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At least 19 recordsLinked to original sources
PubMed1976

Treatment of Behcet disease with chlorambucil. A follow-up report.

In Behcet disease the eyes are involved in most cases, and progression to blindness is almost the rule. When the central nervous system is involved, the prognosis for life is grave. In a preliminary report, 11 patients had the disease arrested as a result of treatment with chlorambucil. Some of these patients had long-term follow-up and are included in this report. Nine others received the same treatment, resulting in arrest of the disease in eight and pronounced improvement in one. Long-term follow-up, in some patients for more than five years after arrest of the disease, has not disclosed any recurrence. These results may appear more important if it is noted that blindness in Behcet disease occurs an average of 3.36 years after onset of eye symptoms.

Adult↗
PubMed1989

Behcet disease: long-term follow-up of three children and review of the literature.

Behcet disease is rare in children. There are only two reports of Behcet disease in childhood, describing seven patients. Three pediatric patients are described, in whom the age of onset ranged from 6 to 11 years. Aphthous stomatitis and arthritis were present in all of the patients; genital ulcers, iridocylitis, erythema nodosum, and CNS involvement were present in two patients. Other manifestations included Stevens-Johnson-like eruption, fever of unknown origin, and testicular involvement. All of the patients responded to glucocorticoids; two were also treated with colchicine and one was treated with chlorambucil. In two patients, follow-up of more than 10 years was done, with complete cure in one patient and benign course of illness in the other. Because of the rarity of the disease in childhood and the difficulty in making the diagnosis, there is not enough awareness by pediatricians concerning this disease.

Behcet Syndrome↗
PubMed1977

Neuro-Behcet disease: two cases and neuroradiologic findings.

In two cases of Behcet disease, neurologic disorders antedated the characteristic ocular or mucocutaneous lesions by 6 to 32 weeks. Neuroradiologic investigation demonstrated expanding avascular foci in the basal ganglia, probably representing areas of infarction secondary to vasculitis.

Adult↗
PubMed1985

Effectiveness of cyclosporin therapy for Behçet's disease.

Behcet's disease is a clinical entity with mouth and genital ulcers, skin lesions, and both anterior and posterior uveitis as its major criteria. It has been theorized that Behcet's disease is immune complex mediated, and is characterized by multiple attacks which often lead to severe visual handicap. Behcet's disease patients with severe ocular involvement and cytotoxic and/or systemic corticosteroid agent failures were treated with cyclosporin (CsA). CsA therapy effectively abrogated the acute phase of the ocular attack and either totally prevented, or markedly reduced, the recurrences of these attacks. Generally, patients tolerated the medication well; occasionally however, renal toxicity secondary to cyclosporin therapy prevented prescription of a maximally effective therapeutic dose of the drug. CsA levels in plasma were dose-dependent for each patient, but the dosage per kg of CsA needed to obtain a specific plasma level varied greatly from patient to patient. Circulating immune complex levels were not universally elevated during the acute ocular attack. However, increases in circulating immune complex concentrations were noted after treatment with CsA was begun and the disease became clinically inactive. These data, as well as the effectiveness of CsA, an agent with predominantly anti-T cell effects, raise a question as to whether circulating immune complexes are centrally relevant to the pathogenesis of this disorder, and if T cell mediation of this disease must be contemplated.

Adult↗
PubMed1985

Immunological studies on aphthous ulcer and erythema nodosum-like eruptions in Behcet's disease.

Patients with Behcet's disease show an intense delayed hypersensitivity (DH) reaction to a group of streptococcal bacteria. We have attempted to detect deposits of immune complexes and to analyse cytological reactions in the aphthous ulcers and erythema nodosum (EN)-like eruptions. Deposits of IgM and positive fluorescence of anti-streptococcal group D serum were found in vessel walls and sites infiltrated by inflammatory cells. Cytological analysis has revealed that the inflammatory infiltrating cells are mainly composed of activated T-cells and macrophages in association with natural killer cells. These results suggest that DH reactions with antigen-antibody mediated cytotoxicity may play an important role in causing the lesions of Behcet's disease.

Adult↗
PubMed1989

Skin hypersensitivity to streptococcal antigens and the induction of systemic symptoms by the antigens in Behçet's disease--a multicenter study. The Behcet's Disease Research Committee of Japan.

Twenty-four different antigens from 6 strains of streptococcus were prepared for skin testing and delayed hypersensitivity testing. The induction of Behçet's disease symptoms by these antigens was studied. Delayed skin reactions to whole cells and cell walls of streptococci were frequently observed in Behçet's disease, but only a few skin reactions were observed in other diseases and healthy controls. The skin reactions to the other bacteria were also strong in Behçet's disease, but the differences between Behçet's disease and the other diseases were not significant. Interestingly, the induction of systemic Behçet's disease symptoms was observed after the streptococcus skin test in 15 of 85 cases tested, but no case of induction by the other bacteria was observed. Our study supports the possible pathogenetic role of certain streptococcal antigens in Behçet's disease.

Adolescent↗
PubMed1985

Neurogenic muscular atrophy in Behcet's disease.

A child is reported with Behcet's disease who presented with skin and joint manifestations, oral ulcers, brainstem syndrome and neurogenic muscular atrophy. The neurogenic muscular atrophy was confirmed by electrophysiologic, histologic and histochemical studies. Electron microscopy of muscle showed a vasculopathy. The possible etiology of the muscle lesions is discussed. The relevant literature on muscle and peripheral nerve involvement in Behcet's disease is reviewed. It is proposed that neuromyopathy be added to the neurological manifestations of Behcet's disease. To our knowledge, this is the first case of neurogenic atrophy reported in Behcet's disease.

Behcet Syndrome↗