Behçet's disease: from innate to adaptive immunity

Excerpt

Both innate and adaptive immune systems are activated in Behçet's disease (BD) with a proinflammatory and Th1 type cytokine profile. BD might be linked to a specific, primary immune abnormality with a genetic mutation effecting an adhesion molecule, a proinflammatory cytokine/chemokine or a transcription or regulatory factor, which predisposes to early or more intense neutrophil and T cell responses. Increased neutrophil responses to urate crystals and fMLP or superantigen-drived interferon-γ response of T cells suggest a model with these characteristics, which also explains the 'pathergy' or 'skin urate' tests. MEFV gene mutations, suggested to be specific for familial mediterranean fever, which decrease the expression of an anti-inflammatory protein 'pyrine' from neutrophils, are also described in BD from Turkey. …