Libra Alessandro, Muscato Giuseppe, Ielo Giuseppe, Spicuzza Lucia, Palmucci Stefano, Fagone Evelina, Fruciano Mary, Gili Elisa, Sambataro Gianluca, Vancheri Carlo
Regional Referral Centre for Rare Lung Disease, Department of Clinical and Experimental Medicine, University Hospital "Policlinico-San Marco", University of Catania, 95123 Catania, Italy.
Radiology Unit I, Department of Medical Surgical Sciences and Advanced Technologies "GF Ingrassia", University Hospital "Policlinico-San Marco", University of Catania, 95123 Catania, Italy.
Diagnostics (Basel). 2023 May 27;13(11):1882. doi: 10.3390/diagnostics13111882.
Perinuclear Anti Neutrophil Cytoplasmic Antibody (p-ANCA) is a serological marker of Microscopic Polyangiitis (MPA), a vasculitis associated with lung involvement potentially mimicking Idiopathic Pulmonary Fibrosis (IPF). In this study, we evaluated the role of p-ANCA in predicting clinical evolution and prognosis in a cohort of IPF patients. In this observational, retrospective, case-control study, we compared 18 patients with an IPF diagnosis and p-ANCA positivity with 36 patients with seronegative IPF, matched for age and sex. IPF patients with and without p-ANCA showed similar lung function decline during the follow-up, but IPF p-ANCA+ showed better survival. Half of IPF p-ANCA+ patients were classified as MPA for the development of renal involvement (55%) or skin signs (45%). The progression towards MPA was associated with high levels of Rheumatoid Factor (RF) at baseline. In conclusion, p-ANCA, mainly when associated with RF, could predict the evolution of Usual Interstitial Pneumonia (UIP) towards a definite vasculitis in patients, with a better prognosis compared with IPF. In this view, ANCA testing should be included in the diagnostic workup of UIP patients.
核周型抗中性粒细胞胞浆抗体（p-ANCA）是显微镜下多血管炎（MPA）的血清学标志物，MPA是一种与肺部受累相关的血管炎，可能酷似特发性肺纤维化（IPF）。在本研究中，我们评估了p-ANCA在预测一组IPF患者临床进展和预后中的作用。在这项观察性、回顾性、病例对照研究中，我们将18例诊断为IPF且p-ANCA阳性的患者与36例年龄和性别相匹配的血清阴性IPF患者进行了比较。有和没有p-ANCA的IPF患者在随访期间肺功能下降相似，但IPF p-ANCA阳性患者的生存率更高。一半的IPF p-ANCA阳性患者因出现肾脏受累（55%）或皮肤体征（45%）而被归类为MPA。向MPA的进展与基线时高水平的类风湿因子（RF）相关。总之，p-ANCA，主要是与RF相关时，可预测普通型间质性肺炎（UIP）患者向明确血管炎的演变，与IPF相比预后更好。据此，ANCA检测应纳入UIP患者的诊断检查中。
