Lalrehman Urooj, Hamza Hafiz Muhammad, Malik Muiz M, Ali Mehdi, Nayab Uzair, Ahmed Somaiya, Awan Ayaz Ahmed
Medicine/Endocrinology, Jinnah postgraduate Medical center/ Jinnah Sindh Medical University, Karachi, Pakistan.
Foundation University School of Health Sciences, Islamabad, Pakistan.
Ann Med Surg (Lond). 2025 Mar 28;87(5):2774-2783. doi: 10.1097/MS9.0000000000003207. eCollection 2025 May.
Autoimmune polyglandular syndrome (APS) is a rare autoimmune disease that affects multiple endocrine glands. It can lead to various clinical manifestations. The primary objective of this review was to provide a comprehensive analysis of the clinical features and management strategies used in managing APS, based on a review of case reports. We emphasize disease pattern recognition and detection of atypical clinical signs, as well as the outcomes of various treatment modalities. Case reports from credible journals were searched in PubMed, resulting in a systematic literature review adopting the 2020 Preferred Reporting Items for Systematic Reviews and Meta-analysis guidelines. Of the 1173 records identified, 474 remained after duplicates were removed, and 49 full-text articles were reviewed. After further screening based on criteria such as associated disorders, patient age, follow-up, and treatment details, 12 articles were selected for the final analysis. The findings revealed that common endocrine dysfunctions in APS include Addison's disease and hypothyroidism, while non-endocrine manifestations, such as autoimmune encephalitis and pernicious anemia, can also occur. The most frequently adopted strategies were immunosuppressive and hormone therapies. The heterogeneity of APS indicates early diagnosis and individualized treatment strategies. Ultimately, this analysis identifies existing insufficiencies and provides recommendations for formulating standard operating procedures for the treatment of APS.
自身免疫性多腺体综合征(APS)是一种罕见的自身免疫性疾病,会影响多个内分泌腺体。它可导致多种临床表现。本综述的主要目的是在对病例报告进行回顾的基础上,对APS的临床特征及管理策略进行全面分析。我们强调疾病模式识别、非典型临床体征的检测以及各种治疗方式的效果。在PubMed中检索了可信期刊的病例报告,从而按照2020年系统评价与Meta分析优先报告条目指南进行了系统的文献综述。在识别出的1173条记录中,去除重复记录后还剩474条,对49篇全文进行了审查。根据相关疾病、患者年龄、随访及治疗细节等标准进一步筛选后,选择了12篇文章进行最终分析。研究结果显示,APS常见的内分泌功能障碍包括艾迪生病和甲状腺功能减退,同时也可能出现自身免疫性脑炎和恶性贫血等非内分泌表现。最常采用的策略是免疫抑制和激素治疗。APS的异质性表明需要早期诊断和个体化治疗策略。最终,本分析确定了现有不足之处,并为制定APS治疗的标准操作程序提供了建议。