Behcet’s disease


Abstract: 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. 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 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, chlorambucil7 and MTX8 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 antitumor necrosis factor-a 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. Arslan proposes the mevalonate kinase gene mutation as an additional gene susceptibility factor.9 Khabbazi et al.10 showed that vitamin D levels were lower in BD patients than the healty subjects, but this did not relate to disease activity. Dormohammadi et al.11 demonstrated OA resistant to treatment was not related to iron deficiency. Yoon in a series of 624 patients reported neuro-BD to be 3.5% in South Korea,12 very near to the previous report of Bang et al.13 from 2001. Shadmanfar reports that, contrary to previous reports, there was no relationship between plasma homocysteine levels and HLA B- 51 in BD patients, nor in their control group.14 Lin reports 6.3% of malignancy in hospitalized BD patients15, which is much higher than previous reports (6.3% vs. 0.24%).16