Department of Internal Medicine and Rheumatology, Sfanta Maria" Hospital, 011172, Bucharest, Romania.
Department of Internal Medicine and Rheumatology, Carol Davila University of Medicine and Pharmacy, 050474, Bucharest, Romania.
Clin Rheumatol. 2023 Jun;42(6):1705-1712. doi: 10.1007/s10067-023-06535-6. Epub 2023 Feb 9.
This report presents a case of a Sheehan syndrome diagnosed with a delay of 29 years after occurrence of first symptoms, following a laborious birth ended with dead fetus and massive hemorrhage. The 50-year-old patient, with early menopause from the age of 21, is referred to our rheumatology department to investigate the etiology of a myopathic syndrome, which started 2 months before and gradually worsened. The differential diagnosis took into consideration the autoimmune, infectious, paraneoplastic, endocrinological, and drug-induced myopathic syndrome. Paraclinical investigations revealed panhypopituitarism, and cerebral magnetic resonance imaging detected empty-sella. The etiology of a myopathic syndrome is often multifactorial; therefore, it is important to continue the investigations even after identifying one possible etiological factor, especially when it does not seem to fully explain the clinical-paraclinical picture. Usually, the multiple dimensions of panhypopituitarism bring the patient to various medical specialties depending on the dominant symptomatology. Given the rarity of the above-mentioned syndrome in the present, and the long gap between the initial event and the final diagnosis, its identification continues to be a challenge.
本报告介绍了一例希恩氏综合征病例,该患者在首次出现症状后 29 年才被诊断出来,此前发生了一次难产,导致死胎和大量出血。这位 50 岁的患者在 21 岁时就出现了早期绝经,因肌肉疾病而被转诊至我们的风湿病科,以调查发病原因,该疾病始于 2 个月前,并逐渐恶化。鉴别诊断考虑了自身免疫性、感染性、副肿瘤性、内分泌性和药物诱导性肌病综合征。临床检查发现全垂体功能减退症,脑部磁共振成像显示空蝶鞍。肌病的病因通常是多因素的;因此,即使确定了一个可能的病因,也很有必要继续进行调查,特别是当它似乎不能完全解释临床-临床检查结果时。通常,全垂体功能减退症的多个方面会根据主要症状使患者就诊于不同的医学专业。鉴于目前这种综合征非常罕见,且初始事件与最终诊断之间存在很长的时间间隔,因此识别它仍然是一个挑战。