Baseline Characteristics and Survival of an Australian Interstitial Pneumonia with Autoimmune Features Cohort

被引:12
|
作者
Jee, Adelle S. [1 ,2 ,3 ]
Parker, Matthew J. S. [2 ,3 ,4 ]
Bleasel, Jane F. [2 ,4 ]
Troy, Lauren K. [1 ,2 ]
Lau, Edmund M. [1 ,2 ]
Jo, Helen E. [1 ,2 ,3 ]
Teoh, Alan K. Y. [1 ,2 ,3 ]
Adelstein, Stephen [2 ,5 ,6 ]
Webster, Susanne [1 ]
Corte, Tamera J. [1 ,2 ,3 ]
机构
[1] Royal Prince Alfred Hosp, Dept Resp, Sydney, NSW, Australia
[2] Univ Sydney, Cent Clin Sch, Sydney, NSW, Australia
[3] Natl Hlth & Med Res Council NHMRC, Ctr Res Excellence Pulm Fibrosis, Sydney, NSW, Australia
[4] Royal Prince Alfred Hosp, Dept Rheumatol, Sydney, NSW, Australia
[5] NSW Hlth Pathol, Cent Immunol Lab, Sydney, NSW, Australia
[6] Royal Prince Alfred Hosp, Dept Clin Immunol & Allergy, Sydney, NSW, Australia
基金
英国医学研究理事会;
关键词
Interstitial lung disease; Connective tissue disease; Autoimmune diseases; Pulmonary fibrosis associated with collagen vascular disorders; Clinical research;
D O I
10.1159/000515396
中图分类号
R56 [呼吸系及胸部疾病];
学科分类号
摘要
Background and objective: The research term "interstitial pneumonia with autoimmune features" (IPAF) encompasses interstitial lung disease (ILD) patients with autoimmune features not meeting diagnostic criteria for a defined connective tissue disease (CTD). It remains unclear if IPAF is a distinct disease entity with implications for management and prognosis. We describe an Australian IPAF population and compare their baseline characteristics and outcomes with distinct cohorts of idiopathic interstitial pneumonia (IIP), CTD-ILD, and unclassifiable ILD. Methods: Review of 291 consecutive patients attending a specialist ILD clinic was performed. Patients with a diagnosis of IIP, CTD-ILD, and unclassifiable ILD by ILD-multidisciplinary meeting (ILD-MDM) were included. Patients meeting the IPAF criteria were identified. Baseline clinical data, survival, and progression were compared between ILD groups. Results: 226 patients were included, 36 meeting the IPAF criteria. IPAF patients demonstrated a high prevalence of autoantibodies to tRNA synthetase (35.3%), Ro52 (27.8%), and neutrophilic cytoplasmic antigens (ANCA; 20.0%). IPAF and CTD-ILD patients demonstrated similar clinical characteristics (mean age 66.6 and 63.7 years, respectively, female predominant, frequent CTD-manifestations). Lung function did not differ between ILD groups. Disease severity, pulmonary hypertension (PH), and ILD-MDM diagnosis were strong predictors of worse transplant-free survival (TFS). Meeting the IPAF criteria was not associated with TFS. Conclusions: We identified IPAF as a heterogeneous phenotype that overlaps considerably with CTD-ILD. Disease severity, PH, and ILD-MDM diagnosis were more powerful predictors of survival outcomes than meeting the IPAF criteria.
引用
收藏
页码:853 / 864
页数:12
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