Chaaban Nourhan, Shaver Timothy, Kshatriya Shilpa
Internal Medicine, University of Kansas School of Medicine-Wichita, Wichita, USA.
Rheumatology, University of Kansas School of Medicine-Wichita, Wichita, USA.
Cureus. 2022 Jun 1;14(6):e25563. doi: 10.7759/cureus.25563. eCollection 2022 Jun.
Sjogren syndrome (SS) is one of the common autoimmune diseases characterized by dryness of the eyes and mouth described as "xerophthalmia" and "xerostomia," respectively. Affected patients may also experience complex neurological symptoms characterized by extremity paresthesia and pain as well as dizziness and presyncope. In fact, patients may present initially with pure neurological symptoms. Thus, the diagnosis of SS remains a clinical challenge. We report a case here of a patient with primary Sjogren syndrome (pSS) experiencing severe autonomic neuropathy symptoms. A 53-year-old female patient diagnosed with pSS at the age of 30 years presented with a four-month history of palpitations and dizziness. A tilt table test showed positive findings with significant symptomatic orthostatic hypotension. The patient was started on midodrine therapy followed by a beta-blocker with improvement in her symptoms. There was also augmentation of her SS disease management by introducing IV IgG therapy. The diagnosis of neuropathy in SS is complex and requires a combination of clinical signs and symptoms. This case report focuses on the neurological manifestations of SS, especially the ones related to autonomic neuropathy. We aim to share awareness of the neurological manifestations of patients with pSS and alert physicians that it could be the initial presentation of this systemic disease.
干燥综合征（SS）是常见的自身免疫性疾病之一，其特征分别为眼睛干涩（称为“干眼症”）和口腔干燥（称为“口干症”）。受影响的患者还可能出现以肢体感觉异常、疼痛以及头晕和晕厥前症状为特征的复杂神经症状。事实上，患者最初可能仅表现为单纯的神经症状。因此，干燥综合征的诊断仍然是一项临床挑战。我们在此报告一例原发性干燥综合征（pSS）患者出现严重自主神经病变症状的病例。一名53岁女性患者，30岁时被诊断为pSS，出现心悸和头晕4个月病史。倾斜试验显示阳性结果，伴有明显的症状性直立性低血压。患者开始接受米多君治疗，随后使用β受体阻滞剂，症状有所改善。通过引入静脉注射免疫球蛋白疗法，她的干燥综合征疾病管理也得到了加强。干燥综合征中神经病变的诊断很复杂，需要结合临床体征和症状。本病例报告重点关注干燥综合征的神经表现，尤其是与自主神经病变相关的表现。我们旨在提高对原发性干燥综合征患者神经表现的认识，并提醒医生这可能是这种全身性疾病的首发表现。
