Idiopathic lung fibrosis and anti myeloperoxidase glomerulonephritis: the tree that hides the forest
Idiopathic lung fibrosis and anti myeloperoxidase glomerulonephritis: the tree that hides the forest
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DOI：
10.1186/s12890-015-0129-5
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发表时间：
2015-10-26
影响因子：
3.1
通讯作者：
Hertig, Alexandre
中科院分区：
文献类型：
--
作者：
de Chambrun, Marc Pineton;Nunes, Hilario;Hertig, Alexandre
背景资料：虽然抗中性粒细胞胞浆抗体[ANCA]经常发现在诊断为特发性肺纤维化[IPF]的患者，目前的指南不建议血清学检测vasculitis.Case介绍：一个71岁的白人男性，诊断为IPF三年前，提出了快速进行性肾小球肾炎。ANCA在当前和历史血清中均被发现。采集肾脏活检样本，结果显示免疫缺陷性肾小球肾炎，但也显示肾小球纤维化区域，因此强烈提示其既往存在未识别的惰性血管炎发作。这使得“特发性”肺纤维化的诊断非常不可能。结论：作为肾脏病学家，我们认为，测试ANCA应进行系统的基础上，至少在老年患者，即使在没有肺外的迹象，血管炎的介绍。
Background: Although anti-neutrophil cytoplasmic antibodies [ANCA] are frequently found in patients diagnosed with idiopathic pulmonary fibrosis [IPF], current guidance does not recommend serologic testing for vasculitis.Case presentation: A 71-year old Caucasian male, diagnosed with IPF three years earlier, presented with rapidly progressive glomerulonephritis. ANCA were found both in current and historical sera. A kidney biopsy sample was taken, which revealed a pauci-immune glomerulonephritis, but also areas of glomerular fibrosis, hence strongly suggesting unrecognized flares of an indolent vasculitis in his past. This made the diagnosis of "idiopathic" pulmonary fibrosis very unlikely.Conclusion: As nephrologists, we argue that testing for ANCA should be performed on a systematic basis, at least in elderly patients, even in the absence of extra-pulmonary signs of vasculitis at presentation.