Department of Rheumatology and Immunology, Children's Hospital, Capital Institute of Pediatrics. , NO.2 Yabao Road, Chaoyang District, Beijing, 100020, China.
Department of Rheumatology and Immunology, Jiangxi Provincial Children's Hospital, No. 1666, Diezihu Avenue, Honggutan District, Nanchang, 330013, Jiangxi Province, China.
Arthritis Res Ther. 2023 Dec 20;25(1):248. doi: 10.1186/s13075-023-03237-x.
Lupus mesenteric vasculitis (LMV) as initial presentation is rare, especially in childhood-onset systemic lupus erythematosus (cSLE). It is a critical complication of lupus. At present, the research on cSLE with LMV as the initial presentation is few. The aim of this study was to analyze the clinical characteristics and prognosis of cSLE with LMV in the Chinese population, compared with non-LMV cSLE.
A retrospective case-controlled study was conducted on 55 cSLE patients between July 2018 and July 2021. The clinical data, laboratory findings, imaging, treatment, and follow-up data were collected and compared between the two groups of cSLE with LMV and non-LMV. Non-LMV cSLE patients were matched according to the age and sex of LMV patients.
A total of 11 cSLE patients with LMV as the LMV group and 44 cSLE patients without LMV as the non-LMV group were included. The average age of onset was 12.55 ± 1.57 years old, the male-to-female ratio was 2:9, and high disease activity was observed in the LMV group. Abdominal pain was most common in LMV. Compared with the non-LMV, the percentage of abdominal pain, vomiting, abdominal distension, and diarrhea was higher, and gastrointestinal tract, serous cavity, kidney, and lung damage were higher in the LMV group (P < 0.05). In abdominal-enhanced CT, the percentage of intestinal wall thickening, peritoneal effusion, mesenteric vascular enhancement, hydronephrosis with ureteral dilatation, intestinal congestion, and gastric mucosa thickening in the LMV group were higher than those in the non-LMV group (P < 0.05). The percentage of receiving methylprednisolone pulse combined with cyclophosphamide pulse therapy in LMV was higher than in non-LMV. The clinical symptoms disappeared quickly, and there were no deaths in the LMV group. Compared with the non-LMV group, the 24-h urinary protein was higher, the complement C3 was lower, and the disease activity was higher in the LMV group (P < 0.05).
LMV often occurs in 12 ~ 13-year-old girls with high disease activity of cSLE. Abdominal pain is the most common and more susceptible to damage to the kidney, serous cavity, and lung in cSLE with LMV. Methylprednisolone pulse combined with CTX pulse therapy is effective. After the treatment above, cSLE with LMV has a good prognosis, but the overall recovery is worse than non-LMV patients.
狼疮肠系膜血管炎（LMV）作为首发表现较为罕见，尤其在儿童起病系统性红斑狼疮（cSLE）中。它是狼疮的一种严重并发症。目前，关于以 LMV 为首发表现的 cSLE 的研究较少。本研究旨在分析中国人群中以 LMV 为首发表现的 cSLE 的临床特征及预后，并与非 LMV cSLE 进行比较。
对 2018 年 7 月至 2021 年 7 月间的 55 例 cSLE 患者进行回顾性病例对照研究。收集并比较两组以 LMV 为首发表现的 cSLE 患者（LMV 组）和非 LMV cSLE 患者（非 LMV 组）的临床资料、实验室检查、影像学、治疗及随访资料。非 LMV cSLE 患者按照 LMV 患者的年龄和性别进行匹配。
共纳入 11 例以 LMV 为首发表现的 cSLE 患者作为 LMV 组，44 例无 LMV 为首发表现的 cSLE 患者作为非 LMV 组。两组患者的发病年龄平均为 12.55±1.57 岁，男女比例为 2:9，且 LMV 组疾病活动度较高。腹痛是 LMV 组最常见的症状。与非 LMV 组相比，LMV 组腹痛、呕吐、腹胀和腹泻的比例较高，胃肠道、浆膜腔、肾脏和肺部损伤的比例也较高（P<0.05）。在腹部增强 CT 中，LMV 组肠壁增厚、腹腔积液、肠系膜血管增强、肾盂积水伴输尿管扩张、肠淤血和胃黏膜增厚的比例高于非 LMV 组（P<0.05）。LMV 组接受甲基强的松龙冲击联合环磷酰胺冲击治疗的比例高于非 LMV 组。LMV 组患者临床症状迅速消失，无死亡病例。与非 LMV 组相比，LMV 组 24 h 尿蛋白更高，补体 C3 更低，疾病活动度更高（P<0.05）。
LMV 常发生于 12~13 岁的 cSLE 女性患儿，其疾病活动度较高。腹痛是 LMV 最常见的症状，且更易导致 cSLE 患者出现肾脏、浆膜腔和肺部损伤。甲基强的松龙冲击联合 CTX 冲击治疗有效。经上述治疗后，以 LMV 为首发表现的 cSLE 患者预后良好，但整体恢复不如非 LMV 患者。
