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PubMed1982

The relationship of HLA-B and DR phenotypes to Behcet's syndrome, recurrent oral ulceration and the class of immune complexes.

A series of eighty Caucasian patients was divided into four groups with the mucocutaneous, arthritic, neurological and ocular types of Behcet's syndrome (BS) and a fifth group of patients with recurrent oral ulcers. The immunogenetic basis of the four types of BS was extended from HLA-B locus to the DR locus. Whereas B5 and more precisely the Bw51 split is the most discriminating marker of the ocular type of BS, DR7 also shows a significant increase in the ocular and neurological types. Indeed, most if not all patients with the ocular type have B5 and/or DR7. B12 and/or DR2 is significantly increased at the less severe end of the spectrum, the mucocutaneous and arthritic types. Patients with recurrent oral ulcers also show an increase in B12 and/or DR2. However, B12 and/or DR7 shows an increase in the relative risk in all four types of BS. These results suggest that HLA-B12, B5, DR7 and DR2 might in some way be associated with tissue localization of disease. Alternatively, since patients with HLA-B12 show a significantly greater ratio of IgG:IgA circulating immune complexes than those with B5/DR7/DR2, tissue localization might be influenced by the immune complex isotype. A significant relationship was also found between the MT2 and MT3 B cell alloantigen system and BS, with particular reference to MT2 and the neurological type and MT3 and the ocular type of BS.

Antigen-Antibody Complex↗
PubMed1981

[Immunosuppressive therapy of Behcet's syndrome. Long term follow-up evaluation (author's transl)].

18 patients suffering from Behcet's disease were treated with chlorambucil. In addition 6 patients who were threatened by acute and permanent blindness received antilymphocyte globulines. The follow-up varies from 1 to 12 years with an average of 4,5 years. Recovery from ocular inflammation was total in 14 patients, and partial in 4 patients. 6 eyes underwent an uneventful cataract extraction. None of the 8 one-eyed patients became blind. The mean improvement of visual acuity at the end of the follow-up period was 0,4. However recurrences occurred in 3 patients when the treatment was stopped. In this series blood cell tolerance was the major problems encountered. The results achieved in this series lead to confirm that chlorambucil and A.L.G. have dramatically improved the ocular prognosis of Behcet's disease. However chlorambucil must not be considered to be the ultimate answer to the treatment of Behcet's disease since recurrences occur when the treatment has been stopped. In addition to this, long term treatments with chlorambucil involve a high risk of severe blood complications.

Adolescent↗
PubMed1977

[Behcet's syndrome with neurologic involvement. Report of a case in a pregnant women].

A case of Behcet's disease in a pregnant patient is reported. The patient showed central nervous system involvement as well eyes, mouth, external genitalia and skin lesions. The neurological signs were cranial nerves palsies and pyramidal tract involvement. The cerebrospinal fluid showed hypercytosis and hyper-gammaglobulin. The patient was treated with sistemic dexamethasone and eye drops of atropine and fluorometolone. The delivery was by elective cesarean section at 8th and a half month of pregnancy. In the examination of 6 month-old the infant was normal. The patient improved but remained with important visual loss. The authors think that the pregnancy helped the partial recovery and the good evolution; similar cases have been reported in the literature. A rapid review about the central nervous system involvement and treatment of Behçet's disease is made.

Behcet Syndrome↗