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PubMed1986

[Behçet syndrome].

Behçet's syndrome is described on the basis of historical facts taken exclusively from original papers, and considering especially those symptoms which are usually neglected. The differentiation of certain symptom-oriented subforms such as angio-Behçet, arthro-Behçet, entero-Behçet, neuro-Behçet, pneumo-Behçet, and uro-Behçet gives a better understanding of this syndrome and also stresses the fact that many fields of medical activity are concerned with it. The historical facts show that ophthalmology and dermatology share the credit for having identified this syndrome.

Behcet Syndrome↗
PubMed2000

Differential diagnosis of fever of unknown origin in children.

Fever of unknown origin in children follows two main clinical patterns, namely fever of unknown origin and chronic episodic fever of unknown origin. Fever of unknown origin is characterized by daily fever persisting for more than 3 weeks. The main causes are infectious, rheumatologic disorders, and malignancy. Chronic episodic fever of unknown origin is characterized by fever lasting for a few days to a few weeks, followed by a fever-free interval and a sense of well-being. The main causes are familial Mediterranean fever, the hyper-immunoglobulin D syndrome, familial Hibernian fever, Behcet disease, the syndrome of periodic fever, aphthous stomatitis, pharyngitis and adenitis, and cyclic neutropenia.

Behcet Syndrome↗
PubMed1989

Immunohistology of oral lesions from patients with recurrent oral ulcers and Behçet's syndrome.

A qualitative and quantitative immunohistological investigation was performed on biopsies of oral ulcers from patients with Behcet's syndrome (BS) and those with recurrent oral ulcers (ROU). The results were compared with control oral biopsies from patients with other diseases and normal oral mucosa. The expression of HLA-DR on the cell membrane of keratinocytes was found in 13 out of 15 lesions from patients with BS and ROU, as compared with only one out of 15 controls. The relative density of HLA-DR was investigated quantitatively by microdensitometry and this confirmed that DR expression in the epithelial cells of patients with BS and ROU was significantly greater than in diseased and normal control oral tissues. A prominent mononuclear cell infiltration consisted predominantly of T lymphocytes and mature macrophages. Analysis of the CD4 and CD8 subsets of T cells failed to show significant differences between BS, ROU and control diseased tissues. Increased numbers of Langerhans cells were found in the epithelium by morphometric analysis with the CD1 monoclonal antibody in BS and ROU but an increased number was also found in lichen planus. The results suggest that the immunohistological changes in oral lesions of BS and ROU manifest an enhanced immune response in the epithelium, keratinocytes express HLA-class II antigen and increased number of Langerhans cells as well as in the lamina propria with a prominent infiltration of CD4, CD8 and macrophage-like cells. The characteristic pattern of exacerbations and remissions of oral ulceration can be interpreted by the hypothesis that an initiating microbial agent may induce a mononuclear cell infiltration, with the release of cytokines, expression of class II antigen in keratinocytes and causing ulceration, followed by down-regulation of immunity by tolerant T cells induced by the class II positive keratinocytes, leading to a remission.

Antigens, Viral↗
PubMed1998

Anticardiolipin antibodies in clinical conditions associated with a risk of thrombotic events.

Anticardiolipin antibodies (ACAs) of IgA, IgG, and IgM isotypes were measured using an enzyme-linked immunosorbent assay (ELISA) in patients with SLE, and in other groups of subjects with a higher or lower risk of developing thrombosis. IgA ACAs were present in high titers in all groups and had little discriminant value in predicting thrombotic risk. In patients without the lupus anticoagulant (LAC) with conditions in which a thrombotic tendency was a feature (primigravidae with preeclampsia or intrauterine growth retardation, patients with angina or acute myocardial infarction, those on anticoagulant therapy for apparently spontaneous thrombosis, and patients with Behcet's syndrome in whom there was a history of thrombosis) ACAs of all isotypes were present in 44/191 (23%). In patients in whom a thrombotic tendency was not a feature (normal controls, primigravidae with normal deliveries, patients with rheumatoid arthritis, and with Behcet's syndrome in whom there was no thrombotic history) 22/241 (9%) had ACAs. Although ACAs were more likely to be present in a subset of patients without systemic lupus erythematosus (SLE) and/or the LAC, their presence was a poor discriminator of increased risk of thrombosis.

Adult↗
PubMed1982

Lymphocyte studies in a patient with neurological complications of Behçet's syndrome.

We rapport here a case of Behcet's disease presenting with neurological complications who made a remarkable recovery to high dose steroids and azathioprine. Prior to initiation of steroid therapy the percentage of T lymphocytes (OKT3) was low, that of helper T lymphocytes (OKT4) was normal and suppressor T lymphocytes (OKT8) was slightly increased. The OKT4: OKT8 ratio was slightly reduced and the percentage of B lymphocytes was high. With steroid therapy there was an increase in the percentage of OKT3 and fall in the percentages of OKT4 and OKT8 and the OKT4: OKT8 ratio was maintained fairly normal. The percentage of B lymphocytes was reduced. On addition of azathioprine the percentage of OKT3 and OKT8 were substantially increased and the percentage of OKT4 was slightly increased. The net result was a significant reduction in the OKT4: OKT8 ratio. The percentage of B lymphocytes remained normal. On reviewing the absolute counts there was a paradoxical rise in OKT4 and OKT8 a week after high dose steroid therapy due to a substantial rise in the total number of lymphocytes. On reducing the dose of steroids the absolute count of OKT4 and OKT8 decreased in parallel with their percentages. A disparity was noted in the percentage of OKT3 and the sum of the percentages of OKT4 and OKT8, prior to commencement of therapy which was significantly reduced after treatment. It is likely that this disparity is due to the presence of doubly labelled cells (cortical thymocytes) released into circulation prematurely.

Azathioprine↗