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Objective. To assess the influence of being free of major organ involvement during the early years of the disease on the prognosis of men with Behç et's syndrome (BS). Methods. Ninety-six men with BS, who had only active mucocutaneous manifestations when entering a controlled trial of thalidomide mean (S.D.) 11.7 (0.8) years ago, were re-evaluated for the use of immunosuppressives as an indication of major organ involvement during the post-trial period. Results. Outcome information was obtained in 91 (95%) patients. Thirty-nine (43%) patients had to use immunosuppressives during the post-trial period. Immunosuppressive use was significantly more frequent among patients developing BS at younger age (⩽24 years) than older age (30%; 525 years). Developing BS at young age (OR=6.3; 95% CI 2.09, 19.04) and not using colchicine during the posttrial period (OR=3.860; 95% CI 1.484, 10.034) were risk factors for immunosuppressive use. However, 82% of the patients using colchicine had onset during old age. Colchicine showed a significant effect in decreasing the use of immunosuppressives only among patients of old age at onset (Fisher's exact test=5.026; P=0.031) in the subgroup analysis. Eye disease (18 patients) and vascular involvement (14 patients) were the most frequent indications for immunosuppressive use. Conclusions. Being free of major organ involvement during the early years of BS does not indicate a mild prognosis for men developing BS at young age. Whether colchicine will reduce the need for immunosuppressive use among men developing BS at old age awaits formal studies. [ABSTRACT FROM AUTHOR]

OBJECTIVES: We had the impression and preliminary evidence that atherosclerosis was not much increased in Behçet's syndrome (BS). Thus, we evaluated the presence of subclinical atherosclerosis in a sizeable group of patients with BS both with major organ involvement and mucocutaneous disease along with diseased and healthy controls. METHODS: We studied 239 (162 M/ 77 F; mean age: 40.7 +/- 7.0) patients with BS. Seventy-two (32 M/ 40 F) had only mucocutaneous and/or joint disease and 167 (130 M/ 37 F) had major organ involvement. Also 100 (24 M/ 76 F; mean age: 44.7 +/- 7.1) patients with rheumatoid arthritis (RA), 74 (58 M/ 16 F; mean age: 39.4 +/- 7.0) patients with ankylosing spondylitis (AS) and 156 (83 M/ 73 F; mean age: 39.2 +/- 6.6) healthy controls (HC) were studied as the control groups. We used B-mode USG to assess the frequency of plaques and intima-media thickness (IMT) in the carotid and femoral arteries. Traditional atherosclerotic risk factors were also evaluated. Men and women were analyzed separately. RESULTS: The frequency of plaques and the mean IMT in the carotid and femoral arteries were similar between patients with BS, AS and HC and also between the 2 subgroups of BS, among both men and women. Only men with RA were found to have significantly increased frequency of carotid artery plaques after adjustment for atherosclerotic risk factors. CONCLUSION: Increased atherosclerosis is not a prominent feature of BS, even among those patients with major organ involvement. © 2008 Elsevier Inc. All rights reserved. [ABSTRACT FROM AUTHOR]

Objective .-To study the frequencies and characteristics of different headache types seen in patients with Behçet's syndrome (BS) in a large cohort of patients. Background .-Patients with BS may present with different neurological problems, related either directly or indirectly to the disease, with headache being the most common neurological symptom seen in this syndrome, as well as independent from neurologic involvement. Method .-This study was carried out at the multidisciplinary Behçet outpatient clinic of the Behçet's Syndrome Research Center. Every fifth admitted patient who had fulfilled the International Study Group for Behçet's Disease classification criteria was recruited for this study. Each patient was interviewed by one of the examining neurologists through a semistructured questionnaire. In case of the presence of more than one headache type, the best defined one was diagnosed and evaluated. All patients received a complete neurological and physical examination. A X2 test was used to evaluate the differences between frequencies. One sample t-test was used to compare means. The prevalence rates of primary headaches were compared with results of the Turkish Headache Epidemiology study by using goodness-of-fit test. Results .-Two hundred and twenty-eight patients were studied. Headache was reported in 66.2% (151/228) of the study cohort. Primary headaches were seen in 38.6% of the patient population, which corresponds to 58% of BS patients with any type of headache. Tension-type headache (TTHA) and migraine were seen in 23.6% and 14.9% of the whole study cohort, respectively. In 5.2% of the study cohort, the headache was associated with neurological involvement and in 3.9% to uveal inflammation. A predominantly frontal, bilateral paroxysmal throbbing pain of moderate severity was reported in 18.4% of the study cohort who did not fulfill the criteria of the International Headache Society (IHS) for any of the primary headaches. It was commonly associated with exacerbations of the mucocutaneous symptoms of the syndrome. The neurological examination was normal in all of these patients. This type of headache was categorized as the nonstructural headache of Behçet. Conclusion .-Headache is the most common neurological symptom seen in BS, both in patients with and without neurological involvement. The results of our study reveal that headache is seen in a majority of patients with neurological involvement due to BS and in a minority with uveal inflammation. The prevalence of migraine and TTHA are close to the population in general, but a nonstructural migrainous headache, which is commonly associated with exacerbations with some of the systemic symptoms of the syndrome, is noteworthy in patients with BS. This form of headache is not specific for this disorder, but may be explained by a vascular headache triggered by the immunomediated disease activity in susceptible individuals. [ABSTRACT FROM AUTHOR]

: Despite being recognised much more frequently than in the past, renal involvement has not previously been regarded as a feature of Behçet’s disease (BD). In this study we aimed to assess the frequency of renal involvement in BD by performing urinalyses of 674 consecutive BD patients; we also retrospectively evaluated the charts of 4212 BD patients for the incidence of glomerulonephritis (GN). Urinary abnormalities (proteinuria and/or haematuria) were present in 10.8%; and during a period of 23 years GN was detected by renal biopsy in seven (0.16%) BD patients. Two patients with GN were lost to follow-up; end-stage renal failure developed in only one patient, and she underwent renal transplantation. We were unable to determine any pathognomonic feature that was predictive of renal involvement. Although males tend to have a more serious clinical course of BD the incidences of urinary abnormalities and GN were similar in both sexes in our series. According to our results, we can conclude that urinary abnormalities are more frequent in BD; however, serious renal lesions develop in only very few of these patients. [ABSTRACT FROM AUTHOR]

Objective: To examine the presence of genetic anticipation in families with Behçet's syndrome (BS).Methods: A total of 18 families with 40 affected members in two successive generations were evaluated by interviewing them for their ages at the onset of the first symptom of BS and for their ages at the time they fulfilled the diagnostic criteria.Results: It was noted that the age of onset of the first symptom was lower in the second generation in 14 families (p = 0.01) with a mean (SD) age of 20.57 (7.47) years in the children compared with 33.29 (9.92) years in the parents (t = 7.79, p < 0.0001), whereas the diagnostic criteria were fulfilled at an earlier age in the children in 15 families (p = 0.01) with a mean age of 21.2 (6.74) years in the children compared with 36.4 (9.55) years in the parents (t = 7.41, p < 0.0001).Conclusion: Genetic anticipation was present in 15 of 18 (84%) of the families with BS in the form of earlier disease onset in the children compared with their parents. [ABSTRACT FROM AUTHOR]

The cardiac involvement in Behçet's syndrome in sixty-five patients (mean disease duration 5.7±4.1 SD years) was studied, in comparison with 70 healthy, age- and sex-matched controls. The electrocardiograms, chest radiographs and M-mode, 2-D and Doppler echocardiography were evaluated in a blind protocol. There were no significant differences in the prevalence of cardiac findings between the probands and the controls. It is concluded that cardiac involvement in Behçet's syndrome occurs only sporadically. [ABSTRACT FROM PUBLISHER]

Background: Recurrent oral and genital ulcers are the most frequent problem in the management of the Behçet syndrome. Uncontrolled experience suggests that thalidomide may help prevent recurrences of these ulcers.Objective: To determine the efficacy of two thalidomide dosages in the treatment of mucocutaneous lesions of the Behçet syndrome.Design: Randomized, double-blind, placebo-controlled trial.Setting: Specialist outpatient clinic for the Behçet syndrome in Turkey.Patients: 96 male patients with the Behçet syndrome who primarily had mucocutaneous lesions without major organ involvement.Intervention: Thalidomide, 100 mg/d or 300 mg/d, or placebo for 24 weeks.Measurements: Sustained absence of any oral and genital ulceration during treatment (complete response) and changes in the number of mucocutaneous lesions. An additional evaluation was done 4 weeks after treatment ended.Results: A complete response occurred in 2 of the 32 patients (6% [95% CI, 0.8% to 20.8%]) receiving thalidomide, 100 mg/d; in 5 of the 31 patients (16% [CI, 5.5% to 33.7%]) receiving thalidomide, 300 mg/d; and in none of the 32 patients (0% [CI, 0% to 10.9%]) receiving placebo (P = 0.031). The suppressive effect of thalidomide with either dosage was evident at 4 weeks for oral ulcers (P < 0.001) and at 8 weeks for genital ulcers (P < 0.001) and follicular lesions (P = 0.008). This effect persisted during treatment but diminished rapidly after treatment was discontinued. Both thalidomide dosages led to significant increases in the number of erythema nodosum lesions during the first 8 weeks of treatment (P = 0.03). Polyneuropathy developed in 4 patients (1 in the 100-mg/d group and 3 in the 300-mg/d group); in 3 of these patients, the condition was diagnosed after the trial had ended.Conclusions: Thalidomide is effective for treating the oral and genital ulcers and follicular lesions of the Behçet syndrome. A dosage of 100 mg/d is as effective as a dosage of 300 mg/day. [ABSTRACT FROM AUTHOR]

Monocytes obtained from patients with Behçet's disease (BD) were examined for differentiation markers (expression of CD14 and antigens reacting with monoclonal antibodies 25F9 and G16/1) and for expression of selected adhesion molecules. There was significantly raised expression of the CD14 molecule, and increased staining with 25F9 and G16/1 antibodies in monocytes obtained from patients with BD. A monocyte activation marker, soluble CD 14, was also found to be raised in patients' serum compared with normal serum (8.1 ± 9.2 vs 1.4 ± 0.7 μg/ml). Furthermore, BD patients' monocyte culture supernatants caused significantly increased adhesion of normal neutrophils to endothelial cell monolayers in vitro. All these findings show that BD patients' monocytes are active in vivo and produce a number of pro-inflammatory cytokines which may play a role in the chronic inflammation found in these patients. [ABSTRACT FROM PUBLISHER]

Pulmonary arterial involvement is an important complication of Behçet's syndrome (BS). Among 2179 patients with BS, 24 (1.1%) were diagnosed as having pulmonary arterial aneurysms (PAAs). Haemoptysis was the presenting symptom in all but one. All were male. The mean age at the time of the diagnosis of PAA was 30 ± 11 S.D. yr (range 17–59 yr). Their mean disease duration was 5 ± 4 yr (range 3 months-16 yr). There was a high prevalence of thrombophlebitis (21/24, 88%). Histopathological examination showed pulmonary vasculitis involving all layers of pulmonary arteries and veins. Twelve patients (50%) died after a mean of 9.5 ± 11 S.D. months (range 1–36 months) after the onset of haemoptysis. The mean duration of follow-up of the remaining 12 patients was 25.5 ± 24 S.D. months (range 1–78 months). The treatment consisted mainly of pulsed or oral cyclophosphamide alone or with prednisolone. As is true with other severe manifestations of Behçet's syndrome, PAAs are more common among males. They are associated with a prevalence of thrombophlebitis and there is high mortality despite treatment. [ABSTRACT FROM PUBLISHER]

A single masked trial of cyclosporin A 5 mg/kg/day versus monthly 1 g intravenous boluses of cyclophosphamide was conducted among 23 patients with Behçet's syndrome and active, potentially reversible uveitis. The trial was unmasked after a mean of 12 (SD 2) months for the cyclosporin A group (n = 12) and a mean of 10 (SD 3) months for the cyclophosphamide group (n = 11). During the initial 6 months the visual acuity significantly improved (p < 0.001) in the cyclosporin A group whereas this was not observed in the cyclophosphamide group. The subsequent follow-up of patients up to 24 months suggested that the initial improvement in visual acuity with cyclosporin A was not sustained. More extensive and especially long-term studies of cyclosporin A in the uveitis of Behçet's syndrome are warranted. [ABSTRACT FROM PUBLISHER]

We surveyed the 10-yr mortality among 152 Behçet's syndrome (BS) patients who had registered at a BS out-patient clinic and compared it to the expected mortahty in the general population. Information on mortality was available in 79% of the study group, among whom six patients (all male) had died. The observed mortality of two patients in the 15–24 yr age bracket was significantly above that expected in the general population. BS is a cause of increased mortality in the young male patients. [ABSTRACT FROM PUBLISHER]