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Pulmonary artery aneurysms in Behçet’s syndrome: a review of the literature with emphasis on geographical differences
S. Celik1, Y. Yazici2, N. Sut3, H. Yazici4
- Division of Rheumatology, NYU Hospital for Joint Diseases, New York, USA. seldasevda@yahoo.com
- Division of Rheumatology, NYU Hospital for Joint Diseases, New York, USA.
- Department of Biostatistics, Trakya University Medical Faculty, Edirne, Turkey.
- Division of Rheumatology, Istanbul University, Cerrahpasa Medical Faculty, Istanbul, Turkey.
CER8269
2015 Vol.33, N°6 ,Suppl.94
PI 0054, PF 0059
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PMID: 26211653 [PubMed]
Received: 07/01/2015
Accepted : 22/04/2015
In Press: 23/07/2015
Published: 04/11/2015
Abstract
OBJECTIVES:
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.
