| H01716 | |
| H number | H01716 |
| Name | Idiopathic interstitial pneumonias |
| Description | Idiopathic interstitial pneumonias (IIP) are a heterogeneous subset of interstitial lung diseases, characterized by unknown aetiology. Despite the varied nature of IIPs, the common histological feature is distortion of lung interstitium by highly variable combinations of inflammation and fibrosis. Patients experience common symptoms related to their chronic lung disease. Dyspnoea, cough, fatigue and depression contribute substantially to morbidity and are often difficult to manage. It has been reported that pulmonary rehabilitation plays a central role in symptom management and has beneficial effects. According to the current American thoracic society/European respiratory society (ATS/ERS), IIPs are categorised as major IIPs, rare IIPs and unclassifiable IIPs. There are six major IIPs, namely, idiopathic pulmonary fibrosis (IPF), nonspecific interstitial pneumonia (NSIP), respiratory bronchiolitis-interstitial lung disease (RB-ILD), desquamative interstitial pneumonia (DIP), cryptogenic organizing pneumonia (COP), and acute interstitial pneumonia (AIP). And they are divided into three major groups; chronic fibrosing IIP (IPF, NSIP), smoking-related IIP (RB-ILD, DIP), and acute/ subacute IIP (COP, AIP). The rare IIPs include idiopathic lymphoid interstitial pneumonia (LIP) and idiopathic pleuroparenchymal fibroelastosis. IPF accounts for the majority of IIP. It is considered to be lethal because prognosis is very poor and far worse than other types of IIP. An early and accurate diagnosis of IPF is critical. |
| Category | Respiratory system disease |
| Network | - |
| Gene | - |
| Pathogen | - |
| Env factor | - |
| Carcinogen | - |
| Drug | - |
| Comment | See also H01299 Idiopathic pulmonary fibrosis. |
| Other DBs | ICD-11: CB03 ICD-10: J84 MeSH: D054988 |
| Reference | PMID:24032382 AUTHORS Travis WD, Costabel U, Hansell DM, King TE Jr, Lynch DA, Nicholson AG, Ryerson CJ, Ryu JH, Selman M, Wells AU, Behr J, Bouros D, Brown KK, Colby TV, Collard HR, Cordeiro CR, Cottin V, Crestani B, Drent M, Dudden RF, Egan J, Flaherty K, Hogaboam C, Inoue Y, Johkoh T, Kim DS, Kitaichi M, Loyd J, Martinez FJ, Myers J, Protzko S, Raghu G, Richeldi L, Sverzellati N, Swigris J, Valeyre D TITLE An official American Thoracic Society/European Respiratory Society statement: Update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. JOURNAL Am J Respir Crit Care Med 188:733-48 (2013) DOI:10.1164/rccm.201308-1483ST PMID:24591661 AUTHORS Antoniou KM, Margaritopoulos GA, Tomassetti S, Bonella F, Costabel U, Poletti V TITLE Interstitial lung disease. JOURNAL Eur Respir Rev 23:40-54 (2014) DOI:10.1183/09059180.00009113 PMID:26011188 AUTHORS Neurohr C, Behr J TITLE Changes in the current classification of IIP: A critical review. JOURNAL Respirology 20:699-704 (2015) DOI:10.1111/resp.12553 PMID:26949346 AUTHORS Hashisako M, Fukuoka J TITLE Pathology of Idiopathic Interstitial Pneumonias. JOURNAL Clin Med Insights Circ Respir Pulm Med 9:123-33 (2015) DOI:10.4137/CCRPM.S23320 PMID:26450007 AUTHORS Garibaldi BT, Danoff SK TITLE Symptom-based management of the idiopathic interstitial pneumonia. JOURNAL Respirology 21:1357-1365 (2016) DOI:10.1111/resp.12649 |