Document Detail

Vasculitis in Sjögren's Syndrome.
MedLine Citation:
PMID:  21870104     Owner:  NLM     Status:  MEDLINE    
Sjögren's syndrome is a chronic autoimmune disease that is commonly manifested by immune attack on the exocrine glands with resultant dry eyes and dry mouth. Sjögren's syndrome patients also have disease in other organs. One of the most common extraglandular manifestations is vasculitis. Skin vasculitis, with palpable purpura clinically and leukocytoclastic vasculitis on pathological examination, is common. Although half of those individuals with subcutaneous vasculitis have only a single episode, skin vasculitic involvement is associated with more severe disease. Necrotizing vasculitis of medium-sized vessels resembling polyarteritis nodosa can occur in Sjögren's syndrome patients. Experience in therapy for vasculitis is limited, but intravenous IgG may be effective. Recent data support a relationship between neuromyelitis optica (Devic disease) and Sjögren's syndrome. Sjögren's syndrome patients with optic neuritis or transverse myelitis have anti-aquaporin-4, which are characteristic of Devic disease. Devic disease patients have salivary lymphocytic infiltration similar to that found among Sjögren's syndrome patients.
R Hal Scofield
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Publication Detail:
Type:  Journal Article; Review    
Journal Detail:
Title:  Current rheumatology reports     Volume:  13     ISSN:  1534-6307     ISO Abbreviation:  Curr Rheumatol Rep     Publication Date:  2011 Dec 
Date Detail:
Created Date:  2011-11-04     Completed Date:  2012-02-28     Revised Date:  2013-04-12    
Medline Journal Info:
Nlm Unique ID:  100888970     Medline TA:  Curr Rheumatol Rep     Country:  United States    
Other Details:
Languages:  eng     Pagination:  482-8     Citation Subset:  IM    
Department of Medicine, University of Oklahoma Health Sciences Center, Oklahoma City, USA.
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MeSH Terms
Aquaporin 4 / immunology
Blood Vessels / pathology
Immunosuppressive Agents / therapeutic use
Neuromyelitis Optica / complications,  immunology,  pathology
Sjogren's Syndrome / complications*,  drug therapy,  pathology
Skin Diseases, Vascular / complications*,  drug therapy,  pathology
Vasculitis / complications*,  drug therapy,  pathology
Grant Support
Reg. No./Substance:
0/AQP4 protein, human; 0/Aquaporin 4; 0/Immunosuppressive Agents

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