Citation, DOI, disclosures and article data
Citation:
Weerakkody Y, Wanderley M, Interstitial pneumonia with autoimmune features. Reference article, Radiopaedia.org (Accessed on 02 Mar 2025) https://doi.org/10.53347/rID-78584
Interstitial pneumonia with autoimmune features (IPAF) is a term given for patients with interstitial lung disease (ILD) who show some features related to autoimmunity but without meeting full criteria for a defined connective tissue disease.
The term is based on the 2015 European Respiratory Society / American Thoracic Society research statement, with the aim of homogenising a specific group of patients, allowing for prospective studies 4.
IPAF is defined by the presence of:
- ILD on high-resolution CT and / or surgical lung biopsy, associated with at least one criteria from two of three different domains:
- clinical
- laboratorial
- morphological
Secondary causes of interstitial lung disease, such as external exposure (i.e. silica; asbestos) or collagen disease must be excluded.
IPAF is an important definition, since only about 15% of this group of patients turn to a collagen disease on a four year follow up 5. On the other hand, the prognosis is better than idiopathic interstitial lung diseases, such as IPF 6.
-
1. Jeong Uk Lim, Bo Mi Gil, Hye Seon Kang, Jongyeol Oh, Yong Hyun Kim, Soon Seog Kwon. Interstitial pneumonia with autoimmune features show better survival and less exacerbations compared to idiopathic pulmonary fibrosis. (2019) BMC Pulmonary Medicine. 19 (1): 1. doi:10.1186/s12890-019-0868-9 - Pubmed
-
2. Bridget Collins, Ganesh Raghu. Interstitial pneumonia with autoimmune features: the new consensus-based definition for this cohort of patients should be broadened. (2016) European Respiratory Journal. 47 (4): 1293. doi:10.1183/13993003.02084-2015 - Pubmed
-
3. Vij R et.al Autoimmune-featured Interstitial Lung Disease: A Distinct Entity. (2011) Chest. doi:10.1378/chest.10-2662 - Pubmed
-
4. Fischer A, Antoniou KM, Brown KK, Cadranel J, Corte TJ, du Bois RM, Lee JS, Leslie KO, Lynch DA, Matteson EL, Mosca M, Noth I, Richeldi L, Strek ME, Swigris JJ, Wells AU, West SG, Collard HR, Cottin V. An official European Respiratory Society/American Thoracic Society research statement: interstitial pneumonia with autoimmune features. (2015) The European respiratory journal. 46 (4): 976-87. doi:10.1183/13993003.00150-2015 - Pubmed
-
5. Pereira DA, Dias OM, Almeida GE, Araujo MS, Kawano-Dourado LB, Baldi BG, Kairalla RA, Carvalho CR. Lung-dominant connective tissue disease among patients with interstitial lung disease: prevalence, functional stability, and common extrathoracic features. (2015) Jornal brasileiro de pneumologia : publicacao oficial da Sociedade Brasileira de Pneumologia e Tisilogia. 41 (2): 151-60. doi:10.1590/S1806-37132015000004443 - Pubmed
-
6. Sebastiani M, Faverio P, Manfredi A, Cassone G, Vacchi C, Stainer A, Pozzi MR, Salvarani C, Pesci A, Luppi F. Interstitial Pneumonia with Autoimmune Features: Why Rheumatologist-Pulmonologist Collaboration Is Essential. (2020) Biomedicines. doi:10.3390/biomedicines9010017 - Pubmed
Promoted articles (advertising)
Unable to process the form. Check for errors and try again.