Abstract
Behcet's disease (BD) is an inflammatory disorder of unknown aetiology, unanimously recognized as both autoimmune and autoinflammatory disease. Indeed many of its classical manifestations overlap with those of monogenic autoinflammatory disorders. Clinically disease is characterized by multiple organ involvement, in particular by the “triple symptom complex”, consisting of recurrent oral aphthosis, genital ulcers and recurrent bilateral uveitis. The abnormal activation of either innate and adaptive immunity, triggered by some microbial agents in genetically predisposed individuals, with consequent interaction of both T lymphocytes and activated neutrophils would seem to be involved in the disease onset. Therefore multiple cytokines may contribute to the pathological scenario of BD playing a pivotal role in the occurrence of the clinical manifestations.
Highlights
Behçet’s disease (BD) is an inflammatory disorder of unknown aetiology, unanimously recognized as both autoimmune and autoinflammatory disease
Disease is characterized by multiple organ involvement, in particular by the “triple symptom complex”, consisting of recurrent oral aphthosis, genital ulcers and recurrent bilateral uveitis
The abnormal activation of either innate and adaptive immunity, triggered by some microbial agents in genetically predisposed individuals, with consequent interaction of both T lymphocytes and activated neutrophils would seem to be involved in the disease onset
Summary
Behçet’s disease (BD) is an inflammatory disorder of unknown aetiology, unanimously recognized as both autoimmune and autoinflammatory disease. Many of its classical manifestations overlap with those of monogenic autoinflammatory disorders. Disease is characterized by multiple organ involvement, in particular by the “triple symptom complex”, consisting of recurrent oral aphthosis, genital ulcers and recurrent bilateral uveitis. The abnormal activation of either innate and adaptive immunity, triggered by some microbial agents in genetically predisposed individuals, with consequent interaction of both T lymphocytes and activated neutrophils would seem to be involved in the disease onset. Multiple cytokines may contribute to the pathological scenario of BD playing a pivotal role in the occurrence of the clinical manifestations
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