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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]

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]

Objective: In Romania, a national statistics report on the mode of delivery in pregnancies that ends in stillbirths has never been conducted. Thus, we decided to analyze the incidence of cesarean section versus vaginal delivery rate over a 10-year period in Filantropia Clinical Hospital of Bucharest., Materials and Methods: We conducted a retrospective analysis over a period of 10 years from January 2005 to December 2015. Maternal age, parity, social status, place of origin, educational level, gestational age, fetal presentation and fetal sex were studied in order to see if there were a scientific correlation with death in utero., Results: Between 2005 and 2015, Filantropia Clinical Hospital had a total of 31676 births after the 28th week of gestation. During this time period, 174 (0.55%) stillbirths were registered. The overall number of cesarean sections in live-births was 13199 (41.7%) and the rate of cesarean sections calculated for the stillbirth was 17.24% (30/174)., Conclusion: Our study revealed that the rate of cesarean sections in stillbirth had a dramatic decrease based on the adoption of guidelines reflecting evidence based medicine. The vast majority of pregnancies included in our study did not undergo standard perinatal tests and screenings that ensure a healthy and safe delivery, as it is a known fact that many causes of perinatal deaths can be prevented by health care access and perinatal regular visits. We suggest that a proper follow up in the last trimester and easy access to health care facilities can lower the incidence of stillbirths in Romania.

Objectives: There had been evidence that low-dose local IFN could be beneficial in the management of recurrent oral ulcers (OUs). We investigated the efficacy and collected initial data on the safety of low-dose natural human IFN-alpha administered by the oral mucosal route in Behçet's syndrome (BS) in a placebo controlled, double blind study., Methods: Eighty-four (59 males and 25 females) patients with BS with mainly skin mucosa disease and a history of recurrent OU for > or = 1 year were studied. When they had at least two OUs with a total diameter of > or = 4 mm, they were randomly allocated to (i) 2000 IFN-alpha IU/day, (ii) 1000 IFN-alpha IU/day and (iii) placebo groups. Subjects were monitored weekly over an initial 4 weeks and bi-weekly for an additional 8 weeks of treatment. OU were counted and measured at each study visit. The primary efficacy end point was the difference in the total ulcer burden at Week 0 compared with that at Week 12., Results: Out of the 84 patients enrolled, 72 completed the trial. There were no statistically significant differences between the treatment arms in terms of the primary endpoint., Conclusions: Low-dose natural human IFN-alpha did not have beneficial effects on reducing the total ulcer burden among BS patients from Turkey. The study also showed that counting the number of ulcers rather than measuring the size would be adequate in future studies., Trial Registration: ClinicalTrials.gov, NCT00483184, http://www.clinicaltrials.gov/ct2/results?term=NCT00483184.

Objective: Behçet's syndrome (BS) has many features that are different from autoimmune diseases, including a lack of association with Sjögren's syndrome. Vitiligo is frequently associated with various autoimmune disorders such as autoimmune thyroiditis, pernicious anemia and Addison's disease. Our informal observation was that vitiligo is also uncommon among BS patients. With this controlled and masked study we formally surveyed the presence of vitiligo among BS patients and suitable controls., Methods: Patients with Behçet's syndrome, Graves' disease, and Hashimoto's thyroiditis being followed in the rheumatology and endocrinology departments of a university hospital and healthy controls were examined. Subjects with hypopigmented lesions were re-examined by a dermatologist in a masked protocol. Wood's lamp was used to confirm the diagnosis of vitiligo in suspected lesions., Results: 253 consecutive BS patients, 34 Graves' disease patients, 32 Hashimoto's thyroiditis patients, and 439 healthy controls were surveyed. None of the BS patients had vitiligo, while 6/34 (17.6%) of Graves' disease patients, 6/32 (18.7%) of Hashimoto's thyroiditis patients, and 4/439 (0.9%) of healthy controls had vitiligo. All the subjects with vitiligo, except for one patient with associated Graves' disease, were women., Conclusion: In contrast to two autoimmune diseases, Hashimoto's thyroiditis and Graves' disease, the frequency of vitiligo was not increased among patients with BS. This constitutes further evidence that traditional autoimmune mechanisms may not be operative in BS.

Objective: Crohn's disease (CD) and ulcerative colitis (UC) share common clinical features with Behçet's syndrome (BS). We surveyed UC and CD patients for pathergy phenomenon and features of BS with the aim of determining how much overlap is present between these 2 entities in a setting where BS is relatively common, the frequency of pathergy positivity in inflammatory bowel disease (IBD) patients and evaluating how International Study Group (ISG) criteria perform in differentiating IBD from BS., Methods: This study was conducted among patients with CD and UC attending the gastroenterology outpatient clinic of a university hospital which is also a referral center for BS. Consecutive CD and UC patients were screened for BS using ISG criteria. Pathergy test was performed and evaluated by 2 independent observers in a masked manner., Results: Ninety-three patients with CD and 130 with UC were surveyed. None of the CD patients fulfilled ISG criteria for BS while 2 of 130 UC patients did. Twenty CD patients had oral ulcers while 4 reported having genital ulcers but no scars could be observed. Twenty-two CD patients had papulopustular lesions, 2 had nodular lesions, 3 had arthritis and none had uveitis. Thirty-two UC patients had oral ulcers, none had genital ulcers, 23 had papulopustular lesions, 3 had nodular lesions, 2 had arthritis and 2 had uveitis. Pathergy test was positive according to at least one of the observers in 10/93 CD and 8/130 UC patients and according to both observers in 4/130 UC patients., Conclusion: Despite similarities between the clinical features of CD and UC with BS, coexistence is uncommon. ISG criteria perform well in differentiating these diseases. About 8% of IBD patients show the pathergy phenomenon.

Background: There is increasing evidence that lactobacilli, having anti-inflammatory activity, may be useful in some diseases, particularly in inflammatory bowel disease., Objectives: We aimed to study the efficacy of lactobacilli lozenges (INERSAN, VSL Pharmaceuticals, USA) in the management of oral ulcers of Behçet's syndrome., Patients and Methods: Twenty-five patients were studied before, during and after lactobacillus use. All patients had mainly skin-mucosa involvement with fresh oral ulcers and none were using systemic immunosuppressives. The patients chewed on 6 lozenges/day at intervals of 2-3 hours during the time they were awake for 7 days. The number of OU before visit 1 and after 1. and 2. weeks after drug use was the main outcome measure., Results: Twenty three patients completed the trial. 1 patient left the trial the second day because of nausea and another was a no show at the 2.visit. There was a significant decrease in the mean number of ulcers following treatment, more pronounced among the females, and this decrease continued at least for 1 week after the treatment was stopped. 4/23 patients had side effects with 3 complaining of nausea and 1 of abdominal fullness., Conclusions: In this pilot and open study Lactobacillus brevis CD2 lozenges seemed to be effective in controlling the OU of BS. Randomized, controlled trials with this seemingly innocuous agent are now needed.

This paper focuses on skin manifestations that can be observed in autoimmune diseases such as rheumatoid arthritis (RA), Sjögren syndrome (SS), dermatomyositis (DM) and Behçet syndrome (BS). In RA the most widely recognized skin lesion is the rheumatoid nodule. Other cutaneous manifestations can be observed either non-specific or related to the disease itself and/or to the commonly used drugs. Cutaneous manifestations are considered one of the most typical extraglandular features of primary SS, generally they are distinguished in vasculitic and non vasculitic lesions. Among non-vasculitc lesions, skin dryness (xerosis) has been shown to be very common in pSS while vasculitis lesions include typically flat and palpable purpura and urticarial vasculits. In DM the skin manifestations are also frequent and include a heliotrope rash (blue-purple discoloration) on the upper eyelids with edema, a flat red rash on the face and upper trunk, and erythema of the knuckles with a raised violaceous scaly eruption (Gottron rash). The most frequent mucocutaneous finding in BS is aphthous stomatitis which can not usually be differentiated from idiopatic reccurrent aphthous stomatitis on clinical grounds. The most typical skin manifestations are nodular lesions, which are commonly seen in BS and may be due to panniculitis [erythema nodosum (EN)-like lesions] or superficial thrombophlebitis.

Background: Eye involvement is the main cause of morbidity in Behçet's syndrome. The efficacy of the combined use of azathioprine and interferon alfa in treating this condition has not been studied., Methods: Ten male BD patients with retinal involvement but no irreversible structural changes were treated with azathioprine 2.5 mg/kg/day and IFN alpha 2b three times weekly for 24 weeks in an open trial. At week 24, IFN alpha was stopped and the patients continued to use azathioprine or received other immunosuppressives as indicated. Clinical response was assessed by visual acuity changes of either eye under the combination treatment and during the follow-up after stopping interferon., Results: As compared to the study entry, the mean visual acuities of either eye increased significantly at the end of the combination treatment (right eye 5.8 +/- 1.26 vs. 8.3 1.14; P = 0.043; left eye 6.3 1.15 SEM vs. 9.1 +/- 0.9 SEM; P = 0.027). The improvement in visual acuity persisted in the nine patients who were followed for 7.2 +/- 1.6 SEM months after stopping interferon. Reversible hematologic toxicity, mostly in the form of leukopenia, was detected in six patients during the combination treatment., Conclusions: The combination of IFN alpha and azathioprine appears to be effective for eye involvement of BD. However, the frequent occurrence of myelosuppression mandates close monitoring.

Objective: Colchicine is a widely used treatment for Behçet's syndrome, even though in a previous 6-month controlled study, it was shown to be effective only in controlling erythema nodosum and arthralgias. We reassessed the effect of colchicine in Behçet's syndrome in a study conducted among a larger group of patients for 2 years., Methods: We randomized 116 patients with Behçet's syndrome (60 male/56 female), who had active mucocutaneous disease without eye or major organ involvement, to receive either placebo or colchicine (1-2 mg/day, adjusted to body weight) in a double-blind trial for 2 years. The primary outcome measure was the sustained absence of any lesions during treatment (complete response). The secondary outcome measure was the difference in the number of mucocutaneous lesions or arthritic joints between the active drug and placebo arms. Women and men were analyzed separately., Results: Eighty-four patients (72%; 45 male, 39 female) completed the 24-month study. Kaplan-Meier analyses showed significantly more complete responses in the colchicine treatment group in terms of reduced occurrence of genital ulcers (P = 0.004), erythema nodosum (P = 0.004), and arthritis (P = 0.033) among the women, and reduced occurrence of arthritis (P = 0.012) among the men. The mean numbers of genital ulcers (P = 0.001), erythema nodosum lesions (P = 0.002), and arthritic joints (P = 0.014) among the women were less in the colchicine group, and the mean number of arthritic joints (P = 0.026) among the men was less in the colchicine group. Adverse effects were similar in both groups., Conclusion: Colchicine may be useful for treating some of the manifestations of Behçet's syndrome, especially among women. This might be a reflection of less severe disease among the women.

Objective: To assess the effect of azathioprine (AZA) treatment on long-term prognosis in Behçet's syndrome., Methods: Patients (all male) who took part in a double-blind, placebo-controlled trial of AZA a mean +/- SD of 94 +/- 10 months previously were reevaluated., Results: The emergence of blindness (log rank chi2 = 5.6, P = 0.02) and a 2-line drop in the visual acuity of the right eye (log rank chi2 = 5.9, P = 0.015) occurred significantly more frequently among the patients originally allocated to the placebo group compared with patients who originally received AZA, despite posttrial treatment for patients in both groups when needed. There was also a trend toward more frequent occurrence of extraocular complications in the placebo group. The beneficial effect of AZA was especially pronounced among patients who had eye involvement of short duration prior to their entry into the trial., Conclusion: Early treatment with AZA tends to favorably affect the long-term prognosis of Behçet's syndrome.