PMID- 31137065 OWN - NLM STAT- MEDLINE DCOM- 20200429 LR - 20200429 IS - 1098-9048 (Electronic) IS - 1069-3424 (Linking) VI - 40 IP - 2 DP - 2019 Apr TI - Interstitial Pneumonia with Autoimmune Features and Undifferentiated Connective Tissue Disease. PG - 271-277 LID - 10.1055/s-0039-1684007 [doi] AB - In 2015, a multidisciplinary task force comprising pulmonologists, rheumatologists, pathologists, and radiologists representing the European Respiratory Society and American Thoracic Society published a diagnostic classification schema for individuals with interstitial lung disease and autoimmune features who did not meet criteria for a defined connective tissue disease. The term interstitial pneumonia with autoimmune features (IPAF) was applied. Classification criteria are often nonspecific, but up to 90% of subjects with IPAF have serological evidence for autoimmunity (particularly (+) antinuclear antibodies). Distinguishing patients with IPAF from idiopathic pulmonary disorders may be difficult. The natural history and appropriate management of IPAF have not been clarified, as data are largely limited to retrospective studies. In this review, we discuss the salient clinical, serologic, histologic, and radiographic features of IPAF and discuss an approach to management. CI - Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA. FAU - Chernau, Andrea K AU - Chernau AK AD - Rocky Vista University College of Osteopathic Medicine, Parker, Colorado. FAU - Leone, Paolo M AU - Leone PM AD - Fondazione Policlinico Universitario A. Gemelli IRCCS, Roma, Italy. AD - Universita Cattolica del Sacro Cuore, Roma, Italy. AD - Interstitial Lung Disease Program, National Jewish Health, Denver, Colorado. FAU - Swigris, Jeffrey J AU - Swigris JJ AD - Interstitial Lung Disease Program, National Jewish Health, Denver, Colorado. LA - eng PT - Journal Article PT - Review DEP - 20190528 PL - United States TA - Semin Respir Crit Care Med JT - Seminars in respiratory and critical care medicine JID - 9431858 SB - IM MH - *Autoimmunity MH - Humans MH - Lung Diseases, Interstitial/classification/complications/diagnosis/*immunology MH - Undifferentiated Connective Tissue Diseases/complications/diagnosis/*immunology COIS- None declared. EDAT- 2019/05/29 06:00 MHDA- 2020/04/30 06:00 CRDT- 2019/05/29 06:00 PHST- 2019/05/29 06:00 [entrez] PHST- 2019/05/29 06:00 [pubmed] PHST- 2020/04/30 06:00 [medline] AID - 10.1055/s-0039-1684007 [doi] PST - ppublish SO - Semin Respir Crit Care Med. 2019 Apr;40(2):271-277. doi: 10.1055/s-0039-1684007. Epub 2019 May 28.