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Update in diffuse parenchymal lung disease 2006.

IIPs Prevalence of disease. Epidemiology is necessarily an inexact science. Previous statements of the incidence and prevalence of IPF have largely centered on death certification and disease registries (1, 2). Clinical diagnostic criteria, which are sensitive but not specific for IPF, capture other disorders with the clinical features of IPF, including NSIP, other IIPs, and patients with undiagnosed hypersensitivity pneumonitis (HP). Imprecision in the estimation of the prevalence of IPF is well illustrated in a recent evaluation of a U.S. health care claims database (3). Based on narrow case definitions, requiring the performance of a biopsy procedure (surgical or transbronchial biopsy) or high-resolution computed tomography (HRCT), the incidence and prevalence of IPF in the United States were estimated to be 6.8 per 100,000 and 14 per 100,000, respectively. However, with more liberal diagnostic criteria, these figures rose strikingly to 16.3 per 100,000 and 42.7 per 100,000, respectively. As the true figure lies somewhere between these estimates, IPF appears to be substantially more prevalent than previously reported, a conclusion supported by a reported incidence of 6.8 per 100,000 in the United Kingdom, in 2000–2003 (4). It is not clear whether these findings primarily represent changes in clinician diagnostic thresholds or whether there has been a real increase in disease prevalence. However, this uncertainty should not obscure the fact that IPF appears to be approximately as prevalent as a number of the more common malignancies, all of which attract a much larger share of community and research resources. Acute exacerbations of IPF have historically been viewed as rare events, a perception challenged by a surprisingly high prevalence of rapid deterioration in the inactive arms of three intervention studies (5–7). A 2-year frequency of acute exacerbations of approximately 10% has now been reported in a cohort of 147 patients with IPF (8), underlining the need for a reappraisal of the risk factors and management of this often-lethal disorder.