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.
Clin Rheumatol. 2020 Dec;39(12):3817-3823. doi: 10.1007/s10067-020-05149-6. Epub 2020 May 23.
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.
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.
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)O and higher PaO and PaCO than those with CTD-ILD (P < 0.05 for all comparisons). Forced vital capacity (FVC) and diffusing capacity of the lungs for carbon monoxide (D) in IPAF patients were higher than those in CTD-ILD patients (P = 0.023 for FVC; P = 0.011 for D). 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 D values increased after treatment in patients with IPAF (P < 0.05).
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.
自身免疫特征性间质性肺炎（IPAF）和结缔组织病相关性间质性肺疾病（CTD-ILD）的临床特征尚未得到充分比较。我们比较了这两种疾病的临床特征，并分析了 IPAF 治疗前后肺功能的变化。
共纳入 412 例患者，评估其临床特征。分析了 12 例 IPAF 的治疗相关变化。
CTD-ILD 组 126 例和 IPAF 组 147 例患者的完整临床资料均可用。所有 IPAF 患者均表现出自身抗体阳性。CTD-ILD 组出现肺外症状的患者比例高于 IPAF 组（P<0.05）。与 CTD-ILD 组相比，IPAF 组患者的肺泡-动脉血氧分压差（P(A-a)O）更低，动脉血氧分压（PaO）和动脉血二氧化碳分压（PaCO）更高（所有比较均 P<0.05）。IPAF 患者的用力肺活量（FVC）和一氧化碳弥散量（D）均高于 CTD-ILD 患者（FVC：P=0.023；D：P=0.011）。在 IPAF 患者中，只有蜂巢征和结节征的比例低于 CTD-ILD 患者（P<0.05）。IPAF 患者治疗后 FVC 和 D 值均增加（P<0.05）。
IPAF 表现出自身抗体阳性和与 CTD-ILD 相似的计算机断层扫描（CT）表现，且 IPAF 患者经免疫抑制治疗后肺功能改善，提示 IPAF 应像 CTD-ILD 一样受到重视并早期进行免疫抑制治疗，即使 IPAF 无肺外症状。
关键点
• IPAF 的临床特征。
