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Clinical manifestations and diagnosis of idiopathic pulmonary fibrosis

Talmadge E King, Jr, MD
Section Editor
Kevin R Flaherty, MD, MS
Deputy Editor
Helen Hollingsworth, MD


Idiopathic pulmonary fibrosis (IPF)/usual interstitial pneumonia (UIP), previously known as cryptogenic fibrosing alveolitis (CFA) in Europe, is the most common type of idiopathic interstitial pneumonia (IIP). IIPs are spontaneously occurring (ie, idiopathic) diffuse parenchymal lung diseases. An American Thoracic Society (ATS) consensus statement defined IPF as a spontaneously occurring (idiopathic) specific form of chronic fibrosing interstitial pneumonia limited to the lung and associated with a pattern of UIP on high resolution computed tomography or histologic appearance on surgical (thoracoscopic or open) lung biopsy [1,2].

The other IIPs include nonspecific interstitial pneumonia (NSIP), desquamative interstitial pneumonia (DIP), respiratory bronchiolitis associated interstitial lung disease (RB-ILD), acute interstitial pneumonia (AIP), lymphocytic interstitial pneumonia (LIP), and cryptogenic organizing pneumonia (COP).

The clinical manifestations, evaluation, and diagnosis of IPF will be reviewed here. The evaluation of interstitial lung disease in general, the diagnosis and management of the other IIPs, and the treatment of IPF are discussed separately. (See "Approach to the adult with interstitial lung disease: Clinical evaluation" and "Approach to the adult with interstitial lung disease: Diagnostic testing" and "Idiopathic interstitial pneumonias: Clinical manifestations and pathology" and "Acute interstitial pneumonia (Hamman-Rich syndrome)" and "Treatment and prognosis of nonspecific interstitial pneumonia" and "Cryptogenic organizing pneumonia" and "Respiratory bronchiolitis-associated interstitial lung disease" and "Lymphoid interstitial pneumonia in adults".)


Reported prevalence and incidence data for idiopathic pulmonary fibrosis (IPF) vary and depend on ascertainment and reporting methods and also the age and geographic location of the population. Both prevalence and incidence increase with advancing age, with presentation commonly occurring in the sixth and seventh decades; rarely is IPF seen in patients aged less than 50 years [1,2]. The prevalence and incidence are higher in men than women [3].

In a systematic review, the prevalence of IPF ranged from 0.5 to 27.9/100,000 and the incidence ranged from 0.22 to 8.8/100,000 [4]. In the United States, IPF incidence estimates range from 7 to 16 cases per 100,000 overall [5]. In contrast, among a random sample of Medicare beneficiaries (largely ≥65 years old), the prevalence of IPF was 494 cases per 100,000, and the incidence was 94 cases per 100,000 person years [6]. In the United Kingdom, the IPF incidence in 2008 was 7.4 per 100,000 person-years [7]. In Europe, IPF prevalence ranged from 1.25 to 23.4 cases per 100,000 population, and the annual incidence ranged between 0.22 and 7.4 per 100,000 population [8]. Overall, the incidence of IPF is increasing worldwide and conservative estimates of the incidence range from 3 to 9 cases per 100,000 per year for Europe and North America [9].

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Literature review current through: Dec 2017. | This topic last updated: Jan 11, 2018.
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