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S Yurdakul

Publications and source records attributed to S Yurdakul.

At least 37 records · Page 2Linked to original sources

The management of Behçet's syndrome.

Behçet's syndrome (BS), originally described as a triad of oral aphthae, genital ulcerations and uveitis, is a systemic vasculitis that is prevalent in the Middle east, Far East and in the Mediterranean basin. It is characterized by a heightened state of inflammation although the main drive that initiates and sustains this is not yet elucidated. Suppression of this inflammatory state constitutes the major goal of treatment and therapy is tailored according to the specific manifestations observed. We now have considerable more insight on drug management of BS compared to 20 years ago. Particularly, within the recent past we have learned to use more rationally the agents that were already available to us. This is especially true for azathioprine, cyclosporin A, thalidomide and colchicine. Promising data are also being collected with alpha-interferon. With these agents, significant progress has been achieved in the management of uveitis and mucocutaneous symptoms but treatment issues related to thrombotic problems, major vessel involvement and neurological disease have not yet been resolved.

Behcet Syndrome↗

Thalidomide in the treatment of the mucocutaneous lesions of the Behçet syndrome. A randomized, double-blind, placebo-controlled trial.

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.

Adolescent↗

Clinical features of Behçet's disease in children: an international collaborative study of 86 cases.

OBJECTIVES: The objective of this study was to characterize the clinical picture of Behçet's disease (BD) in children. STUDY DESIGN: A questionnaire was completed by five BD specialists from Turkey, France, Iran, or Saudi Arabia. We first reviewed 86 cases retrospectively with a specially designed computerized database and then selected 65 who met the criteria of the International Study Group for BD, which include buccal aphthosis plus at least two among recurrent genital aphthosis, eye lesions, skin lesions, and positive pathergy test. The remaining 21 patients, who had features suggestive of BD but did not fulfill the international criteria, were analyzed separately and then compared with the other 65 patients. RESULTS: BD affected boys and girls equally. The clinical picture frequently included mucocutaneous lesions. Uveitis was less frequent than in adults but carried a poor prognosis, especially in male patients (p < 0.001). The mortality rate (3%) was related to large vessel involvement. Familial cases were particularly frequent (15%). Erythema nodosum and skin hypersensitivity were common in Turkish patients, whereas neuro-BD was more frequent in French and Saudi Arabian patients. Patients who did not fulfill the international criteria had significantly less genital aphthosis (p < 0.01), less skin lesions or hypersensitivity (p < 0.01), and less uveitis (p < 0.01). CONCLUSION: BD in children is similar to BD in adults. The high frequency of familial cases calls for further investigation of the immunogenetic factors that may favor early expression of the disease.

Adolescent↗

Wound healing in Behçet's syndrome.

BACKGROUND: Because there is an increased inflammatory response to trauma, particularly of the skin, in patients with Behçet's syndrome (BS), an alteration in wound healing in BS is expected. The aim of this study was to investigate the healing features of punch biopsy wounds in BS and acne vulgaris (AV) patients used as controls. METHOD: Full-thickness skin punch biopsies (4 mm) were taken from the hairless sites of the non-dominant forearms in 20 BS and 20 AV patients. Each patient was examined on days 1, 2, 3, 4, 5, 8, and 10, and the biopsy wound area and induration were marked on sterile glass slides. Other inflammatory changes, such as suppuration and pain, were also recorded. No antiseptic solutions or ointment, except saline, were used. RESULTS: The wound area healed similarly in both groups (p > 0.05). Thirteen (65%) BS patients had erythematous haloes around the wound on the first day. The number of patients presenting inflammatory changes reached 18 (90%) on the second day in the BS group. The area of erythema around the wound in BS patients was significantly greater than that in AV patients (5; 25%). It gradually decreased day by day. Purulent changes were observed in four BS patients on day 1, and induration around the biopsy wound in six patients on day 2. Only one patient with AV had suppuration, which appeared on the second day, while no AV patient displayed induration. CONCLUSION: Biopsy-induced trauma may cause increased inflammation in BS, but wound healing is not altered.

Acne Vulgaris↗

Genetic anticipation in Behçet's syndrome.

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.

Adolescent↗

A case of rapidly progressive pulmonary aneurysm as a rare complication of Behçet's syndrome--a case report.

One of the severe complications of Behçet's syndrome is pulmonary arterial involvement. The authors report a patient with Behçet's syndrome who had the complications of multiple pulmonary arterial aneurysms presenting with hemoptysis and right ventricular thrombus. He underwent emergency surgery because of failure of intensive immunotherapy and died 2 months after the surgery.

Adult↗

Fibromyalgia in Behçet's syndrome.

OBJECTIVE: To ascertain the frequency of fibromyalgia (FM) in Behçet's syndrome (BS) and to evaluate the relationship of FM to Behçet's disease activity. METHOD: Self-questionnaires were completed by 108 patients with BS. Each patient was evaluated by an observer blinded to diagnosis; evaluation included assessment of tender points by palpation. Another observer determined the disease activity of patients at that time. RESULTS: Ten of 108 patients (9.2%) met the American College of Rheumatology criteria for FM. Nine of the patients who met the criteria for FM were women. In contrast to patients without FM, patients with FM had mild to moderate disease activity in which musculoskeletal complaints were common. CONCLUSION: There is a trend for an increased frequency of FM in female BS patients.

Adult↗

Azathioprine in Behcet's syndrome: effects on long-term prognosis.

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.

Adult↗

Management of aneurysms in Behçet's syndrome: an analysis of 24 patients.

BACKGROUND: The surgical therapy of Behçet aneurysms is often unsuccessful, resulting in graft occlusions, anastomoses, and/or new aneurysms. METHODS: Twenty-nine aneurysms were documented in 24 Behçet's patients during a period of 19 years. All patients were male, ranging in age from 20 to 53 years (mean, 35 +/- 7.3 years). The mean duration of disease was 9 +/- 5 years. There were nine abdominal aorta, four iliac, three common femoral, five superficial femoral, four popliteal, one subclavian, one carotid, and one posterior tibial artery aneurysm. In addition, in one patient an aneurysm developed from the arterialized venous conduit that had been inserted for a common femoral artery aneurysm elsewhere. Five patients were already under immunosuppressive therapy for ocular problems at the time of diagnosis. Fifteen patients received immunosuppressive therapy after operation. We performed one abdominal aneurysmorrhaphy, two iliac artery PTFE graft interpositions, two aortobiliac bypasses (PTFE), six aortic tube graft (three PTFE, three Dacron) interpositions, one avrtofemoral bypass (PTFE), two iliofemoral bypasses (PTFE), two superficial femoral artery graft (PTFE) interpositions, and three popliteal graft interpositions (one PTFE, two vein graft). Also as an initial procedure one carotid, one subclavian, four superficial femoral, one popliteal, and one posterior tibial artery were ligated. RESULTS: Nineteen patients were followed up for a mean duration of 47.3 +/- 27 months (range, 1 to 108 months). The patient with a subclavian aneurysm died of massive bleeding on postoperative day 15. Four patients were lost to follow-up. In the abdominal aortic aneurysm group one patient died of gastrointestinal bleeding 4 years after the operation. Another patient from the same group died 5 years after operation without any vascular disease. In the common femoral artery group the patient with an occluded iliofemoral graft died of an exsanguinating pulmonary artery aneurysm in the first year after operation. Overall, there were five anastomotic aneurysms. In addition, after the initial operation two iliofemoral, one aortofemoral, and one popliteal interposition graft were occluded without disabling ischemia. CONCLUSIONS: Aneurysms limited to the extremities could be ligated without disabling ischemia. Abdominal aortic aneurysms could be treated with tube graft insertion, giving satisfactory results. Patients could tolerate graft occlusion without major ischemia.

Adult↗

Small vessel vasculitis and vasculitis confined to skin.

Cutaneous vasculitis is a heterogeneous group of disorders, which can be confined to the skin or may be part of an associated systemic disease. Various aetiological agents as well as conditions that mimic skin vasculitis, usually present with similar clinical features; mainly palpable purpura. The skin biopsies usually show leukocytoclastic vasculitis. This poses a great diagnositc and therapeutic challenge for the physician. The aetiologies, clinical features, diagnosis and treatment modalities for each form (drugs, infections, malignancies, systemic vasculitides, connective tissue disorders. Schönlein-Henoch purpura, cryoglobulinaemia, cutaneous periarteritis nodosa, livedoid vasculitis, erythema elevatum diutinum and urticarial vasculitis) are reviewed.

Humans↗

Seven-year follow-up of neurologic involvement in Behçet syndrome.

OBJECTIVE: To determine the long-term prognosis of neurologic involvement in Behçet syndrome. DESIGN: Forty-six patients with Behçet syndrome, who had been the subjects of a previous report with short-term follow-up, were reexamined 7 years later; 42 of them could be reexamined neurologically. Neuropsychological testing, magnetic resonance imaging, electromyography, and evoked potential studies were performed when available. RESULTS: Of the 27 patients who had had headaches without any neurologic symptoms or signs previously, 2 had developed an acute neurologic attack. In addition, 7 patients in this group showed minor abnormalities on neurologic examination and/or other laboratory investigations, without history of any attacks. Among the previous neuro-Behçet group (n = 15), as defined by the presence of neurologic signs or symptoms, other than headache, 7 had a stationary course, while 8 had been progressive. Three of the latter group had died. Patients with progressive course had had abnormal cerebrospinal fluid findings at the time of the previous report, whereas patients with a stationary course had not. CONCLUSIONS: Silent neurologic involvement may occur in Behçet syndrome. Patients should undergo periodic neurologic evaluation. The long-term prognosis in neuro-Behçet syndrome does not seem to be as favorable as we observed in short-term follow-up. Cerebrospinal fluid findings may predict prognosis.

Adult↗

A controlled study of reliability and validity of MRI findings in neuro-Behçet's disease.

Our aim was to test the reliability of interpreting MRI studies in neuro-Behçet's disease (NBD) and to determine the sensitivity and specificity of different MRI findings. We prospectively studied 50 patients: 24 had chronic NBD, 12 multiple sclerosis, 5 vasculitis other than Behçet's disease (BD) and 9 patients had BD without neurological involvement. MRI studies were performed according to a standard protocol with a 0.2 T imager. Two neuroradiologists, blinded to the diagnosis, age and sex of the subjects, reviewed the films independently, twice. Separate assessments were made for a set of items: dural sinus pathology, widening of ventricles and sulci, brain stem atrophy, lesions of the cerebral cortex, discrete lesions of deep white matter, basal ganglia, brain stem and cerebellum and the presence of smooth periventricular high-signal foci. Intraobserver agreement was substantial or better, and interobserver agreement moderate to substantial for most items. In these patients with chronic NBD we found low sensitivity on all assessed items. Dural sinus pathology or brain stem atrophy were highly specific, but parenchymal lesions in different sites had uniformly low specificity.

Behcet Syndrome↗

Calciphylaxis: a condition mimicking necrotizing vasculitis.

A patient with end stage renal disease developed ischaemic skin necrosis and digital gangrene. He had diffuse arterial calcification associated with hyperparathyroidism secondary to renal failure. The patient received inappropriate cyclophosphamide therapy as he had been misdiagnosed as having an inflammatory vasculitis. This clinical picture, previously named "calciphylaxis" should come into the differential diagnosis of systemic vasculitis in a uraemic patient with hyperparathyroidism.

Arterioles↗

Surgical therapy of pulmonary arterial aneurysms in Behçet's syndrome.

Two patients with Behçet's syndrome were operated for exsanguinating pulmonary artery aneurysm into a bronchus. Lower lobectomy was performed in the first patient. He died of hypoxia and sepsis on the 8th postoperative day. Pneumonectomy was undertaken in the second patient, who is well 30 months after the operation.

Adult↗

The ten-year mortality in Behçet's syndrome.

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 mortality in the general population. Information on mortality was available in 79% of the study group, among whom six patients (all males) 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

Adolescent↗