Prognosis of connective tissue disease related interstitial lung disease after initiation of long-term oxygen therapy: comparison with idiopathic pulmonary fibrosis

Author:

Ishiwari Mayuko1,Kono Yuta1,Togashi Yuki1,Kobayashi Kenichi1,Kikuchi Ryota1,Kogami Mariko1,Abe Shinji1

Affiliation:

1. Tokyo Medical University Hospital

Abstract

Abstract

Background The studies of long-term oxygen therapy (LTOT) for patents with connective tissue disease-related interstitial lung disease (CTD-ILD) are limited. This study aimed to investigate the prognosis of patients with CTD-ILD after the initiation of LTOT, compared to those with idiopathic pulmonary fibrosis (IPF). Methods We retrospectively investigated patients with CTD-ILD and IPF who were introduced to LTOT between January 2014 and December 2020. Results The study included 24 patients with CTD-ILD and 55 patients with IPF. Female gender, never-smoking history, higher body mass index (BMI), higher lactate dehydrogenase (LDH) level, lower pulmonary Surfactant Protein-D (SP-D) level and lower Gender-Age-Physiology (GAP) scores were more common in the CTD-ILD group (all < 0.05). The time from interstitial lung disease (ILD) diagnosis to LTOT initiation for patients with CTD-ILD was significantly longer than for patients with IPF (36.0 vs 23.5 months, p = 0.028). Log-rank tests showed that patients with CTD-ILD had significantly longer survival compared to IPF patients after the diagnosis of ILD (p < 0.001). However, no significant difference in survival after the initiation of LTOT was noted between patients with CTD-ILD and IPF (p = 0.276). Conclusion Although patients with CTD-ILD had longer overall survival than those with IPF, there was no significant difference in prognosis after the initiation of LTOT between the two groups.

Publisher

Springer Science and Business Media LLC

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