PDF(Pubmed)
Abstract：
BACKGROUND: We proposed the term \"UIPAF\" to define patients with Usual Interstitial Pneumonia (UIP) associated with only one domain of the classification called \"Interstitial Pneumonia with Autoimmune Features\" (IPAF). The objective of this study was to evaluate the clinical presentation and prognosis of UIPAF patients, compared with two cohorts, composed of IPAF and idiopathic pulmonary fibrosis (IPF) patients, respectively.
METHODS: The patients were enrolled as IPAF, UIPAF, or IPF based on clinical, serological, and radiological data and evaluated by a multidisciplinary team.
RESULTS: We enrolled 110 patients with IPF, 69 UIPAF, and 123 IPAF subjects. UIPAF patients were similar to IPAF regarding autoimmune features, except for the prevalence of Rheumatoid Factor in UIPAF and anti-SSA in IPAF. A similar proportion of the two cohorts progressed toward a specific autoimmune disease (SAD), with differences in the kind of SAD developed. The real-life management and prognosis of UIPAF patients proved to be almost identical to IPF.
CONCLUSIONS: UIPAF shared with IPAF similar autoimmune features, suggesting the opportunity to be considered IPAF, excluding the morphological domain by the classification. However, the real-life management and prognosis of UIPAF are similar to IPF. These data suggest a possible modification in the therapeutic management of UIPAF.
METHODS: The patients were enrolled as IPAF, UIPAF, or IPF based on clinical, serological, and radiological data and evaluated by a multidisciplinary team.
RESULTS: We enrolled 110 patients with IPF, 69 UIPAF, and 123 IPAF subjects. UIPAF patients were similar to IPAF regarding autoimmune features, except for the prevalence of Rheumatoid Factor in UIPAF and anti-SSA in IPAF. A similar proportion of the two cohorts progressed toward a specific autoimmune disease (SAD), with differences in the kind of SAD developed. The real-life management and prognosis of UIPAF patients proved to be almost identical to IPF.
CONCLUSIONS: UIPAF shared with IPAF similar autoimmune features, suggesting the opportunity to be considered IPAF, excluding the morphological domain by the classification. However, the real-life management and prognosis of UIPAF are similar to IPF. These data suggest a possible modification in the therapeutic management of UIPAF.
摘要：
背景：我们提出了术语“UIPAF”来定义通常间质性肺炎（UIP）患者，仅与“具有自身免疫特征的间质性肺炎”（IPAF）分类的一个领域相关。这项研究的目的是评估UIPAF患者的临床表现和预后，与两个队列相比，由IPAF和特发性肺纤维化（IPF）患者组成，分别。
方法：将患者纳入IPAF，UIPAF,或基于临床的IPF，血清学,和放射学数据，并由多学科小组进行评估。
结果：我们招募了110例IPF患者，69UIPAF，和123名IPAF受试者。UIPAF患者在自身免疫特征方面与IPAF相似，除了类风湿因子在UIPAF中的流行和抗SSA在IPAF中的流行。两个队列中相似的比例进展为特定的自身免疫性疾病（SAD），随着SAD种类的不同而发展。UIPAF患者的现实生活管理和预后被证明与IPF几乎相同。
结论：UIPAF与IPAF具有相似的自身免疫特征，建议有机会被认为是IPAF，通过分类排除形态域。然而,UIPAF的实际管理和预后与IPF相似.这些数据表明UIPAF的治疗管理可能有所改变。
方法：将患者纳入IPAF，UIPAF,或基于临床的IPF，血清学,和放射学数据，并由多学科小组进行评估。
结果：我们招募了110例IPF患者，69UIPAF，和123名IPAF受试者。UIPAF患者在自身免疫特征方面与IPAF相似，除了类风湿因子在UIPAF中的流行和抗SSA在IPAF中的流行。两个队列中相似的比例进展为特定的自身免疫性疾病（SAD），随着SAD种类的不同而发展。UIPAF患者的现实生活管理和预后被证明与IPF几乎相同。
结论：UIPAF与IPAF具有相似的自身免疫特征，建议有机会被认为是IPAF，通过分类排除形态域。然而,UIPAF的实际管理和预后与IPF相似.这些数据表明UIPAF的治疗管理可能有所改变。
