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IPAF should receive early treatment for sharing similar clinical characteristics as CTD-ILD: a report from 273 Chinese patients

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Abstract

Objectives

The clinical characteristics of interstitial pneumonia with autoimmune features (IPAF) and connective tissue disease interstitial lung disease (CTD-ILD) have not been adequately compared. We compared the clinical characteristics of these two conditions and analyzed the changes in lung function before and after treatment of IPAF.

Methods

A total of 412 patients were enrolled in the study, and their clinical characteristics were assessed. The treatment-related changes in 12 cases of IPAF were analyzed.

Results

Complete clinical data were available for 126 patients with CTD-ILD and 147 with IPAF. All IPAF patients showed autoantibody positivity. The proportion of patients showing extrapulmonary symptoms in the CTD-ILD group was higher than that in the IPAF group (P < 0.05). Patients with IPAF demonstrated lower P(A-a)O2 and higher PaO2 and PaCO2 than those with CTD-ILD (P < 0.05 for all comparisons). Forced vital capacity (FVC) and diffusing capacity of the lungs for carbon monoxide (DLCO) in IPAF patients were higher than those in CTD-ILD patients (P = 0.023 for FVC; P = 0.011 for DLCO). Among patients with IPAF, only the proportions of honeycombing and nodules were lower than those in CTD-ILD patients (P < 0.05). Both FVC and DLCO values increased after treatment in patients with IPAF (P < 0.05).

Conclusion

IPAF showed autoantibody positivity and similar computed tomography (CT) findings as CTD-ILD, and lung function in patients with IPAF improved after immunosuppressive treatment, indicating that IPAF should receive attention and early immunosuppressive treatment like CTD-ILD, even though IPAF exhibits no extrapulmonary symptoms.

Key Points

• Clinical characteristics of IPAF

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Acknowledgments

I would like to thank those colleagues who were not included in the author list but give many help, including YanHong Wang, ChunMei Fan, Jia Li, Xin Luo, Xin Zhang, Yan Zheng, and YingMing Xu.

Funding

This study was funded by the National Nature Science Foundation Key Research Project of China (2017YFC0909002).

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Author notes

  1. Ying Li and ZhaoHui Zheng contributed equally to this work.

Authors and Affiliations

  1. Department of Clinical Immunology, Institute of Rheumatism & Immunity, PLA, Branch of Immune Cell Biology, State Key Discipline of Cell Biology, Xijing Hospital, Fourth Military Medical University, No. 127 West Changle Road, Xi’an, 710032, Shaanxi Province, People’s Republic of China

    Ying Li, ZhaoHui Zheng, Qing Han, ZhiQin Li, RongHua Xie, Rui Zhang, Bei Zhang & Ping Zhu

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  1. Ying Li
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  2. ZhaoHui Zheng
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Correspondence to Ping Zhu.

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All authors have read and approved the content, and agree to submit it for consideration for publication in your journal, and agree to bear the applicable publication charges if our manuscript is accepted for publication. There are no ethical/legal conflicts involved in the article. The authors have declared no conflicts of interest.

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The study was performed according to the 1997 Declaration of Helsinki of the World Medical Association and has been approved by the ethics committee of each participating center.

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Li, Y., Zheng, Z., Han, Q. et al. IPAF should receive early treatment for sharing similar clinical characteristics as CTD-ILD: a report from 273 Chinese patients. Clin Rheumatol 39, 3817–3823 (2020). https://doi.org/10.1007/s10067-020-05149-6

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  • DOI: https://doi.org/10.1007/s10067-020-05149-6

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