S. Celik1, Y. Yazici2, N. Sut3, H. Yazici4
2015 Vol.33, N°6 ,Suppl.94 - PI 0054, PF 0059
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