RT Journal Article
SR Electronic
T1 Charactaristics and prognosis of autoimmune antibody-positive patients of idiopathic pulmonary arterial hypertension
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP PA3600
DO 10.1183/13993003.congress-2021.PA3600
VO 58
IS suppl 65
A1 Masashi Sakayori
A1 Ayumi Sekine
A1 Nobuhiro Tanabe
A1 Akira Naito
A1 Takayuki Jujo
A1 Toshihiko Sugiura
A1 Ayako Shigeta
A1 Seiichiro Sakao
A1 Koichiro Tatsumi
YR 2021
UL http://erj.ersjournals.com/content/58/suppl_65/PA3600.abstract
AB Background: High mortality of connective tissue diseases (CTDs)-associated pulmonary arterial hypertension (PAH); especially systemic sclerosis (SSc) has been reported. While patients with autoimmune-antibodies positive, clinically asymptomatic CTDs could be diagnosed as idiopathic pulmonary arterial hypertension (IPAH). However, the features of this group of IPAH remain unclear.Purpose: To clarify the prognosis and characteristics of autoimmune-antibody positive patients of IPAH.Subjects and methods: Fifty-eight patients diagnosed with IPAH at Chiba University Hospital; pulmonary hypertension expert center of east Japan, from June 1990 to April 2020. They were divided into autoimmune antibody positive (Au-Ab [+]) group and negative (Au-Ab [-]) group. We retrospectively analyzed patient’s background and prognosis between the two groups. Moreover, patients with SSc related antibody-positive (SSc-Ab [+]) group were separated among (Au-Ab [+]) group and analyzed in the same manner.Results: Both Au-Ab [+] group and SSc-Ab [+] group tended to show more older, and lower FEV1/FVC and DLco than Au-Ab [-] group. According to the Kaplan Meier method, there was no significant difference in survival between Au-Ab [+] group and Au-Ab [-] group, but SSc-Ab [+] group had a significantly worse survival than Au-Ab [-] group (Wilcoxon test, p = 0.049).Conclusion: Au-Ab [+] group of IPAH, especially SSc-Ab [+] group had specific characteristics compared to Au-Ab [-] group; and might result in poor prognosis among IPAH.FootnotesCite this article as: European Respiratory Journal 2021; 58: Suppl. 65, PA3600.This abstract was presented at the 2021 ERS International Congress, in session “Prediction of exacerbations in patients with COPD”.This is an ERS International Congress abstract. No full-text version is available. Further material to accompany this abstract may be available at www.ers-education.org (ERS member access only).