End-stage pulmonary fibrosis of unknown origin, taken from an autopsy
Specialty
Pulmonology
Complications
Respiratory failure
Usual onset
Months – years, depending on cause/type
Duration
Months – years, depending on cause/type
Frequency
1.9 million (2015)[1]
Deaths
122,000 (2015)[2]
Interstitial lung disease (ILD), or diffuse parenchymal lung disease (DPLD),[3] is a group of respiratory diseases affecting the interstitium (the tissue) and space around the alveoli (air sacs) of the lungs.[4] It concerns alveolar epithelium, pulmonary capillary endothelium, basement membrane, and perivascular and perilymphatic tissues. It may occur when an injury to the lungs triggers an abnormal healing response. Ordinarily, the body generates just the right amount of tissue to repair damage, but in interstitial lung disease, the repair process is disrupted, and the tissue around the air sacs (alveoli) becomes scarred and thickened. This makes it more difficult for oxygen to pass into the bloodstream. The disease presents itself with the following symptoms: shortness of breath, nonproductive coughing, fatigue, and weight loss, which tend to develop slowly, over several months. The average rate of survival for someone with this disease is between three and five years.[5] The term ILD is used to distinguish these diseases from obstructive airways diseases.
There are specific types in children, known as children's interstitial lung diseases. The acronym ChILD is sometimes used for this group of diseases.[6]
Thirty to 40% of those with interstitial lung disease eventually develop pulmonary fibrosis which has a median survival of 2.5-3.5 years.[7] Idiopathic pulmonary fibrosis is interstitial lung disease for which no obvious cause can be identified (idiopathic) and is associated with typical findings both radiographic (basal and pleural-based fibrosis with honeycombing) and pathologic (temporally and spatially heterogeneous fibrosis, histopathologic honeycombing, and fibroblastic foci).
In 2015, interstitial lung disease, together with pulmonary sarcoidosis, affected 1.9 million people.[1] They resulted in 122,000 deaths.[2]
^ abGBD 2015 Disease and Injury Incidence and Prevalence Collaborators (8 October 2016). "Global, regional, and national incidence, prevalence, and years lived with disability for 310 diseases and injuries, 1990–2015: a systematic analysis for the Global Burden of Disease Study 2015". Lancet. 388 (10053): 1545–1602. doi:10.1016/S0140-6736(16)31678-6. PMC 5055577. PMID 27733282.
^ abGBD 2015 Mortality and Causes of Death Collaborators (8 October 2016). "Global, regional, and national life expectancy, all-cause mortality, and cause-specific mortality for 249 causes of death, 1980–2015: a systematic analysis for the Global Burden of Disease Study 2015". Lancet. 388 (10053): 1459–1544. doi:10.1016/S0140-6736(16)31012-1. PMC 5388903. PMID 27733281.
^King TE (August 2005). "Clinical advances in the diagnosis and therapy of the interstitial lung diseases". American Journal of Respiratory and Critical Care Medicine. 172 (3): 268–79. doi:10.1164/rccm.200503-483OE. PMID 15879420. S2CID 29591585.
^"Frequently Asked Questions About Interstitial Lung Disease". University of Chicago Medical Center.
^Meyer, Keith C; Decker, Catherine A (2017-04-03). "Role of pirfenidone in the management of pulmonary fibrosis". Therapeutics and Clinical Risk Management. 13: 427–437. doi:10.2147/TCRM.S81141. ISSN 1176-6336. PMC 5388201. PMID 28435277.
^Bush A, Cunningham S, de Blic J, Barbato A, Clement A, Epaud R, Hengst M, Kiper N, Nicholson AG, Wetzke M, Snijders D, Schwerk N, Griese M (November 2015). "European protocols for the diagnosis and initial treatment of interstitial lung disease in children". review. Thorax. 70 (11): 1078–84. doi:10.1136/thoraxjnl-2015-207349. hdl:10044/1/24599. PMID 26135832.
^Maher, Toby M. (22 April 2024). "Interstitial Lung Disease: A Review". JAMA. doi:10.1001/jama.2024.3669.
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