Sjogren Syndrome-Associated Autonomic Neuropathy

Abstract

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.

Keywords: acute motor and sensory axonal neuropathy (amsan); anti-ro/ssa; autonomic neuropathy; primary sjogren syndrome (pss); xerophthalmia; xerostomia.

Figure 1. Lip biopsy: minor salivary glands with approximately one focus of lymphoplasmacytic infiltration per 2 sq/mm (one focus score), compatible with Sjogren’s syndrome

Figure 2. Tilt table test

SBP: Systolic blood pressure; DBP: Diastolic blood pressure.