent in interstitial lung disease (ILD) from a variety of etiologies [4,7]
Summary
Idiopathic interstitial pneumonias represent a difficult group of diseases where the disease evolution is unpredictable and there are no good treatment options. A benefit of treating OSA on progression of IPF has been shown [8], but similar results are unavailable for other types of ILDs. IPF can be a difficult diagnosis especially if radiologic patterns do meet criteria for UIP on high-resolution chest CT (HRCT) [9]. IPF can be a difficult diagnosis especially if radiologic patterns do meet criteria for UIP on high-resolution chest CT (HRCT) [9] Such patients with radiologic possible usual interstitial pneumonia (UIP) are not always subjected to a definitive surgical lung biopsy and are followed expectantly. A retrospective analysis of patients with idiopathic NSIP and possible IPF (possible UIP by radiology) was done to assess the implications of the diagnosis of OSA and potential benefit of CPAP therapy in non-IPF interstitial lung disease
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