Sjögren’s Syndrome (SS) is a systemic autoimmune disease where the immune system attacks moisture-producing glands, causing severe dry eyes and dry mouth (xerostomia and keratoconjunctivitis sicca). The disease extends beyond glandular issues, affecting multiple organ systems, including the nervous system. Dizziness, which can manifest as lightheadedness, unsteadiness, or true vertigo, is a documented neurological manifestation of SS.
Autonomic Nervous System Disruption
The most frequent cause of dizziness in Sjögren’s Syndrome (SS) stems from a malfunction of the Autonomic Nervous System (ANS), known as dysautonomia. The ANS is the body’s involuntary control center, regulating functions like heart rate, breathing, digestion, and blood pressure. When the autoimmune process attacks the nerves controlling these functions, the body struggles to maintain stability, particularly in response to gravity.
This dysregulation commonly presents as Orthostatic Intolerance (OI), the inability to remain upright without symptoms. When standing, gravity pulls blood into the lower extremities and abdomen. A healthy ANS quickly constricts blood vessels to counteract this pooling.
In SS-related dysautonomia, this constriction response is impaired, causing blood pressure to drop or remain unstable, a phenomenon called orthostatic hypotension. The resulting reduction in blood flow to the brain causes lightheadedness or presyncope, the feeling of being about to faint. Approximately 50% of people with SS experience some form of dysautonomia. The dizziness experienced in these cases is typically lightheadedness or unsteadiness rather than a true spinning sensation.
Systemic Inflammation and Vestibular Involvement
While ANS malfunction is the primary culprit for lightheadedness, systemic inflammation can cause dizziness, including true vertigo. This inflammation may manifest as vasculitis, disrupting the blood supply to the vestibulocochlear nerve (Cranial Nerve VIII) or the inner ear organs responsible for balance.
Studies confirm that individuals with SS exhibit measurable impairments in vestibular function, suggesting a direct neurosensory component. The disease process compromises the inner ear’s ability to sense motion and position. This form of dizziness is often described as vertigo, a distinct spinning sensation that contrasts with orthostatic lightheadedness.
A simpler cause of lightheadedness is severe chronic dehydration, a direct consequence of the disease’s hallmark dryness. Sjögren’s reduces the body’s fluid retention capacity due to impaired exocrine function, leading to chronic low blood volume. Low blood volume directly exacerbates orthostatic intolerance, as less fluid is available to be pumped back to the brain upon standing.
Identifying the Specific Cause
Identifying the source of dizziness is crucial because treatment strategies for ANS dysfunction and vestibular inflammation differ significantly. A detailed medical history is the starting point, focusing on the quality of the dizziness—whether it is lightheadedness (feeling faint or woozy) or vertigo (a spinning sensation). Lightheadedness occurring specifically upon standing or prolonged standing strongly suggests an ANS issue.
Specific diagnostic tools confirm the underlying cause. Tilt Table Testing is the gold standard for diagnosing Postural Orthostatic Tachycardia Syndrome or Orthostatic Hypotension by monitoring heart rate and blood pressure changes as the patient is tilted upright. If true vertigo is reported, a neurologist or otolaryngologist may perform Vestibular Function Tests, such as video head impulse testing (vHIT) or caloric testing, to assess inner ear function and the integrity of central balance pathways.
A comprehensive medication review is also necessary, as common Sjögren’s treatments can mimic or worsen dizziness. For example, Hydroxychloroquine (Plaquenil) can cause dizziness and vertigo. Cholinergic agonists like cevimeline (Evoxac), used to stimulate saliva production, can cause dizziness directly or indirectly through excessive sweating and dehydration. Ruling out these medication-induced symptoms is an important diagnostic step.
Management Strategies for Relief
Once the specific cause is identified, management focuses on targeted interventions to stabilize the system. For dysautonomia and orthostatic lightheadedness, the initial strategy involves rigorous lifestyle modifications designed to increase blood volume and improve vascular tone.
- Aggressive fluid intake, often recommending 2 to 3 liters of water or electrolyte solutions daily.
- Increased sodium consumption to help retain that fluid.
- Physical counter-maneuvers, such as crossing the legs or squatting, to temporarily raise blood pressure and abort presyncopal episodes.
- Compression garments (20-30 mmHg) extending up to the waist, to mechanically prevent blood from pooling in the lower body.
For persistent symptoms, medications like fludrocortisone (which helps the body retain salt and water) or midodrine (a vasoconstrictor) may be prescribed to stabilize blood pressure.
If the dizziness is confirmed as vertigo stemming from inner ear or central nervous system involvement, a specialized approach is necessary. Vestibular rehabilitation therapy (VRT) is used to train the brain to compensate for inner ear deficits and improve balance. In cases where inflammation is the suspected cause, immunomodulatory drugs, such as intravenous immunoglobulins (IVIG), may be considered to address the underlying autoimmune activity. Patients should also make simple lifestyle adjustments, such as rising slowly and avoiding environmental triggers like excessive heat or large, high-carbohydrate meals.