2%), neurological manifestations (15.3%), gastrointestinal manifestations (6.3%) and epididymitis (7.2%). Some manifestations are rarely seen, such as pleuropulmonary (1.8%), and cardiac manifestations (1.8%). Diagnosis is mainly clinical, on the association of symptoms, but diagnosis/classification

criteria may help. Sixteen sets of criteria were created for BD. The first was in 1946 and the last in 2010. The International Study Group (ISG) criteria, created by seven countries in 1990, had a low sensitivity and accuracy. The ICBD was created by 27 countries in 2006. It was revised in 2010.[5] In ICBD, skin lesions, vascular lesions, neurological manifestations and positive pathergy tests each get one point. Oral aphthosis, genital aphthosis and ocular lesions each get two points. To be classified/diagnosed as BD, a patient has to get four points (or more). Behcet’s disease manifestations are self-limiting, but recurrent. Selleckchem ABT 263 learn more Some heal without a sequelae, but others are the main cause of morbidity, such as ophthalmological manifestations which may cause blindness if not aggressively treated. Some may cause mortality, as in some of the vascular, neurological, cardiac or pulmonary involvements. The first group of manifestations (healing without sequelae) may need no treatment if the frequency of their recurrence is low and

the burden acceptable when the lesion is active.[4] In the others colchicine is the main treatment, given at 1 mg daily, at night. If not effective in reducing the frequency of attacks, or their severity, low-dose methotrexate (MTX) with low-dose prednisolone can be given. The same will be given for genital aphthoses.

For resistant cases, pimecrolimus, an ointment for local application, may be effective. Skin manifestations may respond to colchicine. If they do not, non-steroidal anit-inflammatory check details drugs (NSAIDs) or MTX with low-dose prednisolone will suffice in the majority of cases. Joint manifestations are usually transient and NSAIDs will work. In cases of chronic arthritis, the patient can be treated as with rheumatoid arthritis or seronegative sponyloarthritis. The second group, those with high morbidity or mortality, need aggressive treatment with cytotoxic drugs and medium- to high-dose steroids.[4] Pulse cyclophosphamide,[6] azathioprine, cyclosporine A, chlorambucil[7] and MTX[8] can be used, depending on the severity of lesions. Prednisolone has to be associated at 0.5 mg to 1 mg/kg of body weight. In resistant cases to cytotoxic drugs and prednisolone, a combination of cytotoxics can be used.[6] Another choice is the addition of biologic agents,[4],whether anti-tumor necrosis factor-α or anti-CD20. There are still no long-term reports or control studies for cytotoxic drugs or biologic agents. In this issue of the International Journal of Rheumatic Diseases, six papers on BD are presented.