Background PAA secondary to BD is a rare condition but also is the leading cause of mortality. It is not usually investigated in BD unless there is a specific complaint. Although there have been increasing numbers of case reports on PAA from the countries in which it is common, total case numbers published, treatment modalities used and prognosis in the literature to date are not documented.
Objectives To review the literature based on the cases with PAA and BD.
Methods The countries which have over four million people from each continent were chosen (n=128). A PubMed search for “BD, PAA and the country name” was conducted and 18 countries with BD and PAA were identified. The full texts of articles (n=76) were obtained from these countries and the data including gender, age, treatment modalities and mortality rates were collected. When more than 5 cases were presented, it was accepted as case series.
Results There were a total of 191 cases (171 Male, 20 Female). The mean age of patients was 32±10. These have been published between 1981 and 2012. There were 82 case reports and 8 case series in total. The case series were reported from Turkey (n=7) and France (n=1). The most of the cases were from Turkey (n=135) (71%). One hundred eleven patients (58%) were treated with immunosuppressive therapy. The biologic treatment was only used in two (1%) patients from UK and USA. In 17 (9%) patients, embolization was chosen and it was more commonly used in patients from France. Forty one (22%) patients underwent surgery and it was more common in Iran, Israel and Tunisia. Of the 191 patients, 59 (31%) patients died and the death in 18 (31%) patients was related to the surgery (table).
Conclusions PAA is a rare condition and it is mostly reported as case reports from the countries where BD is more commonly found. Although the treatment modalities chosen could be different among the countries, the immunosuppressive treatment is the most common treatment used. The surgery carries out a high mortality risk.
Disclosure of Interest None declared