PMID- 28807021 OWN - NLM STAT- MEDLINE DCOM- 20180508 LR - 20220331 IS - 1471-2466 (Electronic) IS - 1471-2466 (Linking) VI - 17 IP - 1 DP - 2017 Aug 14 TI - Serological and morphological prognostic factors in patients with interstitial pneumonia with autoimmune features. PG - 111 LID - 10.1186/s12890-017-0453-z [doi] LID - 111 AB - BACKGROUND: To identify the prognostic factors for survival in patients with interstitial pneumonia with autoimmune features (IPAF) who meet the serological domain of the IPAF criteria. METHODS: We retrospectively analysed 99 IPAF patients who met the serological domain and were hospitalised at the Respiratory Medicine Unit of Kurashiki Central Hospital from 1999 to 2015. The high-resolution computed tomography findings were usual interstitial pneumonia (UIP; n = 1), non-specific interstitial pneumonia (NSIP; n = 63), NSIP with organizing pneumonia (OP) overlap (n = 15), and OP (n = 20). One patient who had radiological UIP pattern, and met the serological and clinical domains was excluded. The clinical characteristics, radiological findings, administered therapy, and prognosis of the remaining 98 IPAF patients who met the serological and morphological domains were analysed. RESULTS: The median age of the 98 IPAF patients was 68 years, and 41 (41.8%) of them were men. Twelve (12.2%) of the 98 IPAF patients developed other characteristics and were diagnosed with connective tissue disease (CTD) later during the median follow-up of 4.5 years. Univariate Cox analysis revealed systemic sclerosis (SSc)-specific and SSc-associated antibodies (ANA nucleolar pattern, ANA centromere pattern, anti-ribonucleoprotein and anti-Scl-70) positive IPAF, radiological NSIP pattern, bronchoalveolar lavage fluid lymphocytes >15%, and age as significant prognostic factors for survival. Multivariate Cox analysis revealed radiological NSIP pattern (hazard ratio [HR], 4.48; 95% confidence interval [CI], 1.28-15.77, p = 0.02) and age (HR, 1.07; 95% CI, 1.02-1.11, p = 0.01) were significantly associated with worse survival. CONCLUSIONS: We confirmed that radiological NSIP pattern and age are poor prognostic factors for the survival of IPAF patients. This study suggested that the autoantibodies that are highly specific for certain connective tissue diseases might be less important for the prognosis of IPAF compared with the radiological-pathological patterns. The relatively high proportion of IPAF patients who developed CTD later suggests the importance of careful observation for evolution to CTD in IPAF. FAU - Ito, Yuhei AU - Ito Y AD - Department of Respiratory Medicine, Kurashiki Central Hospital, Kurashiki, Japan. yi14402@gmail.com. FAU - Arita, Machiko AU - Arita M AD - Department of Respiratory Medicine, Kurashiki Central Hospital, Kurashiki, Japan. FAU - Kumagai, Shogo AU - Kumagai S AD - Department of Respiratory Medicine, Kurashiki Central Hospital, Kurashiki, Japan. FAU - Takei, Reoto AU - Takei R AD - Department of Respiratory Medicine, Kurashiki Central Hospital, Kurashiki, Japan. FAU - Noyama, Maki AU - Noyama M AD - Department of Respiratory Medicine, Kurashiki Central Hospital, Kurashiki, Japan. FAU - Tokioka, Fumiaki AU - Tokioka F AD - Department of Respiratory Medicine, Kurashiki Central Hospital, Kurashiki, Japan. FAU - Nishimura, Keisuke AU - Nishimura K AD - Department of Endocrinology and Rheumatology, Kurashiki Central Hospital, Kurashiki, Japan. FAU - Koyama, Takashi AU - Koyama T AD - Department of Radiology, Kurashiki Central Hospital, Kurashiki, Japan. FAU - Notohara, Kenji AU - Notohara K AD - Department of Pathology, Kurashiki Central Hospital, Kurashiki, Japan. FAU - Ishida, Tadashi AU - Ishida T AD - Department of Respiratory Medicine, Kurashiki Central Hospital, Kurashiki, Japan. LA - eng PT - Journal Article DEP - 20170814 PL - England TA - BMC Pulm Med JT - BMC pulmonary medicine JID - 100968563 RN - 0 (Antibodies, Antinuclear) RN - 0 (Jo-1 antibody) RN - 0 (Nuclear Proteins) RN - 0 (Peptides, Cyclic) RN - 0 (Ribonucleoproteins) RN - 0 (Scl 70 antigen, human) RN - 0 (anticentromere antibody) RN - 0 (cyclic citrullinated peptide) RN - EC 5.99.1.2 (DNA Topoisomerases, Type I) SB - IM MH - Aged MH - Antibodies, Antinuclear/immunology MH - Autoimmune Diseases/diagnostic imaging/*immunology MH - Cryptogenic Organizing Pneumonia/diagnostic imaging/immunology MH - DNA Topoisomerases, Type I MH - Female MH - Humans MH - Idiopathic Pulmonary Fibrosis/diagnostic imaging/immunology MH - Lung Diseases, Interstitial/diagnostic imaging/*immunology MH - Male MH - Middle Aged MH - Multivariate Analysis MH - Nuclear Proteins/immunology MH - Peptides, Cyclic/immunology MH - Prognosis MH - Proportional Hazards Models MH - Retrospective Studies MH - Ribonucleoproteins/immunology MH - Tomography, X-Ray Computed PMC - PMC5554971 OTO - NOTNLM OT - Autoimmune disease OT - Collagen vascular disease OT - Interstitial lung disease OT - Interstitial pneumonia with autoimmune features OT - Systemic sclerosis COIS- CONSENT FOR PUBLICATION: Not applicable. COMPETING INTERESTS: All authors declare they have no competing interests. PUBLISHER'S NOTE: Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations. EDAT- 2017/08/16 06:00 MHDA- 2018/05/09 06:00 PMCR- 2017/08/14 CRDT- 2017/08/16 06:00 PHST- 2017/01/29 00:00 [received] PHST- 2017/07/31 00:00 [accepted] PHST- 2017/08/16 06:00 [entrez] PHST- 2017/08/16 06:00 [pubmed] PHST- 2018/05/09 06:00 [medline] PHST- 2017/08/14 00:00 [pmc-release] AID - 10.1186/s12890-017-0453-z [pii] AID - 453 [pii] AID - 10.1186/s12890-017-0453-z [doi] PST - epublish SO - BMC Pulm Med. 2017 Aug 14;17(1):111. doi: 10.1186/s12890-017-0453-z.