title: Pulmonary artery involvement in Behçet׳s syndrome: Effects of anti-Tnf treatment.
abstract: OBJECTIVE
  Anti-TNF agents are being increasingly used in patients with Behçet׳s syndrome (BS) when conventional immunosuppressives fail. However, experience with anti-TNF treatment on pulmonary artery involvement (PAI) of BS is limited.
  
  
  METHODS
  A chart review revealed 13 patients with PAI (all men) treated with anti-TNF agents (12 infliximab and 1 adalimumab) following an inadequate response to immunosuppressives for 12.2 ± 9.5 SD months and 2 male patients who developed PAI while receiving infliximab for large vein thrombosis for 10 months and for parenchymal central nervous system involvement for 2 years, respectively.
  
  
  RESULTS
  The first patient developing PAI while receiving infliximab responded to cyclophosphamide and prednisolone but the second died with hemoptysis within 1 month. At the end of the survey, 6 of the 13 patients with PAI were continuing these agents for 25.5 ± 16.2 SD months with good response, 4 stopped anti-TNF treatment after a mean of 23 ± 9.8 SD months after achieving clinical and radiologic response and 1 patient with good response went to another center after receiving infliximab for 10 months and the remaining 2 experienced serious infections (lung tuberculosis and aspergillosis) necessitating early withdrawal. Two patients relapsed within 3 years after stopping anti-TNF agents and concomitant azathioprine. One developed mesenteric vein thrombosis necessitating bowel resection and the second developed new PAI that was controlled with cyclophosphamide and prednisolone after short courses of infliximab, adalimumab, and canakinumab.
  
  
  CONCLUSIONS
  Anti-TNF treatment seems to be effective for refractory PAI of BS but may not prevent its development. Relapses can be seen after withdrawal. Caution is required for their serious adverse effects.
authors.affiliation: Department of Internal Medicine, Cerrahpasa Medical Faculty, Istanbul University, Fatih, Istanbul, Turkey. Electronic address: vhamuryudan@yahoo.com.
authors.forename: Vedat
authors.initials: V
authors.lastname: Hamuryudan
authors.affiliation: Department of Internal Medicine, Cerrahpasa Medical Faculty, Istanbul University, Fatih, Istanbul, Turkey.
authors.forename: Emire
authors.initials: E
authors.lastname: Seyahi
authors.affiliation: Department of Internal Medicine, Cerrahpasa Medical Faculty, Istanbul University, Fatih, Istanbul, Turkey.
authors.forename: Serdal
authors.initials: S
authors.lastname: Ugurlu
authors.affiliation: Department of Internal Medicine, Cerrahpasa Medical Faculty, Istanbul University, Fatih, Istanbul, Turkey.
authors.forename: Melike
authors.initials: M
authors.lastname: Melikoglu
authors.affiliation: Department of Internal Medicine, Cerrahpasa Medical Faculty, Istanbul University, Fatih, Istanbul, Turkey.
authors.forename: Gulen
authors.initials: G
authors.lastname: Hatemi
authors.affiliation: Department of Internal Medicine, Cerrahpasa Medical Faculty, Istanbul University, Fatih, Istanbul, Turkey.
authors.forename: Yesim
authors.initials: Y
authors.lastname: Ozguler
authors.affiliation: Department of Radiology, Cerrahpasa Medical Faculty, Istanbul University, Istanbul, Turkey.
authors.forename: Canan
authors.initials: C
authors.lastname: Akman
authors.affiliation: Department of Cardiothoracic Surgery, Cerrahpasa Medical Faculty, Istanbul University, Istanbul, Turkey.
authors.forename: Hasan
authors.initials: H
authors.lastname: Tuzun
authors.affiliation: Department of Internal Medicine, Cerrahpasa Medical Faculty, Istanbul University, Fatih, Istanbul, Turkey.
authors.forename: Sebahattin
authors.initials: S
authors.lastname: Yurdakul
authors.affiliation: Department of Internal Medicine, Cerrahpasa Medical Faculty, Istanbul University, Fatih, Istanbul, Turkey.
authors.forename: Hasan
authors.initials: H
authors.lastname: Yazici
mesh_terms: D000068879:Adalimumab / Q000494:pharmacology / Q000627:therapeutic use*; D000328:Adult; D000783:Aneurysm / Q000188:drug therapy* / Q000209:etiology; D018501:Antirheumatic Agents / Q000494:pharmacology / Q000627:therapeutic use*; D001528:Behcet Syndrome / Q000150:complications / Q000188:drug therapy*; D006801:Humans; D000069285:Infliximab / Q000494:pharmacology / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D011651:Pulmonary Artery / Q000187:drug effects*; D012189:Retrospective Studies; D013927:Thrombosis / Q000188:drug therapy* / Q000209:etiology; D016896:Treatment Outcome; D014409:Tumor Necrosis Factor-alpha / Q000037:antagonists & inhibitors
chemical_list: D018501:Antirheumatic Agents; D014409:Tumor Necrosis Factor-alpha; D000069285:Infliximab; D000068879:Adalimumab