Ying Li1, ZhaoHui Zheng1, Qing Han1, ZhiQin Li1, RongHua Xie1, Rui Zhang1, Bei Zhang1, Ping Zhu2. 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. 2. 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. zhuping@fmmu.edu.cn.
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.
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 IPAFpatients 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 IPAFpatients were higher than those in CTD-ILDpatients (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-ILDpatients (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.
Entities:
Keywords:
Clinical characteristics; Connective tissue disease; Interstitial lung disease; Interstitial pneumonia with autoimmune features