title: Pulmonary artery aneurysms in Behçet's disease treated with anti-TNFα: A case series and review of the literature.
abstract: Behçet's disease (BD) is a systemic inflammatory disorder of unknown aetiology. Pulmonary haemorrhage from ruptured pulmonary artery aneurysms (PAA) in this condition carries a high mortality but treatment has largely been empiric with use of glucocorticoids and cyclophosphamide. Tumour necrosis factor α (TNF-α) was recently recognised as a mediator in the pathogenesis of BD inflammatory lesions. TNFα inhibitors have been shown in various case reports/series to have beneficial effects in uveoretinitis, entero-Behçet's, neuro-Behçet's and BD arthritis. We describe the efficacy and tolerability of infliximab in 2 patients with Behçet's disease complicated by pulmonary vasculitis admitted to our unit during the years 2004-2015, and discuss the previously published data in this area.
authors.affiliation: Louise Coote Lupus Unit, Fourth Floor Tower Wing, Guy's Hospital, London SE1 9RT, United Kingdom.
authors.forename: Estee
authors.initials: E
authors.lastname: Chan
authors.affiliation: Louise Coote Lupus Unit, Fourth Floor Tower Wing, Guy's Hospital, London SE1 9RT, United Kingdom.
authors.forename: Shirish R
authors.initials: SR
authors.lastname: Sangle
authors.affiliation: Department of Cardiology, Royal Free Hospital, London NW3 2QG, United Kingdom.
authors.forename: J Gerry
authors.initials: JG
authors.lastname: Coghlan
authors.affiliation: Louise Coote Lupus Unit, Fourth Floor Tower Wing, Guy's Hospital, London SE1 9RT, United Kingdom.
authors.forename: David D
authors.initials: DD
authors.lastname: D'Cruz
mesh_terms: D000328:Adult; D000783:Aneurysm / Q000209:etiology*; D000906:Antibodies / Q000627:therapeutic use*; D001528:Behcet Syndrome / Q000188:drug therapy* / Q000276:immunology / Q000473:pathology; D006801:Humans; D008297:Male; D011651:Pulmonary Artery; D014409:Tumor Necrosis Factor-alpha / Q000276:immunology*
chemical_list: D000906:Antibodies; C439524:TNF protein, human; D014409:Tumor Necrosis Factor-alpha
keywords: Anti-TNF; Behçet's; Infliximab; Pulmonary artery aneurysm; Tumour necrosis factor-α; Vasculitis