Pulmonary artery aneurysms in Behçet’s syndrome: a review of the literature with emphasis on geographical differences

1, 2, 3, 4

  1. Division of Rheumatology, NYU Hospital for Joint Diseases, New York, USA. seldasevda@yahoo.com
  2. Division of Rheumatology, NYU Hospital for Joint Diseases, New York, USA.
  3. Department of Biostatistics, Trakya University Medical Faculty, Edirne, Turkey.
  4. Division of Rheumatology, Istanbul University, Cerrahpasa Medical Faculty, Istanbul, Turkey.

CER8269 Submission on line
2015 Vol.33, N°6 ,Suppl.94 - PI 0054, PF 0059
Full Papers

Rheumatology Article



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.
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.
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.
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.

PMID: 26211653 [PubMed]

Received: 07/01/2015 - Accepted : 22/04/2015 - In Press: 23/07/2015 - Published: 04/11/2015