[Vasculopathy in Sjögren's syndrome]. / Vaskulopathien bei Sjögren-Syndrom.
Z Rheumatol
; 68(4): 305-11, 2009 Jun.
Article
em De
| MEDLINE
| ID: mdl-19357858
ABSTRACT
Sjögren's syndrome is a systemic autoimmune disease with a predominant involvement of exocrine glands leading to sicca symptoms. Extraglandular involvement occurs in about 40% of patients with skin, musculoskeletal, neurological and organ manifestations. Systemic vasculitic manifestations of Sjögren's syndrome can be assumed in approximately 5%-10% of patients. Leukocytoclastic or cryoglobulinemic vasculitis represent classic vasculitic manifestations of Sjögren's syndrome. In the pathogenesis of vasculitis, B-cell-driven autoimmune processes play a major role by producing autoantibodies against the Ro/SS-A and La/SS-B antigens and cryoglobulins. In patients with Sjögren's syndrome, manifestation of vasculitis, non-Hodgkin's lymphoma and glomerulonephritis, as well as positive cryoglobulins and decreased levels of complement factors, are considered negative prognostic markers. Various immunosuppressive strategies, usually in co-medication with glucocorticoids, are used for the treatment of vasculitis in Sjögren's syndrome. For refractory and severe manifestations, a B-cell-targeted therapy with Rituximab should be also considered.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Vasculite
/
Síndrome de Sjogren
/
Imunossupressores
Tipo de estudo:
Prognostic_studies
Limite:
Humans
Idioma:
De
Revista:
Z Rheumatol
Ano de publicação:
2009
Tipo de documento:
Article
País de afiliação:
Alemanha