Document Detail

Myelopathy in Sjögren's syndrome: role of nonsteroidal immunosuppressants.
MedLine Citation:
PMID:  14717615     Owner:  NLM     Status:  MEDLINE    
The incidence, aetiology and optimal treatment of CNS Sjögren's syndrome, including myelopathy associated with Sjögren's syndrome, are unknown at the present time. CNS Sjögren's syndrome is thought to be the result of an autoimmune vasculitis, but other mechanisms may be important. Spinal cord involvement in CNS Sjögren's syndrome may present as acute transverse myelitis, progressive myelitis, Brown-Séquard syndrome, neurogenic bladder or lower motor neurone disease. Optic nerve pathology frequently accompanies spinal cord involvement. Acute transverse myelitis has a high mortality and appears to be the most frequent form of spinal cord involvement in CNS Sjögren's syndrome, occurring in about 1% of all patients with Sjögren's syndrome. The patient's symptomatology and clinical course dictate current treatment of myelopathy. First-line treatment appears to be corticosteroid therapy. However, when the patient's condition fails to improve or deteriorates a nonsteroidal immunosuppressant agent should be considered. Agents used to treat myelopathy include cyclophosphamide, chlorambucil, azathioprine, ciclosporin (cyclosporin) and methotrexate in conjunction with corticosteroids. Most data exist as anecdotal reports. The agent of first choice, based on adverse effect profile and efficacy, appears to be cyclophosphamide given intravenously in pulse doses. Other nonsteroidal immunosuppressant agents should be considered, especially when lack of efficacy of, or intolerance to, cyclophosphamide exists in the patient's history. Glandular and other extraglandular symptoms may benefit concomitantly from the immunosuppressant treatment. In addition, when acute relief of symptomatology is needed, the patient may benefit from a trial of plasmapheresis or intravenous immunoglobulin. Infliximab (anti-tumour necrosis factor-alpha antibodies) has not been used as a treatment modality for myelopathy, but has shown some usefulness in the treatment of extraglandular symptoms, as well as peripheral nervous system manifestations of Sjögren's syndrome. This agent might be considered when all other treatment modalities have failed given the presumed importance of tumour necrosis factor in the pathogenesis of Sjögren's syndrome.
Susan J Rogers; Christopher S Williams; Gustavo C Román
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Publication Detail:
Type:  Journal Article; Review    
Journal Detail:
Title:  Drugs     Volume:  64     ISSN:  0012-6667     ISO Abbreviation:  Drugs     Publication Date:  2004  
Date Detail:
Created Date:  2004-01-13     Completed Date:  2004-05-04     Revised Date:  2009-11-03    
Medline Journal Info:
Nlm Unique ID:  7600076     Medline TA:  Drugs     Country:  New Zealand    
Other Details:
Languages:  eng     Pagination:  123-32     Citation Subset:  IM    
University of Texas Health Sciences Center at San Antonio, San Antonio, Texas 78229-3900, USA.
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MeSH Terms
Clinical Trials as Topic
Immunosuppressive Agents / therapeutic use*
Myelitis, Transverse / diagnosis,  drug therapy,  etiology
Sjogren's Syndrome / complications,  drug therapy*
Spinal Cord / pathology
Spinal Cord Diseases / diagnosis,  drug therapy*,  etiology
Reg. No./Substance:
0/Immunosuppressive Agents
Comment In:
Drugs. 2006;66(5):729   [PMID:  16620149 ]

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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