Department of Paediatrics, Kamuzu Central Hospital, Lilongwe, Malawi.
Pediatr Nephrol. 2010 Aug;25(8):1543-6. doi: 10.1007/s00467-010-1470-9. Epub 2010 Feb 27.
A 14-year-old boy with known stable cystinosis, treated with cysteamine since infancy, presented with a deterioration of renal function with haematuria in conjunction with a nodular rash, arthralgia, leucopenia, hypocomplementaemia and raised antinuclear antibodies. He was diagnosed with spontaneous onset of systemic lupus erythematosus (SLE), and his renal biopsy was consistent with lupus nephritis. It is unusual for patients with one severe disease to develop another disease process completely unrelated to their original condition, but it can occur. However, other distinct variants of lupus have been described, including drug-induced lupus (DIL), which have features that over-lap with SLE. The potential differential diagnosis of the SLE as a form of DIL in association with cysteamine is discussed.
一名 14 岁男孩患有已知的稳定型胱氨酸病,自婴儿期以来一直接受半胱氨酸治疗,他出现肾功能恶化,伴有血尿、结节性皮疹、关节痛、白细胞减少、低补体血症和抗核抗体升高。他被诊断为自发性系统性红斑狼疮(SLE),他的肾活检与狼疮肾炎一致。患有一种严重疾病的患者会出现与原始疾病完全无关的另一种疾病过程并不常见,但这种情况确实会发生。然而,已经描述了其他不同类型的狼疮,包括药物诱导的狼疮(DIL),其特征与 SLE 重叠。讨论了 SLE 作为 DIL 的一种形式与半胱氨酸相关的潜在鉴别诊断。