PMID- 34019264 OWN - NLM STAT- MEDLINE DCOM- 20210525 LR - 20210525 IS - 0065-2598 (Print) IS - 0065-2598 (Linking) VI - 1304 DP - 2021 TI - Interstitial Lung Disease Associated with Connective Tissue Diseases. PG - 73-94 LID - 10.1007/978-3-030-68748-9_5 [doi] AB - Pulmonary manifestations of connective tissue diseases (CTD) carry high morbidity and potential mortality, and the most serious pulmonary type is interstitial lung disease (ILD). Identifying and promptly intervening CTD-ILD with immune suppressor therapy will change the natural course of the disease resulting in survival improvement. Compared to idiopathic pulmonary fibrosis, the most common presentation of idiopathic interstitial pneumonia (IIP), CTD-ILD carries a better prognosis due to the response to immune suppressor therapy. Nonspecific interstitial pneumonia (NSIP) is the most common type of CTD-ILD that is different from the fibrotic classical presentation of IPF, known as usual interstitial pneumonia (UIP). An exception is rheumatoid arthritis that presents more frequently with UIP type. Occasionally, IPF may not have typical radiographic features of UIP, and a full assessment to differentiate IPF from CTD-ILD is necessary, including the intervention of a multidisciplinary team and the histopathology. Interstitial pneumonia with autoimmune features (IPAF) shows promising advantages to identify patients with ILD who have some features of a CTD without a defined autoimmune disease and who may benefit from immune suppressors. A composition of clinical, serological, and morphologic features in patients presenting with ILD will fulfill criteria for IPAF. In summary, the early recognition and treatment of CTD-ILD, differentiation from IPF-UIP, and identification of patients with IPAF fulfill the assessment by the clinician for an optimal care. FAU - Peredo, Ruben A AU - Peredo RA AD - Division of Rheumatology, Department of Medicine, Albany Medical College, Albany, NY, USA. peredor@amc.edu. FAU - Mehta, Vivek AU - Mehta V AD - Rheumatology, Alaska Native Medical Center, Anchorage, AK, USA. FAU - Beegle, Scott AU - Beegle S AD - Division of Pulmonary & Critical Care Medicine, Albany Medical College, Albany, NY, USA. LA - eng PT - Journal Article PL - United States TA - Adv Exp Med Biol JT - Advances in experimental medicine and biology JID - 0121103 SB - IM MH - *Connective Tissue Diseases/complications/diagnosis MH - Humans MH - *Idiopathic Pulmonary Fibrosis/complications/diagnosis MH - Lung MH - *Lung Diseases, Interstitial/complications/diagnosis MH - Tomography, X-Ray Computed OTO - NOTNLM OT - Connective tissue disease OT - Interstitial lung disease OT - Interstitial pneumonia with autoimmune features EDAT- 2021/05/22 06:00 MHDA- 2021/05/26 06:00 CRDT- 2021/05/21 12:30 PHST- 2021/05/21 12:30 [entrez] PHST- 2021/05/22 06:00 [pubmed] PHST- 2021/05/26 06:00 [medline] AID - 10.1007/978-3-030-68748-9_5 [doi] PST - ppublish SO - Adv Exp Med Biol. 2021;1304:73-94. doi: 10.1007/978-3-030-68748-9_5.