Abstract
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.
Footnotes
Cite 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).
- Copyright ©the authors 2021