METHODS: Patients diagnosed with ANCA-IIP and those with IPAF were analyzed in a retrospective study of ILD. The clinical outcomes were determined through pulmonary function tests (PFTs) after a one-year follow-up, as well as assessing all-cause mortality.
RESULTS: 27 patients with ANCA-IIP and 143 patients with IPAF were analyzed from a cohort of 995 patients with ILD. Patients in the ANCA-IIP group had an older age and a high proportion of males compared to those in the IPAF group. PFT results at baseline were similar between the two groups, except for a better FEV1% in the ANCA-IIP group. Glucocorticoid and immunosuppressive therapy improved pulmonary function in patients with IPAF, but it continued to deteriorate after one year of treatment in the ANCA-IIP group. Furthermore, the all-cause mortality rate was significantly higher in the ANCA-IIP group than in the IPAF group (22.2% vs. 6.3%, P = 0.017).
CONCLUSIONS: The responses to glucocorticoid and immunosuppressive therapy differ between the ANCA-IIP and IPAF groups, leading to divergent prognoses. Therefore, it is inappropriate to classify ANCA-IIP as part of IPAF.
方法:在一项ILD的回顾性研究中分析了诊断为ANCA-IIP和IPAF的患者。随访一年后通过肺功能测试(PFTs)确定临床结果,以及评估全因死亡率。
结果:从995例ILD患者中分析了27例ANCA-IIP患者和143例IPAF患者。与IPAF组相比,ANCA-IIP组的患者年龄更大,男性比例高。两组基线时的PFT结果相似,除了在ANCA-IIP组中更好的FEV1%。糖皮质激素和免疫抑制治疗可改善IPAF患者的肺功能,但ANCA-IIP组治疗1年后继续恶化.此外,ANCA-IIP组的全因死亡率明显高于IPAF组(22.2%vs.6.3%,P=0.017)。
结论:ANCA-IIP组和IPAF组对糖皮质激素和免疫抑制治疗的反应不同,导致不同的预后。因此,将ANCA-IIP归类为IPAF的一部分是不合适的。