Tomassetti Sara, Ravaglia Claudia, Puglisi Silvia, Wells Athol U, Ryu Jay H, Bosi Marcello, Dubini Alessandra, Piciucchi Sara, Girelli Francesco, Parronchi Paola, Lavorini Federico, Rosi Elisabetta, Luzzi Valentina, Cerinic Marco Matucci, Poletti Venerino
Department of Experimental and Clinical Medicine, Careggi University Hospital, Florence, Italy.
Interventional Pulmonology Unit, Careggi University Hospital, Florence, Italy.
Front Med (Lausanne). 2023 Feb 16;10:1087485. doi: 10.3389/fmed.2023.1087485. eCollection 2023.
A subgroup of IPF patients can meet IPAF criteria (features suggesting an underlying autoimmune process without fulfilling established criteria for a CTD). This study was aimed to evaluate whether IPAF/IPF patients compared to IPF patients differ in clinical profile, prognosis and disease course.
This is a retrospective, single center, case-control study. We evaluated 360 consecutive IPF patients (Forlì Hospital, between 1/1/2002 and 28/12/2016) and compared characteristics and outcome of IPAF/IPF to IPF.
Twenty-two (6%) patients met IPAF criteria. IPAF/IPF patients compared to IPF were ( = 9/22, 40.9% vs. = 68/338, 20.1%, = 0.02), suffered more frequently from gastroesophageal reflux (54.5% vs. 28.4%, = 0.01), and showed a higher prevalence of (86.4% vs. 4.8%, < 0.0001), (14.3% vs. 0.3%, = 0.001) and (18.2% vs. 1.9%, = 0.002). The serologic domain was detected in all cases (the most frequent were ANA in 17 and RF in nine cases) and morphologic domain (histology features) was positive in 6 out of 10 lung biopsies (lymphoid aggregates). Only patients with IPAF/IPF evolved to CTD at follow-up (10/22, 45.5%; six rheumatoid arthritis, one Sjögren's and three scleroderma). The presence of IPAF was a positive prognostic determinant (HR 0.22, 95% CI 0.08-0.61, = 0.003), whereas the isolated presence of circulating autoantibody did not impact prognosis (HR 1.00, 95% CI 0.67-1.49, = 0.99).
The presence of IPAF criteria in IPF has a major clinical impact correlating with the risk of evolution to full blown-CTD during follow-up and identifying a subgroup of patients with a better prognosis.
特发性肺纤维化（IPF）患者中的一个亚组可符合自身免疫性肺炎相关间质性肺疾病（IPAF）标准（提示存在潜在自身免疫过程，但未满足结缔组织病（CTD）的既定标准）。本研究旨在评估与IPF患者相比，IPAF/IPF患者在临床特征、预后和病程方面是否存在差异。
这是一项回顾性、单中心病例对照研究。我们评估了360例连续的IPF患者（弗利医院，2002年1月1日至2016年12月28日），并比较了IPAF/IPF与IPF的特征和结局。
22例（6%）患者符合IPAF标准。与IPF患者相比，IPAF/IPF患者男性比例更高（9/22，40.9% 对68/338，20.1%，P = 0.02），更频繁地患有胃食管反流（54.5% 对28.4%，P = 0.01），并且抗核抗体（ANA）（86.4% 对4.8%，P < 0.0001）、类风湿因子（RF）（14.3% 对0.3%，P = 0.001）和抗平滑肌抗体（ASMA）（18.2% 对1.9%，P = 0.002）的患病率更高。所有病例均检测到血清学指标（最常见的是17例ANA阳性和9例RF阳性），10例肺活检中有6例形态学指标（组织学特征）呈阳性（淋巴样聚集）。只有IPAF/IPF患者在随访中发展为CTD（10/22，45.5%；6例类风湿关节炎，1例干燥综合征和3例硬皮病）。IPAF的存在是一个阳性预后决定因素（风险比[HR] 0.22，95% 置信区间[CI] 0.08 - 0.61，P = 0.003），而单纯循环自身抗体的存在对预后无影响（HR 1.00，95% CI 0.67 - 1.49，P = 0.99）。
IPF中IPAF标准的存在具有重大临床影响，与随访期间发展为完全型CTD的风险相关，并识别出一个预后较好的患者亚组。
