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Ulcerative esophagitis and colitis in a pediatric pantient with Behcet's syndrome. Response to steroid therapy.

The gastrointestinal manifestations of Behcet's syndrome including colitis and esophageal ulceration have been recognized in recent years. In the present case, a girl with Behcet's syndrome was followed since age 11 for oral, vaginal, perianal ulcers and neurological involvement. At age 16 she developed esophageal ulceration and colitis, was treated with steroids and improved. Endoscopic and radiographic findings are presented, previously reported cases are reviewed and treatment is discussed.

Adolescent↗

Caeruloplasmin concentration and oxidase activity in polyarthritis.

Caeruloplasmin (Cp) concentration and oxidase activity have been shown to be elevated in rheumatoid arthritis (RA) and psoriatic arthritis, but normal in Reiter's syndrome, Behcet's syndrome and juvenile seronegative polyarthritis. Synovial fluid Cp was significantly depressed in comparison with serum Cp in RA. During second-line therapy in RA, Cp concentration and activity fell significantly (P less than 0.001), but the change in Cp did not correlate with plasma viscosity.

Acute-Phase Reaction↗

Budd-Chiari syndrome and renal failure in Behcet disease. Report of a case and review of the literature.

A case of Behcet syndrome associated with three rare complications is presented. The patient initially presented with asymmetric polyarticular arthritis, subcutaneous nodules, conjunctivitis, and episcleritis. Necrotizing vasculitis and renal failure subsequently evolved. Despite immunosuppressive therapy, Budd-Chiari syndrome with high grade hepatic obstruction developed. A Denver shunt procedure failed to alter the terminal course. At postmortem examination, skin lesions, carditis, hepatic vein thrombosis, gastric ulcerations, and focal proliferative glomerulonephritis were found. The immunoglobulins and complement present in skin and renal tissue suggest an immune complex pathogenesis.

Adult↗

Behcet's syndrome.

Explore the source record for details and available documents.

Behcet Syndrome↗

Obstructive lung disease in Behcet's syndrome.

Obstructive lung disease in two patients with Behcet's syndrome is presented. In both cases increased airways resistance, impaired PEF and FEV1.0 were found. In addition, diffusion capacity was slightly decreased in spite of enlarged total lung capacity and increased residual volumes. Both patients had mild hypoxaemia despite slight hyperventilation; a normal chest X-ray, and a decreased serum complement C3 level. Corticosteroids had a beneficial effect on VC in one of the two cases but failed to have any significant effect on the impaired ventilatory function in the other.

Adrenal Cortex Hormones↗

Arthritis in Behcet's syndrome.

The clinical and radiological features of fourteen patients with Behcet's syndrome have been reviewed with particular reference to joint disease. Thirteen patients (93%) suffered recurrent peripheral oligo- or poly-arthritis, this symptom being the presenting complaint in two (14%). There were no radiographic abnormalities in peripheral joints. Ten patients (71%) complained of low back pain and in seven (50%) there was a mild erosive sacro-iliitis. Five patients (36%) had an enthesopathy (calcaneal spurs) one of whom also demonstrated vertebral body squaring. Patients with sacro-iliitis did not possess the HLA B27 antigen. Behcet's syndrome should be entertained in the radiological differentiation of sacro-iliitis with a clinical arthropathy.

Adolescent↗

Incomplete Behcet's syndrome in the differential diagnosis of genital ulceration and postcoital bleeding. A case report.

Behcet's syndrome occasionally produces significant vaginal bleeding requiring operative intervention. Our patient had genital and oral ulcerations but did not have uveitis or iritis, therefore representing incomplete Behcet's syndrome. The oral and genital lesions associated with Behcet's syndrome resemble those produced by herpes infection and must be considered in the differential diagnosis of genital ulceration.

Adult↗

Synovial pathology in Behcet's syndrome.

Eight specimens of synovial membrane from 6 patients with 'definite' Behcet's syndrome were available for histological examination. Only the superficial zones of the synovia were affected, all except one being replaced by dense inflamed granulation tissue composed of lymphocytes mingled with macrophages, vascular elements, fibroblasts, and neutrophils. There was a marked plasma cell infiltrate and lymphoid follicle formation in one synovium only, and there was no evidence of infection. Pannus and erosive change were present in the three specimens which included the articular surface, the erosive change being visualised radiologically in two of these. It is suggested that these appearances are characteristic of Behcet's syndrome and should be added to the list of diagnostic criteria.

Adult↗

Acute phase proteins and C9 in patients with Behcet's syndrome and aphthous ulcers.

Estimation of the concentration of C9, C-reactive protein (CRP) and alpha1-antitrypsin in forty sera from patients with Behcet's syndrome and recurrent oral ulcers showed significantly increased amounts of C9 and CRP in Behcet's syndrome. The concentration of C9 was also significantly raised in recurrent oral ulceration, though to a lesser extent than in Behcet's syndrome. The assay C9 and CRP might be useful in the differential diagnosis of Behcet's syndrome, especially from recurrent oral ulcers. It is suggested that during epithelial inflammation in recurrent oral ulcers some of the acute phase proteins are increased and in some patients these may modulate the immunological mechanism in such a way as to induce a transition from focal oral ulceration to the multifocal Behcet's syndrome.

Behcet Syndrome↗

Behcet's syndrome: an unusual cause of colonic ulceration and perforation.

Behcet's syndrome is a multisystem disease which may produce not only the original triad of relapsing iridocyclitis and recurrent oral and genital ulceration but also skin, central nervous system, joint and gastrointestinal disease. A fatal outcome is uncommon but may occur when the central nervous system or the gastrointestinal tract is involved. We present a patient with colonic ulceration progressing rapidly to free perforation and generalized peritonitis successfully treated by emergency resection.

Behcet Syndrome↗

Pharmacotherapy for Behcet's syndrome.

OBJECTIVES: To determine the effects of available pharmacological interventions in treating the different clinical features of Behcet's syndrome. SEARCH STRATEGY: We searched the Cochrane Musculoskeletal Group's trials register, the Cochrane Controlled Trials Register, and Medline up to January 1998. The computer search was complemented by a hand search of all bibliographic references from the reference lists of included trials. Principal investigators were contacted to seek unpublished literature. All languages were included. SELECTION CRITERIA: Studies were eligible if they fulfilled all of the four following criteria: 1. Randomized controlled trials, single or double-blind; 2. Participants were patients with Behcet's Syndrome as defined by the International Study Group, 1990 (Int Study Group, 1990); 3. Interventions included any pharmacological therapy compared to placebo or some other pharmacological intervention for the treatment of Behcet's syndrome. 4. Outcome measures included active ocular inflammatory processes, arthritis, mucocutaneous manifestations (oral ulcer, genital ulcer, erythema nodosum), laboratory changes and major events such as adverse effects and death. DATA COLLECTION AND ANALYSIS: The 32 potentially relevant references were assessed by two independent reviewers (MA, AS) according to the inclusion criteria. Ten trials fit the inclusion criteria and were included in this review. From the 10 included trials, data were independently extracted by the same two observers and crosschecked. The quality of the included trials was assessed independently by two observers (MA, AS) using a validated scale (Jadad 1996). For dichotomous measures, the treatment effect for each trial was calculated using a fixed effect model [Peto model (Petitti 1994)]. The weighted mean differences were based, if available, on end-of-trial results. The analysis was conducted separately for each different intervention. Since the trials could not be pooled it was not possible to carry out a sensitivity analysis by quality scores or a subgroup analysis by drug dosages. Because of this lack of comparability across trials and the small number of trials, we could not conduct a heterogeneity test or a funnel plot. MAIN RESULTS: Ten trials and 679 patients were included. The main results were the lack of efficacy of some of the classic treatments for Behcet's syndrome, including colchicine, cyclophosphamide and steroids for eye involvement, azapropazone and colchicine for arthritis and acyclovir, colchicine and topical interpheron for aphthas. The results confirm the protective effects of cyclosporine and azathioprine for eye involvement and benzathine-penicillin for arthritis. REVIEWER'S CONCLUSIONS: We conclude that further randomized, placebo-controlled, double-blind trials should be carried out to compare cyclosporine, azathioprine and benzathine-penicillin versus placebo in order to make the results generalizable and comparable.

Acyclovir↗

Damaged membrane fragments and immune complexes in the blood of patients with Behcet's syndrome.

Electron microscopic examination of centrifuged pellets of serum from patients with Behcet's syndrome and recurrent oral ulcers revealed the presence of a large number of membrane fragments. Some of these membranes showed numerous 10 nm holes that were identical to lesions produced by the action of complement. An attempt was made to correlate complement levels, antibodies and cellular immunity with the presence of the membrane fragments, without success. However, a significant correlation was found between the membranes and the IgG class of immune complexes. The finding of membrane fragments with complement-induced damage predominantly in the blood of patients with Behcet's syndrome, and the association with soluble immune complexes suggest that the latter may generate C5b-9 complexes which may bind to the surface of cells in result in cell lysis.

Adult↗