Centre for Rare Diseases - Disorders of Iron Metabolism - ASST-Monza, Centre of European Reference Network (EuroBloodNet), San Gerardo Hospital Monza, Monza, Italy.
Department of Medicine and Surgery, University of Milano-Bicocca, Monza, Italy.
Liver Int. 2021 Jul;41(7):1600-1607. doi: 10.1111/liv.14874. Epub 2021 Mar 22.
BACKGROUND & AIMS: Inhalation of welding fume may cause pulmonary disease known as welder's lung. At our centre we came across a number of welders with systemic iron overload and prolonged occupational history and we aimed at characterizing this novel clinical form of iron overload.
After exclusion of other known causes of iron overload, 20 welders were fully evaluated for working history, hepatic, metabolic and iron status. MRI iron assessment was performed in 19 patients and liver biopsy in 12. We included 40 HFE-HH patients and 24 healthy controls for comparison.
75% of patients showed lung HRCT alterations; 90% had s-FERR > 1000 ng/mL and 60% had TSAT > 45%. Liver iron overload was mild in 8 and moderate-severe in 12. The median iron removed was 7.8 g. Welders showed significantly lower TSAT and higher SIS and SIS/TIS ratio than HFE-HH patients. Serum hepcidin was significantly higher in welders than in HFE-HH patients and healthy controls. At liver biopsy, 50% showed liver fibrosis that was mild in four, and moderate-severe in two. Liver staging correlated with liver iron overload.
Welders with prolonged fume exposure can develop severe liver iron overload. The mechanism of liver iron accumulation is quite different to that of HFE-HH suggesting that reticuloendothelial cells may be the initial site of deposition. We recommend routine measurement of serum iron indices in welders to provide adequate diagnosis and therapy, and the inclusion of prolonged welding fume exposure in the list of acquired causes of hyperferritinemia and iron overload.
吸入焊接烟尘可能导致一种称为焊工肺的肺部疾病。在我们中心,遇到了一些具有系统性铁过载和长期职业史的焊工,我们旨在描述这种新型铁过载的临床形式。
在排除其他已知的铁过载原因后,对 20 名焊工进行了全面的工作史、肝脏、代谢和铁状态评估。对 19 名患者进行了 MRI 铁评估,对 12 名患者进行了肝活检。我们纳入了 40 名 HFE-HH 患者和 24 名健康对照者进行比较。
75%的患者出现肺部 HRCT 改变;90%的患者 s-FERR>1000ng/ml,60%的患者 TSAT>45%。肝脏铁过载程度为轻度 8 例,中重度 12 例。中位数去除铁量为 7.8g。焊工的 TSAT 明显低于 HFE-HH 患者,SIS 和 SIS/TIS 比值明显高于 HFE-HH 患者。血清铁调素在焊工中明显高于 HFE-HH 患者和健康对照组。肝活检显示 50%的患者有肝纤维化,其中 4 例为轻度,2 例为中重度。肝分期与肝脏铁过载相关。
长期暴露于烟尘的焊工可能会发生严重的肝脏铁过载。肝脏铁蓄积的机制与 HFE-HH 患者明显不同,提示网状内皮细胞可能是铁沉积的初始部位。我们建议对焊工常规检测血清铁指标,以提供充分的诊断和治疗,并将长期接触焊接烟尘纳入高血清铁蛋白血症和铁过载的获得性病因列表中。