Trends in diagnosis and management of idiopathic pulmonary fibrosis in Canada

Abstract

ABSTRACTRATIONALE: Idiopathic pulmonary fibrosis (IPF) is a progressive lung disease with a poor prognosis. Physician practice patterns on the diagnosis and management of IPF in Canada have not been described.OBJECTIVE: To describe the practice patterns of Canadian respirologists on the diagnosis and management of IPF and to determine if these patterns reflect current guidelines.METHODS: We conducted surveys of Canadian physicians and fellows-in-training in 2009 and 2013. Questions addressed the diagnosis of IPF, screening for comorbidities, treatment of IPF, and acute exacerbations of IPF.MEASUREMENTS AND MAIN RESULTS: Response rates in the 2009 and 2013 surveys were 22% and 17%, respectively. Most respondents felt that high resolution CT scan can diagnose IPF in most cases, with lung biopsy rarely performed. The majority screened for hypoxemia, pulmonary hypertension, gastroesophageal reflux, and autoimmune disease. In 2013, more respondents used no pharmacologic therapy to treat IPF (2009, 36% vs. 2013, 56%, p < 0.01) and 34% used first-line pirfenidone. Fewer respondents used prednisone (2009, 61% vs. 2013, 7%, p < 0.001) or azathioprine (2009, 38% vs. 2013, 1%, p < 0.001), while many employed first-line N-acetylcysteine (2009, 47% vs. 2013, 42%, p = 0.47). In 2013, 20% had no access to multidisciplinary diagnosis discussion, 33% accessed specialty IPF clinics, and <1% accessed palliative care. Referral for lung transplantation was higher in 2013 than 2009 (26% vs. 13%, p = 0.01). Treatment of acute exacerbations changed, with more use of corticosteroids and antibiotics, and less additional immunosuppression in 2013.CONCLUSIONS: Practice patterns for diagnosis and treatment of IPF in Canada evolved in concordance with the publication of international guidelines in 2011. Access to palliative care, lung transplantation referral, multidisciplinary diagnosis discussion, and specialty IPF clinics were identified as gaps in IPF care in Canada.