Pirfenidone在具有和不具有自體免疫抗體的特發性肺纖維化患者中的療效比較

Comparative Efficacy of Pirfenidone in Patients of Idiopathic Pulmonary Fibrosis with and without Autoantibodies

目的：計抗纖維化藥物是過去十年中特發性肺纖維化(idiopathic pulmonary fibrosis, IPF)患者新發展的治療選項之一。Pirfenidone則是其中一種抗纖維化藥物。本篇研究是希望探究在同樣是IPF患者且使用Pirfenidone藥物，有無自體免疫抗體是否會影響其預後。
方法：本篇研究收錄了從2019年1月至2020年12月，來自台灣四間醫院的符合最新診斷標準的IPF患者，並根據其確診時的自體免疫抗體血清學檢查分成陽性組及陰性組。所有的病人都在至少一年的追蹤期中使用Pirfenidone。根據其在一年中追蹤是否用力肺活量(forced vital capacity, FVC)下降大於原基準10%，急性惡化和死亡當作綜合不良預後指標，並利用多變數分析評估其危險因子。
結果：在50名收錄的IPF患者，11名是自體免疫血清抗體陽性，39名陰性。在最初進入試驗並診斷IPF的年齡並無差異(74.8±9.8歲vs 73.9±10.3歲, p=0.781)，一開始的呼吸困難量表評分(mMRC dyspnea scale)和用力肺活量(FVC)也都無差異。在經過一年使用Pirfenidone及追蹤後，其綜合不良預後指標個案數比例在抗體陽性組(佔63.3%)較抗體陰性組(佔38.5%)高，但其P值仍無達到顯著差異(p=0.178)。在多變數分析危險因子中，不管是血清抗體陽性、年齡、性別、抽菸甚至GAP index，皆沒有達到統計上顯著意義。
結論：在特發性肺纖維化( I P F )的患者中，不管有無帶有自體免疫抗體，並不影響其在使用Pirfenidone一年內的預後。

Purpose: Pirfenidone is one of the anti-fibrotic drugs that are newly developed treatment options for patients with idiopathic pulmonary fibrosis (IPF) over the past 10 years. Our study aimed to investigate whether there is a prognostic difference between patients of IPF with or without autoantibodies treated with pirfenidone.
Patients and Methods: Patients with IPF, diagnosed based on the“2018 ATS/ERS/JRS/ALAT guideline for IPF”, were enrolled from January 2019 to December 2020 in four hospitals in Taiwan and divided into those with and without autoantibodies. All patients received pirfenidone and were followed up for at least 1 year. The primary composite outcomes include a decline of≥10% in forced vital capacity (FVC), acute exacerbation, and all-cause mortality after receiving 1-year pirfenidone treatment. Multivariate logistic regression analysis for risk factors, including positive autoantibodies, was performed.
Results: Of the 50 patients with IPF enrolled, 11(22%) had positive autoantibodies, and 39 (78%) did not. The mean age of diagnosis and enrollment into the study of the two groups showed no difference (74.8±9.8 vs 73.9±10.3, p=0.781), nor did the modified Medical Research Council (mMRC) dyspnea scale (1.5±0.8 vs 1.8±0.7, p=0.188) or the FVC (67.6±10.6% vs 66.0±11.7%, p=0.679). After 1-year follow-up after pirfenidone treatment, the incidence of composite outcome of the autoantibody-positive group (63.3%) was higher than that of the opposing group (38.5%), although it is not statistically significant (p=0.178). The multivariate analysis for risk factors showed no significant statistical differences.
Conclusion: No significant prognostic difference was observed in patients of IPF with or without autoantibodies after receiving 1-year pirfenidone treatment.