病例报告

以假性肠梗阻为首发表现的干燥综合征1例

  • 刘美兰 ,
  • 王领 ,
  • 杨蓉 ,
  • 王嘉乐 ,
  • 涂慧敏 ,
  • 李慧凛
展开
  • a.上海市浦东新区公利医院 风湿免疫科, 上海 200135
    b.上海市浦东新区公利医院 肾脏内科, 上海 200135
李慧凛 E-mail: medlhl@163.com

收稿日期: 2024-11-20

  网络出版日期: 2025-10-27

基金资助

上海市浦东新区卫生健康委员会领先人才培养计划(PWRl2024-01);上海市浦东新区卫生健康委员会2025年度科教兴医培育学科建设类项目(PWZbr2025-03)

A case of Sjögren syndrome with intestinal pseudo-obstruction as the initial symptom

  • LIU Meilan ,
  • WANG Ling ,
  • YANG Rong ,
  • WANG Jiale ,
  • TU Huimin ,
  • LI Huilin
Expand
  • a. Department of Rheumatology & Immunology, Shanghai Pudong New Area Gongli Hospital, Shanghai, 200135, China
    b.Department of Nephrology, Shanghai Pudong New Area Gongli Hospital, Shanghai, 200135, China

Received date: 2024-11-20

  Online published: 2025-10-27

摘要

干燥综合征(Sjögren syndrome, SS)是一种以外分泌腺受累为主的系统性自身免疫性疾病,临床表现多样,易被误诊。本文报道1例以假性肠梗阻(intestinal pseudo-obstruction, IPO)为首发症状的SS病例。患者为31岁女性,因恶心、呕吐伴排便停止就诊,影像学提示结肠积粪但无机械性梗阻证据,血清学检测显示抗SSA/SSB/Ro-52抗体阳性,唇腺活检符合SS特征。经糖皮质激素及免疫调节治疗后症状缓解。本病例提示,SS可表现为非典型胃肠及神经系统症状,临床中需结合自身抗体检测及多学科评估以提高诊断准确性。

本文引用格式

刘美兰 , 王领 , 杨蓉 , 王嘉乐 , 涂慧敏 , 李慧凛 . 以假性肠梗阻为首发表现的干燥综合征1例[J]. 内科理论与实践, 2025 , 20(04) : 319 -321 . DOI: 10.16138/j.1673-6087.2025.04.11

Abstract

Sjögren syndrome (SS) is a systemic autoimmune disease primarily affecting exocrine glands. It has diverse clinical manifestations and is easily misdiagnosed. This article reports a case of SS presenting with intestinal pseudo-obstruction (IPO) as the initial symptom. A 31-year-old female presented with nausea, vomiting and bowel movement cessation. Imaging revealed colonic fecal impaction without mechanical obstruction. Serological tests showed positive anti-SSA/SSB/Ro-52 antibodies, and labial gland biopsy confirmed SS. The patient’s symptoms were relieved after receiving glucocorticoid and immunomodulatory therapy. This case highlights that SS may present with atypical gastrointestinal and neurological symptoms, suggesting the importance of multidisciplinary evaluation and autoantibody screening for early diagnosis.

参考文献

[1] Brito-Zerón P, Retamozo S, Ramos-Casals M. Sj?gren syndrome[J]. Med Clin (Barc), 2023, 160(4):163-171.
[2] 李慧敏, 余陈欢, 黄燕静, 等. 400例干燥综合征患者临床并发症的特点分析[J]. 国际流行病学传染病学杂志, 2019, 46(3):247-250.
  Li HM, Yu CH, Huang YJ, et al. Characteristics of clinical complications in 400 patients with Sjogren’s syndrome[J]. Int J Epidemiol Infect Dis, 2019, 46(3):247-250.
[3] 陈金梅, 曾安祥. 以假性肠梗阻为首发症状的干燥综合征1例[J]. 中国校医, 2023, 37(7):553-554.
  Chen JM, Zeng AX. A case of Sj?gren’s syndrome with pseudointestinal obstruction as the first symptom[J]. Chin J School Doctor, 2023, 37(7):553-554.
[4] 程昉, 赵福涛, 沈雪敏, 等. 原发性干燥综合征多学科诊疗专家共识(2024版)[J]. 内科理论与实践, 2024, 19(6): 357-362.
  Cheng F, Zhao FT, Shen XM, et al. Expert consensus on multidisciplinary diagnosis and treatment of primary Sj?gren syndrome (2024 ed)[J]. J Intern Med Concepts Pract, 2024, 19(6): 357-362.
[5] Shiboski CH, Shiboski SC, Seror R, et al. 2016 American College of Rheumatology/European League Against Rheumatism Classification Criteria for primary Sjögren’s syndrome[J]. Arthritis Rheumatol, 2017, 69(1):35-45.
[6] 王也, 聂英坤. 结缔组织病相关性假性肠梗阻的研究进展[J]. 疑难病杂志, 2019, 18(3):311-314.
  Wang Y, Nie YK. Research progress of connective tissue disease-related pseudointestinal obstruction[J]. Chin J Diffic and Compl Cas, 2019, 18(3):311-314.
[7] 方均燕, 宋阿会, 刘英莉, 等. 以假性肠梗阻为首发表现的原发性干燥综合征合并桥本甲状腺炎并文献复习[J]. 重庆医学, 2019, 48(5):898-900.
  Fang JY, Song AH, Liu YL, et al. Primary Sj?gren’s syndrome with pseudo-obstruction as the initial manifestation complicated with Hashimoto’s thyroiditis[J]. Chong-qing Med, 2019, 48(5):898-900.
[8] Liu C, Lio KU, Le Y, et al. Acute intestinal pseudo-obstruction secondary to Sjogren’s syndrome in pregnancy: a case report and literature review[J]. BMC Pregnancy Childbirth, 2023, 23(1):473.
[9] 陈爱凤, 沈晓强, 谢文君, 等. 以双侧胸腔积液为首发表现的原发性干燥综合征一例报道并文献复习[J]. 中国全科医学, 2019, 22(17):2136-2140.
  Chen AF, Shen XQ, Xie WJ, et al. Primary Sjogren’s syndrome with bilateral pleural effusion as the initial manifestation:a case report and literature review[J]. Chinese General Practice, 2019, 22(17):2136-2140.
[10] Perzyńska-Mazan J, Ma?lińska M, Gasik R. Neurological manifestations of primary sj?gren’s syndrome[M]. Poland: Reumatologia Termedia Publishing House Ltd, 2018:99-105.
[11] Berkowitz AL, Samuels MA. The neurology of Sjogren’s syndrome and the rheumatology of peripheral neuropathy and myelitis[J]. Pract Neurol, 2014, 14(1):14-22.
[12] Barsottini OGP, Moraes MPM, Fraiman PHA, et al. Sjogren’s syndrome: a neurological perspective[J]. Arq Neuropsiquiatr, 2023, 81(12):1077-1083.
[13] Popescu A, Hickernell J, Paulson A, et al. Neurological and psychiatric clinical manifestations of sj?gren syndrome[J]. Curr Neurol Neurosci Rep, 2024, 24(8):293-301.
[14] Kranthi D, Banerjee D, Dahale A, et al. Systemic lupus erythematosus unmasked: Intestinal pseudo- obstruction as the first presentation[J]. BMJ Case Rep, 2025, 18(2):e263491.
[15] Nyabera A, Elfishawi M, Cuevas F, et al. Intestinal pseudo-obstruction as the initial clinical presentation in systemic lupus erythematosus: a rare and severe disorder[J]. Case Rep Gastrointest Med, 2020, 2020:8873917.
[16] Ramos-Casals M, Brito-Zerón P, Bombardieri S, et al. EULAR recommendations for the management of Sj?gren’s syndrome with topical and systemic therapies[J]. Ann Rheum Dis, 2020, 79(1):3-18.
文章导航

/