Approaches to Immunosuppression in Behcet's Disease
Approaches to Immunosuppression in Behcet's Disease
Behçet's disease (BD) is a systemic large-vessel vasculitis characterized by a wide clinical spectrum including recurrent oral and genital ulcerations, uveitis, and vascular, neurological, articular, renal and gastrointestinal manifestations. Therapeutic management of BD depends on the clinical presentation and organ involved. Although colchicine, nonsteroidal anti-inflammatory agents and topical treatments with corticosteroids are often sufficient for mucocutaneous and joint involvement, a more aggressive approach with immunosuppressive agents is warranted for severe manifestations such as posterior uveitis, retinal vasculitis and vascular, neurological and gastrointestinal involvement. However, some patients still have refractory disease, relapse, sight-threatening eye disease or irreversible organ damage. Recent improvements in the understanding of the pathogenic mechanisms have led to the identification of potential targets and future therapies for BD. In contrast to current nonspecific immunosuppressive agents, the emergence of immunomodulatory drugs provides the possibility of interfering with specific pathogenic pathways. Novel targeted immunosuppressive therapies might be used in the future for BD.
Behçet's disease (BD) is a systemic vasculitis affecting arterial or venous vessels of any size, and characterized by a wide clinical spectrum including recurrent oral and genital ulcerations, uveitis, and vascular, neurological, articular, renal and gastrointestinal manifestations. Diagnosis is only based on clinical criteria as for BD there is no relevant biological test for diagnosis. BD is especially frequent along the ancient Silk Road, which extends from eastern Asia to the Mediterranean basin. Carrying HLA-B51 increases the risk of developing BD by 1.5–16 times. Except for the severity of ocular disease, HLA-B51 does not seem to be correlated with the prognosis of the disease. The exact cause of the disease remains unknown, but it is believed that both genetic and environmental factors contribute to its development.
BD significantly increases morbidity and mortality. The leading cause of morbidity in BD is eye involvement with the potential threat of visual loss. Male sex, arterial involvement and the number of flares are associated with mortality in BD.
Therapeutic management of BD depends on the clinical presentation and organ involved. Although colchicine, nonsteroidal anti-inflammatory agents and topical treatments are often sufficient for mucocutaneous and joint involvement, a more aggressive approach with immunosuppressive agents is warranted for severe manifestations such as posterior uveitis, retinal vasculitis, vascular, neurological and gastrointestinal involvement. However, some patients still have refractory disease, relapse, sight-threatening eye disease or irreversible organ damage.
Recent improvements in the understanding of the pathogenic mechanisms have led to the identification of potential targets and future therapies for BD. In contrast to current nonspecific immunosuppressive agents mainly used empirically, the emergence of immunomodulatory drugs provides the possibility of interfering with specific pathogenic pathways. Novel targeted immunosuppressive therapies might be used in the future for BD.
Abstract and Introduction
Abstract
Behçet's disease (BD) is a systemic large-vessel vasculitis characterized by a wide clinical spectrum including recurrent oral and genital ulcerations, uveitis, and vascular, neurological, articular, renal and gastrointestinal manifestations. Therapeutic management of BD depends on the clinical presentation and organ involved. Although colchicine, nonsteroidal anti-inflammatory agents and topical treatments with corticosteroids are often sufficient for mucocutaneous and joint involvement, a more aggressive approach with immunosuppressive agents is warranted for severe manifestations such as posterior uveitis, retinal vasculitis and vascular, neurological and gastrointestinal involvement. However, some patients still have refractory disease, relapse, sight-threatening eye disease or irreversible organ damage. Recent improvements in the understanding of the pathogenic mechanisms have led to the identification of potential targets and future therapies for BD. In contrast to current nonspecific immunosuppressive agents, the emergence of immunomodulatory drugs provides the possibility of interfering with specific pathogenic pathways. Novel targeted immunosuppressive therapies might be used in the future for BD.
Introduction
Behçet's disease (BD) is a systemic vasculitis affecting arterial or venous vessels of any size, and characterized by a wide clinical spectrum including recurrent oral and genital ulcerations, uveitis, and vascular, neurological, articular, renal and gastrointestinal manifestations. Diagnosis is only based on clinical criteria as for BD there is no relevant biological test for diagnosis. BD is especially frequent along the ancient Silk Road, which extends from eastern Asia to the Mediterranean basin. Carrying HLA-B51 increases the risk of developing BD by 1.5–16 times. Except for the severity of ocular disease, HLA-B51 does not seem to be correlated with the prognosis of the disease. The exact cause of the disease remains unknown, but it is believed that both genetic and environmental factors contribute to its development.
BD significantly increases morbidity and mortality. The leading cause of morbidity in BD is eye involvement with the potential threat of visual loss. Male sex, arterial involvement and the number of flares are associated with mortality in BD.
Therapeutic management of BD depends on the clinical presentation and organ involved. Although colchicine, nonsteroidal anti-inflammatory agents and topical treatments are often sufficient for mucocutaneous and joint involvement, a more aggressive approach with immunosuppressive agents is warranted for severe manifestations such as posterior uveitis, retinal vasculitis, vascular, neurological and gastrointestinal involvement. However, some patients still have refractory disease, relapse, sight-threatening eye disease or irreversible organ damage.
Recent improvements in the understanding of the pathogenic mechanisms have led to the identification of potential targets and future therapies for BD. In contrast to current nonspecific immunosuppressive agents mainly used empirically, the emergence of immunomodulatory drugs provides the possibility of interfering with specific pathogenic pathways. Novel targeted immunosuppressive therapies might be used in the future for BD.