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Idiopathic Pulmonary Fibrosis (IPF)

Name of disease:

Idiopathic Pulmonary Fibrosis (IPF)

ICD-10 diagnosis code:

J84.112

Causes:

Idiopathic Pulmonary Fibrosis (IPF) is a type of interstitial lung disease. The condition develops when lung tissue becomes thick and stiff due to unknown causes. Although the cause of the scarring is unknown, smoking, family history, and aging increase the risk of developing IPF

Pattern of inheritance:

The pathogenesis is not well understood. Although genetic variants contribute to the risk of developing IPF in some patients with sporadic or familial IPF, their clinical relevance needs to be demonstrated in future studies.

Prevalence:

The prevalence of IPF in Asia-Pacific regions ranged from 0.57 to 4.51 per 10,000 population.

Diagnosis:

Evaluations include clinical presentation, detailed history assessment, smoking status, lung function evolution, serological test results, imaging and, if required, lung biopsy.

Age of onset:

Peak age of onset of IPF is 50-60. Patients with IPF who are younger than 50 years old are rare.

Common signs and symptoms:

Dry cough that does not improve, shortness of breath that worsens over time, achy joints and muscles, fatigue and weakness, loss of appetite, loss of weight.

Available treatments (medicinal and non-medicinal):

Drug treatment for symptomatic relief, anti-fibrotics, pulmonary rehabilitation, oxygen therapy, lung transplant.

Disease management tips:

N/A

References:

Maher TM, Bendstrup E, Dron L, Langley J, Smith G, Khalid JM, Patel H, Kreuter M. Global incidence and prevalence of idiopathic pulmonary fibrosis. Respir Res. 2021 Jul 7;22(1):197. doi: 10.1186/s12931-021-01791-z. PMID: 34233665; PMCID: PMC8261998.
Raghu G, Remy-Jardin M, Myers JL, Richeldi L, Ryerson CJ, Lederer DJ, Behr J, Cottin V, Danoff SK, Morell F, Flaherty KR, Wells A, Martinez FJ, Azuma A, Bice TJ, Bouros D, Brown KK, Collard HR, Duggal A, Galvin L, Inoue Y, Jenkins RG, Johkoh T, Kazerooni EA, Kitaichi M, Knight SL, Mansour G, Nicholson AG, Pipavath SNJ, Buendía-Roldán I, Selman M, Travis WD, Walsh S, Wilson KC; American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Latin American Thoracic Society. Diagnosis of Idiopathic Pulmonary Fibrosis. An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline. Am J Respir Crit Care Med. 2018 Sep 1;198(5):e44-e68. doi: 10.1164/rccm.201807-1255ST. PMID: 30168753.

Other useful websites:

What Is Idiopathic Pulmonary Fibrosis?
https://www.nhlbi.nih.gov/health/idiopathic-pulmonary-fibrosis