REVIEW - Treatment of CNS involvement associated with primary Sjogren's syndrome

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Curr Pharm Des. 2008;14(13):1270-3.

Treatment of central nervous system involvement associated with

primary Sjögren's syndrome.

Ozgocmen S, Gur A.

Division of Rheumatology, Department of Physical Medicine and

Rehabilitation, Firat University, Faculty of Medicine, Elazig, Turkey.

Sjögren's syndrome (SS) is a chronic autoimmune disease that mainly

affects the exocrine glands and usually presents with sicca symptoms

of the main mucosal surfaces. The prevalence and the type of central

nervous system (CNS) tissue damage caused by SS are debatable. The

wide spectrum of CNS manifestations, different classification criteria

used and unclear inclusion or exclusion criteria pose some difficulty

reviewing these studies. Careful examination of the SS patients and to

be aware of neurological findings which may be associated with

suspicious CNS involvement is highly important. Central nervous system

may also hypothetically have a role in the pathophysiology of SS. The

wide spectrum of CNS involvement includes focal (sensorial and motor

deficits, brain stem, cerebellar lesions, seizure, migraine etc.) or

non-focal (encephalomyelitis, aseptic meningitis, neuropsychiatric

dysfunctions), spinal cord (myelopathy, transverse myelitis, motor

neuron disease etc.) findings or multiple sclerosis-like illness and

optic neuritis. Evolving imaging techniques such as single photon

emission computed tomography (SPECT), magnetic resonance spectroscopy

or magnetization transfer imaging are promising for better

understanding the nature of CNS involvement in SS. Treatments usually

comprise symptomatic approach in milder cases however, pulse

cyclophosphamide and steroids or other immunosuppressants

(chlorambucil or azathioprine) are required in cases with progressive

symptoms leading to neurological impairment. Anti-TNF agents

(infliximab and etanercept) and B cell targeted therapies (rituximab

and epratuzumab) are used in primary SS however their efficacy on CNS

manifestation is still unclear. Randomized, multicenter studies are

warranted to confirm the efficacy of treatment regimes which were

reported to be effective in anecdotal reports or in small uncontrolled

series. This article reviews the clinical approach to current therapy

of CNS involvement in patients with primary SS.

PMID: 18537651

http://www.ncbi.nlm.nih.gov/pubmed/18537651

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