Lin Qiang, Zhang Man, Tang Hanyun, Shen Yunyan, Zhu Yun, Xu Qinying, Li Xiaozhong
Department of Nephrology and Immunology, Children's Hospital of Soochow University, 303, Jingde Road, Suzhou, Jiangsu, The People's Republic of China.
Rheumatol Int. 2020 May;40(5):811-819. doi: 10.1007/s00296-019-04388-4. Epub 2019 Aug 3.
Pancreatitis is uncommon in systemic lupus erythematosus (SLE) and is rarely reported in children, possibly being related to macrophage activation syndrome (MAS). The incidence of MAS in children with lupus pancreatitis is unknown, as is their prognosis. In this case-based review, we report a pediatric patient with SLE complicated with pancreatitis and MAS, and performed a literature review. We report an 11-year-old girl with SLE and MAS who developed pancreatitis on the second day of methylprednisolone pulse therapy (500 mg/day). We continued methylprednisolone pulse therapy, and performed three rounds of DNA-immunoadsorption and three rounds of hemoperfusion. A second course of methylprednisolone pulse therapy was initiated 9 days later. The patient received a monthly cyclophosphamide pulse therapy (10 mg/kg/day, 2 consecutive days every month) for 6 months, after which she was treated with mycophenolate mofetil 20 mg/kg/day. The condition of the patient gradually improved, her blood amylase and lipase decreased. She was in a stable condition during 13-month follow-up period. Review of the literature of pediatric patients with SLE and pancreatitis showed that there are 127 cases that have been reported in the past 30 years, 40 cases were excluded in our study because of inadequate information. Of the 87 patients included in our literature review, the mortality rate was 33.33%, and 52.86% of the patients with pancreatitis had MAS at the same time. Pancreatitis is uncommon in SLE, but must be suspected if a patient with SLE develops digestive symptoms. Patients with SLE with pancreatitis have a high incidence of MAS and high mortality rate; however, early recognition and effective treatment can relieve the disease symptoms.
胰腺炎在系统性红斑狼疮（SLE）中并不常见，在儿童中鲜有报道，可能与巨噬细胞活化综合征（MAS）有关。狼疮性胰腺炎患儿中MAS的发生率以及其预后尚不清楚。在本病例回顾中，我们报告了一名患有SLE并合并胰腺炎和MAS的儿科患者，并进行了文献综述。我们报告了一名11岁患有SLE和MAS的女孩，在甲泼尼龙冲击治疗（500mg/天）的第二天发生了胰腺炎。我们继续进行甲泼尼龙冲击治疗，并进行了三轮DNA免疫吸附和三轮血液灌流。9天后开始第二轮甲泼尼龙冲击治疗。患者接受了为期6个月的每月一次环磷酰胺冲击治疗（10mg/kg/天，每月连续2天），之后用霉酚酸酯20mg/kg/天进行治疗。患者病情逐渐改善，血淀粉酶和脂肪酶下降。在13个月的随访期内病情稳定。对SLE和胰腺炎儿科患者的文献回顾显示，在过去30年中已报道127例，在我们的研究中，40例因信息不足被排除。在我们纳入文献综述的87例患者中，死亡率为33.33%，52.86%的胰腺炎患者同时患有MAS。胰腺炎在SLE中并不常见，但如果SLE患者出现消化系统症状则必须怀疑。患有胰腺炎的SLE患者MAS发生率高且死亡率高；然而，早期识别和有效治疗可缓解疾病症状。
