Define IPF.
most common type of ILD, characterized by irreversible pulmonary fibrosis and impaired pulmonary function
Describe the epidemiology.
Incidence: 10:100,000 cases per year [2]
Affects mostly men 50–70 years of age
Describe the diagnosis and prognosis.
Diagnosis
Requires the absence of other known causes of interstitial lung disease (e.g., medication, environmental exposures, CTD-ILD)
Presence of usual interstitial pneumonia (UIP) pattern on HRCT or histopathologic studies
Honeycomb appearance with or without traction bronchiectasis
Ground-glass opacification with superimposed reticular abnormalities
Bibasal subpleural distribution
Prognosis: Respiratory failure usually occurs within 3–7 years.
List clinical features.
Progressive dyspnea
Exertional dyspnea that progresses to dyspnea at rest
Patients may present with high-frequency, shallow breathing to compensate for dyspnea. [17]
Persistent nonproductive cough
Bibasilar, inspiratory crackles or rales (velcro-like rales) on auscultation
Fatigue
Fever is common in hypersensitivity pneumonitis and sarcoidosis, but otherwise uncommon.
Findings suggestive of connective tissue disease, sarcoidosis, or vasculitis
Digital clubbing due to chronic hypoxia
Cyanosis
Loud inspiratory wheeze
Last changed2 years ago