Kataria Riya, Suja Lakshmanan, Anil Archa Anna, Senthil Narayanasamy
Sri Ramachandra Institute of Higher Education and Research (Deemed to be University), Chennai, Tamil Nadu, India
General Medicine, Sri Ramachandra University Medical College, Chennai, Tamil Nadu, India.
BMJ Case Rep. 2023 Dec 9;16(12):e256412. doi: 10.1136/bcr-2023-256412.
Primary Sjogren's syndrome (pSS) is an autoimmune connective tissue disorder with multisystem manifestations. We here report a previously healthy woman who presented with autonomic dysfunction in the form of severe dizziness without any apparent sensory neuropathy. Detailed history and examination revealed the signs and symptoms of Sjogren's syndrome such as constipation and dry eyes and mouth, following which anti-SSA and SSB antibodies were found to be positive. Finally, a diagnosis of pSS was established after ruling out all the other causes of autonomic dysfunction in addition to the clinical and laboratory evidence. The patient was treated with the maximum doses of midodrine and fludrocortisone, yet no progress was noticed. Hence, a trial of steroids was started and she showed a significant clinical improvement. Our patient presented with pure autonomic failure associated with Sjogren's syndrome, making it an extremely rare entity.
原发性干燥综合征（pSS）是一种具有多系统表现的自身免疫性结缔组织疾病。我们在此报告一名既往健康的女性，她以严重头晕的形式出现自主神经功能障碍，且无任何明显的感觉神经病变。详细的病史和检查揭示了干燥综合征的体征和症状，如便秘、眼干和口干，随后发现抗SSA和SSB抗体呈阳性。最终，在排除了自主神经功能障碍的所有其他病因并结合临床和实验室证据后，确诊为pSS。该患者接受了最大剂量的米多君和氟氢可的松治疗，但未见进展。因此，开始试用类固醇，她的临床症状有了显著改善。我们的患者表现为与干燥综合征相关的单纯性自主神经功能衰竭，这是一种极为罕见的情况。
