Abstract
Behçet's disease (BD) is a multisystem disorder characterized by vasculitis. Pulmonary vascular problems such as pulmonary artery aneurysms (PAA) are reported to indicate poor prognosis and high mortality. We describe a 43-year-old man who presented with life threatening bilateral PAA and thromboembolic disease due to BD. He was treated with prednisone and pulse cyclophosphamide and was poorly responsive to the conventional immunosuppression. The introduction of adalimumab therapy stabilized his PAA. We report that the inhibition of TNF-alpha using the neutralizing monoclonal antibody adalimumab has the potential to induce rapid, complete, and long-lasting remission in a life-threatening manifestation of BD.
MeSH terms
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Adalimumab
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Adult
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Aneurysm / diagnostic imaging
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Aneurysm / drug therapy
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Aneurysm / etiology*
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Anti-Inflammatory Agents / therapeutic use*
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Antibodies, Monoclonal / therapeutic use*
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Antibodies, Monoclonal, Humanized
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Behcet Syndrome / complications*
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Behcet Syndrome / diagnostic imaging
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Behcet Syndrome / drug therapy
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Cyclophosphamide / therapeutic use
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Drug Therapy, Combination
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Humans
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Immunosuppressive Agents / therapeutic use
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Male
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Prednisone / therapeutic use
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Pulmonary Artery* / diagnostic imaging
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Tomography, X-Ray Computed
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Treatment Outcome
Substances
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Anti-Inflammatory Agents
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Antibodies, Monoclonal
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Antibodies, Monoclonal, Humanized
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Immunosuppressive Agents
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Cyclophosphamide
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Adalimumab
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Prednisone