Sjogren Syndrome-Associated Autonomic Neuropathy

Area of Science:

• Rheumatology
• Neurology
• Autoimmunology

Background:

• Sjogren syndrome (SS) is a systemic autoimmune disorder primarily causing xerophthalmia and xerostomia.
• Neurological manifestations, including autonomic neuropathy, can occur in SS, sometimes preceding classic symptoms.
• Diagnosis of SS can be challenging due to varied clinical presentations.

Observation:

• A 53-year-old female with primary SS (pSS) presented with palpitations and dizziness.
• The patient exhibited significant symptomatic orthostatic hypotension on tilt table testing.
• Neuropathy symptoms, specifically autonomic neuropathy, were severe in this case.

Findings:

• Tilt table testing confirmed orthostatic hypotension.
• Treatment with midodrine and a beta-blocker improved the patient's symptoms.
• Intravenous immunoglobulin (IV IgG) therapy was initiated as an augmentation of SS management.

Implications:

• This case highlights the importance of considering neurological symptoms, particularly autonomic neuropathy, in the differential diagnosis of pSS.
• Physicians should be aware that neurological manifestations can be the initial presentation of pSS.
• Comprehensive evaluation combining clinical signs and diagnostic tests is essential for diagnosing neuropathy in SS.