Interstitial pneumonia with autoimmune features: A single center prospective follow-up study

被引:35
|
作者
Sebastiani, Marco [1 ]
Cassone, Giulia [1 ,2 ,3 ]
De Pasquale, Lisa [1 ]
Cerri, Stefania [4 ]
Della Casa, Giovanni [5 ]
Vacchi, Caterina [1 ]
Luppi, Fabrizio [4 ]
Salvarani, Carlo [1 ,3 ]
Manfredi, Andreina [1 ]
机构
[1] Univ Modena & Reggio Emilia, Azienda Osped Univ Policlin Modena, Chair & Rheumatol Unit, Modena, Italy
[2] Univ Modena & Reggio Emilia, Clin & Expt Med PhD Program, Modena, Italy
[3] Azienda USL IRCCS Reggio Emilia, Rheumatol Unit, Reggio Emilia, Italy
[4] Univ Modena & Reggio Emilia, Azienda Osped Univ Policlin Modena, Resp Dis Unit, Modena, Italy
[5] Univ Modena & Reggio Emilia, Azienda Osped Univ Policlin Modena, Radiol Unit, Modena, Italy
关键词
Connective tissue diseases (CTD); Interstitial lung disease (ILD); Interstitial pneumonia with autoimmune features (IPAF); Idiopathic pulmonary fibrosis (IPF); Survival; CONNECTIVE-TISSUE DISEASE; CLASSIFICATION CRITERIA; RHEUMATOID-ARTHRITIS; RHEUMATOLOGY/EUROPEAN LEAGUE; SYSTEMIC-SCLEROSIS; AMERICAN-COLLEGE; LUNG-DISEASE; SOCIETY; EXPERIENCE; DIAGNOSIS;
D O I
10.1016/j.autrev.2019.102451
中图分类号
R392 [医学免疫学]; Q939.91 [免疫学];
学科分类号
100102 ;
摘要
Background and objective: Recently the term "interstitial pneumonia with autoimmune features" (IPAF) has been proposed to identify patients with interstitial lung disease and autoimmune characteristics, not fulfilling the criteria for specific connective tissue diseases (CTD). Only few data are available about the clinical and serological features of IPAF patients, their survival and the possible evolution in a CTD. The aims of the study were to investigate the demographic and clinico-serologic features of patients with IPAF, their relationship to survival, and the possible evolution in a definite CTD. Patients and methods: Fifty-two patients were consecutively enrolled and prospectively followed for 45 +/- 31.6 months. Data about disease onset, serological, clinical and therapeutic features, pulmonary function tests and high-resolution computed tomography were periodically repeated. The survival of patients with IPAF was compared with that of 104 patients with idiopathic pulmonary fibrosis (IPF). Results: The clinical domain for IPAF was satisfied in 44 patients, serological domain in 49 and the morphological domain in 29 patients. During the follow-up, a definite CTD was diagnosed in 7 patients, in particular Sjogren's syndrome in 4 patients, rheumatoid arthritis in 2, and polymyositis in the last. The estimated 5-year survival of IPAF patients 69.5 +/- 7.8%, significantly higher than survival observed in IPF patients, and the baseline value of FVC and DLCO were the only factors associated to death. Conclusions: IPAF seems to a distinct entity, with a low tendency to evolve in a definite CTD. Nevertheless, further studies are needed to better define the clinical evolution and the outcome of IPAF.
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页数:6
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