title: Pulmonary artery aneurysms in Behçet's syndrome: a review of the literature with emphasis on geographical differences.
abstract: OBJECTIVE
  To investigate the frequency of Behçet's syndrome (BS) with pulmonary artery aneurysms (PAA) publications, the most lethal complication of BS, as reported from different countries and to provide a review of diagnostic techniques, treatment approaches and prognosis.
  
  
  METHODS
  Countries from each continent with a population of 4 million and over were chosen (n=128). A PubMed search for "BS, PAA and the country name" was conducted and 23 countries with BS and PAA were identified. The full texts of articles (n=91) were analysed for data including gender, age, accompanying vascular findings, diagnostic techniques, treatment modalities and mortality rates.
  
  
  RESULTS
  A total of 207 (183 males, 24 females) patients with BS and PAA were reported in 91 articles originating from 23 countries. As expected there was a significant correlation (r=0.88, p<0.001) between the total number of articles about BS (n=4431) and those related to PAA and BS. In a simple linear regression analysis the number of BS and PAA articles from Japan was significantly below the identity line while in Turkey there was a propensity to publish more articles related to PAA than expected. One hundred and sixteen patients (56%) were treated with immunosuppressive therapy. Biologics were used only in 5 patients (2%). Of the 207 patients, 62 (30%) died.
  
  
  CONCLUSIONS
  PAA is mostly reported as case reports from countries where BS is common. PAA might be uncommon in Japan. The prognosis of PAA could be getting better.
authors.affiliation: Division of Rheumatology, NYU Hospital for Joint Diseases, New York, USA. seldasevda@yahoo.com.
authors.forename: Selda
authors.initials: S
authors.lastname: Celik
authors.affiliation: Division of Rheumatology, NYU Hospital for Joint Diseases, New York, USA.
authors.forename: Yusuf
authors.initials: Y
authors.lastname: Yazici
authors.affiliation: Department of Biostatistics, Trakya University Medical Faculty, Edirne, Turkey.
authors.forename: Necdet
authors.initials: N
authors.lastname: Sut
authors.affiliation: Division of Rheumatology, Istanbul University, Cerrahpasa Medical Faculty, Istanbul, Turkey.
authors.forename: Hasan
authors.initials: H
authors.lastname: Yazici
mesh_terms: D000293:Adolescent; D000328:Adult; D000783:Aneurysm / Q000175:diagnosis / Q000453:epidemiology* / Q000628:therapy; D001528:Behcet Syndrome / Q000175:diagnosis / Q000453:epidemiology* / Q000628:therapy; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D011651:Pulmonary Artery; D012307:Risk Factors; D055815:Young Adult