Abstract
Idiopathic pulmonary fibrosis (IPF) is a specific form of progressive, chronic fibrosing
interstitial lung disease occurring primarily in older adults and limited to the lungs.
The prognosis is dire, with half of all patients progressing to death from respiratory
failure within 3 to 5 years from initial diagnosis. The pathogenesis of IPF is complex
and incompletely understood, and the natural history of this disease is variable and
unpredictable. There is no widely accepted treatment for IPF, except for lung transplantation.
Nevertheless, these are important and exciting times for the diagnosis and management
of patients with IPF. Novel diagnostic approaches (e.g., biomarker-based), improved
prognostic models, and clinical trials of novel drug agents may alter disease management
substantially over the next few years. For now, clinicians should practice comprehensive
management, including symptom-based management, aggressive management of comorbidities,
and patient education and support.
Keywords
idiopathic pulmonary fibrosis - usual interstitial pneumonia - familial interstitial
pneumonia
