Is vertigo a symptom of Sjögren's (Sjögren's syndrome) disease?

Vertigo in Sjögren's Syndrome

Vertigo can be a neurological manifestation of Sjögren's syndrome, though it is not among the most common symptoms of the disease. While Sjögren's syndrome is primarily characterized by dryness of the eyes and mouth, it can affect multiple organ systems including the nervous system.

Neurological Involvement in Sjögren's Syndrome

Neurological manifestations occur in approximately 20% of patients with primary Sjögren's syndrome 1. These manifestations can include:

• Central nervous system (CNS) involvement (brain and spinal cord)
• Peripheral nervous system (PNS) involvement
• Autonomic nervous system dysfunction

Vertigo as a Manifestation

Vertigo in Sjögren's syndrome can occur through several mechanisms:

1. Autonomic neuropathy: Patients may experience dizziness and presyncope due to autonomic dysfunction 2
2. Central nervous system involvement: CNS manifestations can include symptoms that mimic multiple sclerosis, which may present with vertigo 1
3. Cranial nerve involvement: Affecting the vestibulocochlear nerve (CN VIII) 1

Diagnostic Considerations

When evaluating vertigo in a patient with suspected or confirmed Sjögren's syndrome:

• Determine if the vertigo fits into one of the vestibular syndrome patterns:

  • Acute vestibular syndrome (continuous dizziness lasting days to weeks)
  • Triggered episodic vestibular syndrome (brief episodes triggered by position changes)
  • Spontaneous episodic vestibular syndrome (untriggered episodes lasting minutes to hours)
  • Chronic vestibular syndrome (dizziness lasting weeks to months) 3

• Rule out other common causes of vertigo that may coexist but are not related to Sjögren's syndrome:

  • Benign paroxysmal positional vertigo (BPPV)
  • Menière's disease
  • Vestibular migraine
  • Stroke/vascular causes 4

Differential Diagnosis

The American Academy of Otolaryngology-Head and Neck Surgery provides a framework for differentiating various causes of vertigo 4:

Condition	Clinical Presentation	Differentiation
Sjögren's-related vertigo	May be episodic or chronic, often with other neurological symptoms	Associated with other Sjögren's manifestations (dry eyes, dry mouth), positive autoantibodies
BPPV	Positional vertigo lasting seconds	Not associated with hearing loss or other Sjögren's symptoms
Menière's disease	Episodes with hearing loss, tinnitus, aural fullness	Characteristic audiometric findings
Vestibular migraine	Variable duration, history of migraine	Photophobia, mild or absent hearing loss
Stroke/TIA	Sudden onset, often with other neurological deficits	Abnormal HINTS exam, risk factors for vascular disease

Management Approach

For vertigo in Sjögren's syndrome:

1. Treat the underlying Sjögren's syndrome:

   • Immunomodulatory therapy may help when vertigo is due to CNS or PNS involvement
   • Cyclophosphamide has shown efficacy in patients with CNS manifestations 1

2. Symptomatic management:

   • Vestibular rehabilitation for chronic symptoms
   • Short-term vestibular suppressants for acute symptoms
   • Management of orthostatic hypotension if present (midodrine, beta-blockers) 2

3. Monitor for progression:

   • Regular follow-up to assess response to treatment
   • Screening for other neurological manifestations

Red Flags and Pitfalls

• Neurological manifestations frequently precede the diagnosis of Sjögren's syndrome (in up to 81% of patients) 1
• Anti-Ro/SSA or anti-La/SSB antibodies may be negative initially but become positive during follow-up 1
• CNS involvement in Sjögren's syndrome is associated with more severe disability than peripheral involvement 1
• Vertigo with acute onset, associated neurological deficits, or abnormal HINTS examination requires urgent neuroimaging to rule out stroke 3

Conclusion

When evaluating a patient with vertigo and suspected or confirmed Sjögren's syndrome, consider both common causes of vertigo and potential neurological manifestations of Sjögren's. The presence of other systemic symptoms of Sjögren's syndrome, positive autoantibodies, and response to immunomodulatory therapy can help establish the relationship between vertigo and Sjögren's syndrome.