The effect of immunosuppressive use on the development new major organ involvement in behcet's disease

INTRODUCTION: Behcet's disease (BD) is a chronic inflammatory disease that can affect eye, nervous system (CNS), vascular and gastrointestinal systems (GIS) as well as skin, mucosa and joint involvement. Immunosuppressive (IS) agents are used for the treatment and the prevention of major organ involvement in Behcet's disease. OBJECTIVES: We aimed to investigate the rate of new major organ involvement development in patients under IS treatment for any reason during follow-up and to assess the characteristics and treatment protocols of these patients. MATERIALS AND METHODS: Data were collected retrospectively from files of 1114 patients who were followed in Marmara University Faculty of Medicine, Rheumatology Clinic. 54 patients were excluded from the study because they failed to meet the criteria of the International Behcet's Disease Study Group (ISBGD). Patients with a follow-up period of 6 months or less were excluded, and a total of 806 patients were included into the analysis. Demographic and clinical characteristics, follow-up and treatment data of the patients were recorded from files. Relapse of the same organ and/or new major organ development during the follow-up period of patients receiving IS was defined as "event under IS". RESULTS: 806 patients were included in the study, of whom %56 were male. Median follow-up time was 64 months and median age at diagnosis was 29. Genital ulcer, erythema nodosum, arthritis / arthralgia were more common in women whereas papulopustular lesions and major organ involvement were more common in men. 232 (%50,5) patients had major organ involvement at diagnosis, 227 (%49,5) patients developed new major organ involvement at follow-up. Major organ involvement developed earlier in males (p = 0.012) and in those with a first-degree relative history of BD (1 year after diagnosis). The frequency of vascular, ocular, CNS and GIS involvement was 29.8%, 33,5%, 9.7%, and 2%, respectively. The majority (86.8%) of 440 patients were received IS due to major organ involvement. In these patients, the rate of relapse and new major organ involvement was %24 and %11.6, respectively. In the patients with "event under IS" , %91 of the patients with relapse and %75 of the patients with new major organ involvement were using azathioprine. CONCLUSION: Major organ involvement occurred in %57 of the patients. In addition, it was found that the course of the disease was more severe under IS treatment in male patients who were diagnosed at a younger age and had a history of BD in their 1st degree relatives. In %36 of the patients who received IS therapy, relapse or new major organ involvement developed under IS treatment. The majority of IS agents was azathioprine. These results suggest that earlier and more aggressive treatment approaches may be more beneficial both in mucocutaneous disease and in major organ involvement.