Celik Selda, Yazici Yusuf, Sut Necdet, Yazici Hasan
Division of Rheumatology, NYU Hospital for Joint Diseases, New York, USA.
Department of Biostatistics, Trakya University Medical Faculty, Edirne, Turkey.
Clin Exp Rheumatol. 2015 Nov-Dec;33(6 Suppl 94):S54-9. Epub 2015 Jul 23.
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.
调查不同国家报道的白塞病（BS）合并肺动脉瘤（PAA）这一BS最致命并发症的文献发表频率，并对诊断技术、治疗方法及预后进行综述。
选取每个大陆人口达400万及以上的国家（n = 128）。在PubMed上搜索“BS、PAA及国家名称”，确定了23个有BS和PAA报道的国家。对91篇文章的全文进行分析，获取包括性别、年龄、伴随血管表现、诊断技术、治疗方式及死亡率等数据。
来自23个国家的91篇文章共报道了207例（183例男性，24例女性）BS合并PAA患者。正如预期，关于BS的文章总数（n = 4431）与关于PAA和BS的文章数量之间存在显著相关性（r = 0.88，p < 0.001）。在简单线性回归分析中，日本关于BS和PAA的文章数量显著低于恒等线，而在土耳其，与PAA相关的文章发表倾向比预期更多。116例患者（56%）接受了免疫抑制治疗。生物制剂仅用于5例患者（2%）。207例患者中，62例（30%）死亡。
PAA大多作为病例报告在BS常见的国家报道。PAA在日本可能不常见。PAA的预后可能正在改善。
