Ty-Jour T1 - 慢性铍病:Azathioprine作为皮质类固醇治疗的替代品JF - 欧洲呼吸杂志Jo - Eur Respir J SP - 234 LP - 236 Do - 10.1183 / 09031936.00095712 VL - 41是 - 1 Au - Salvator,HélèneAU- Gille,Thomas Au - Hervé,AurélieAu - Bron,Camille Au - Lamberto,Christine Au - Valeyre,Dominique Y1 - 2013/01/01 UR - //www.qdcxjkg.com/content/41/1/234.Abstract N2 - 编辑:慢性铍病(CBD)是一种主要影响肺部的慢性肉芽肿疾病。它在遗传易感个体中的铍暴露后发生,最常见的HLA-DPβ1(Glu69)多态性[1]。铍颗粒慢慢地冲出,导致疾病的延迟持续,在接触铍[2,3]后长期爆发。CBD的临床,放射性和病理介绍与顺序病得非常相似。因此,误诊并不罕见,如Fireman等人所述。[4]和Müller-Quernheim等人。[5]。这些作者设法在4-6%的患者中纠正慢性结节病到CBD的诊断,因为仔细回顾铍暴露[4,5]。在CBD,肺部受到弥漫性的非碳粉颗粒状肿块,这可能导致纤维化。美国铍案件登记处确定了以下特定标准的CBD诊断:铍曝光史; relevant clinical and radiological signs (breathlessness, reduced pulmonary capacity and diffuse interstitial opacities); evidence of beryllium sensitisation with positive beryllium lymphocyte proliferation test in blood or in bronchoalveolar lavage (BAL); and histopathological features such as noncaseating granulomas or mononuclear tissue infiltration without any infection. Long-term prognosis of CBD is poor with a mortality rate ranging from 5.8% … ER -