Perrin Laurence, Giurgea Irina, Baudet-Bonneville Valérie, Deschênes Georges, Bensman Albert, Ulinski Tim
Department of Paediatric Nephrology, Hôpital Trousseau, Paris, France.
Acta Paediatr. 2006 Jan;95(1):121-4. doi: 10.1080/08035250500325090.
Acute pancreatitis (AP) rarely complicates the clinical course of systemic lupus erythematosus (SLE). AP as the initial manifestation of SLE is exceptional, but its outcome is often fatal. Corticosteroids have been suspected to play a role in the development of AP, but the therapeutic benefit seems to be far above the risk of exacerbation of pancreatic lesions. We report a 13-y-old girl presenting with arthralgia and malaise, followed by abdominal pain, generalized oedema and haemodynamic instability. Increased CRP (325 ng/ml), serum amylase (14,000 IU/l) and lipase (2500 IU/l) levels suggested AP. Acute anuric renal failure required haemodialysis. Multiorgan involvement suggested SLE, which was confirmed 3 d later by increased anti-ds-DNA levels. Three methylprednisolone pulses were administered promptly, followed by oral prednisone (1.5 mg/kg/d) and six pulses of cyclophosphamide (500 mg/1.73 m2/2 wk). Mycophenolate mofetil was introduced for long-term disease control. Amylase and lipase levels decreased over 4 wk. Renal function was normal after 3 wk and proteinuria negative after 6 wk. This case suggests that steroid pulse therapy should be promptly administered if clinical and biochemical investigations suggest SLE to be responsible for AP. Aggressive treatment may be life saving.
急性胰腺炎（AP）很少会使系统性红斑狼疮（SLE）的临床病程复杂化。AP作为SLE的初始表现较为罕见，但其后果往往是致命的。皮质类固醇被怀疑在AP的发生中起作用，但其治疗益处似乎远高于胰腺病变加重的风险。我们报告一名13岁女孩，最初出现关节痛和不适，随后出现腹痛、全身水肿和血流动力学不稳定。CRP（325 ng/ml）、血清淀粉酶（14,000 IU/l）和脂肪酶（2500 IU/l）水平升高提示AP。急性无尿性肾衰竭需要进行血液透析。多器官受累提示SLE，3天后抗双链DNA水平升高证实了这一点。立即给予三次甲泼尼龙冲击治疗，随后口服泼尼松（1.5 mg/kg/d）和六次环磷酰胺冲击治疗（500 mg/1.73 m2/2周）。引入霉酚酸酯用于长期疾病控制。淀粉酶和脂肪酶水平在4周内下降。3周后肾功能恢复正常，6周后蛋白尿转阴。该病例表明，如果临床和生化检查提示SLE是AP的病因，应立即给予类固醇冲击治疗。积极治疗可能挽救生命。
