Dell'era L, Boati E, Nebbia G, Corona F
Fondazione IRCCS Policlinico, Mangiagalli e Regina Elena, University of Milan, Milan, Italy.
Minerva Pediatr. 2012 Feb;64(1):55-7.
Systemic lupus erythematosus (SLE) has been reported to be associated to Wilson's disease, as a complication of treatment with penicillamine. Even though drug-induced lupus erythematosus (DILE) has some features in common with SLE, they are distinct entities. We report the case of a young girl who at the age of five had a diagnosis of Wilson's disease and she started therapy with penicillamine. Eight years after the beginning of therapy, she developed proteinuria, which was considered to be related to penicillamine. Two years later, she developed arthritis, malar rash and laboratory findings suggestive for lupus erythematosus. At the beginning her symptoms, due to the known association between penicillamine and DILE, were thought to be related to this treatment. In this hypothesis, she was referred to the Rheumatology Centre; zinc acetate was substituted for penicillamine and she started naproxen for the treatment of arthritis. Anyway, the subsequent clinical course and laboratory findings led us to a diagnosis of idiopathic SLE. A renal biopsy detected massive mesangiocapillary proliferation with subendothelial deposits (wire loops) and duplication of glomerular basement membrane (active diffuse global proliferative lupus nephritis, class IV G A). To our knowledge, this is the first report of an association between Wilson's disease and SLE.
据报道，系统性红斑狼疮（SLE）与威尔逊病有关，是青霉胺治疗的一种并发症。尽管药物性狼疮（DILE）与SLE有一些共同特征，但它们是不同的疾病实体。我们报告了一名年轻女孩的病例，她在5岁时被诊断为威尔逊病，并开始接受青霉胺治疗。治疗开始8年后，她出现蛋白尿，这被认为与青霉胺有关。两年后，她出现关节炎、蝶形红斑以及提示红斑狼疮的实验室检查结果。起初，由于已知青霉胺与DILE之间的关联，她的症状被认为与这种治疗有关。基于这一假设，她被转诊至风湿病中心；用醋酸锌替代了青霉胺，并开始使用萘普生治疗关节炎。然而，随后的临床病程和实验室检查结果使我们诊断为特发性SLE。肾脏活检发现大量系膜毛细血管增生伴内皮下沉积物（铁丝圈）和肾小球基底膜双轨征（活动性弥漫性全肾小球增生性狼疮性肾炎，IV级G A）。据我们所知，这是威尔逊病与SLE关联的首例报告。
