Idiopathic pulmonary fibrosis - Symptoms, Causes, Images, and Treatment Options

Diseases

Highlights & Basics

Key Highlights

Idiopathic pulmonary fibrosis (IPF) is a rare fibrosing interstitial pneumonia of unknown cause. The disease is chronic, progressive, and ultimately fatal.
Diagnosis is suspected clinically in patients with bibasilar inspiratory crackles, older age (e.g., >60 years), and unexplained symptomatic or asymptomatic bilateral pulmonary fibrosis on a chest x-ray or high-resolution computed tomography.
In the absence of findings suggesting an alternative disease process, IPF is usually confirmed by multidisciplinary review based on the presence of a radiologic (high-resolution chest CT) pattern of usual interstitial pneumonia (UIP). Lung biopsy histopathology may be considered when radiologic and clinical data result in an uncertain diagnosis.
Initial therapy is generally with an antifibrotic agent (either pirfenidone or nintedanib), although some patients might benefit from other treatment options depending on disease severity. Important supportive measures include smoking cessation, pulmonary rehabilitation, and supplemental oxygen.
Palliative care options and referral for lung transplantation should be considered early, even if a patient is being actively treated.

Chest computed tomography scan image of idiopathic pulmonary fibrosis

From the collection of Jeffrey C. Munson, MD, MS; used with permission

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Definition

Epidemiology

Etiology

Pathophysiology

Images

Chest computed tomography scan image of idiopathic pulmonary fibrosis
Pathology slide of usual interstitial pneumonia, the characteristic biopsy appearance of a patient with idiopathic pulmonary fibrosis

Citations

Key Articles

Raghu G, Collard HR, Egan JJ, et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011 Mar 15;183(6):788-824.[Abstract][Full Text]
Raghu G, Rochwerg B, Zhang Y, et al. An official ATS/ERS/JRS/ALAT clinical practice guideline: treatment of idiopathic pulmonary fibrosis - an update of the 2011 clinical practice guideline. Am J Respir Crit Care Med. 2015 Jul 15;192(2):e3-19.[Abstract][Full Text]
Raghu G, Remy-Jardin M, Richeldi L, et al; American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Latin American Thoracic Society. Idiopathic pulmonary fibrosis (an update) and progressive pulmonary fibrosis in adults: an official ATS/ERS/JRS/ALAT clinical practice guideline. Am J Respir Crit Care Med. 2022 May 1;205(9):e18-e47.[Abstract][Full Text]
Collard HR, Ryerson CJ, Corte TJ, et al. Acute exacerbation of idiopathic pulmonary fibrosis. an international working group report. Am J Respir Crit Care Med. 2016 Aug 1;194(3):265-75.[Abstract][Full Text]
Borie R, Kannengiesser C, Antoniou K, et al. European Respiratory Society statement on familial pulmonary fibrosis. Eur Respir J. 2023 Mar;61(3):2201383.[Abstract][Full Text]

Other Online Resources

British Lung Foundation: idiopathic pulmonary fibrosis (IPF)
University of Virginia Health System: idiopathic pulmonary fibrosis
Centers for Disease Control and Prevention: Advisory Committee on Immunization Practices (ACIP)

Referenced Articles

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