Cases reported "Behcet Syndrome"

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1/17. Clonally accumulating T cells in the anterior chamber of Behcet disease.

    PURPOSE: To clarify the nature of infiltrating T cells into the anterior chamber of a patient with Behcet disease. methods: aqueous humor was obtained from a patient with ocular Behcet disease by paracentesis. rna isolated from the cells in aqueous humor was reverse transcribed into complementary dna. Complementary dna encoding the variable (V) diversity (D) joining (J) (junctional) region of T-cell receptor beta chain V domain (TCR BV) chain was amplified by T-cell receptor BV family polymerase chain reaction. RESULTS: polymerase chain reaction Southern blot analysis showed that T-cell receptor BV3, BV5, and BV7 were dominantly expressed on ocular T cells of this patient. In addition, dna sequencing revealed that monoclonal or oligoclonal T-cell accumulation was found in T-cell receptor BV3( ), BV5( ), and BV7( ) T cells. CONCLUSION: These findings suggest that some T cells infiltrating into the anterior chamber of a patient with ocular Behcet disease expand by antigen-driven stimulation, indicating the pivotal role of T cells in the pathogenesis of ocular Behcet disease.
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2/17. The comparison between Behcet's disease and spondyloarthritides: does Behcet's disease belong to the spondyloarthropathy complex?

    This study was to clarify whether Behcet's disease (BD) could be classified into the spondyloarthropathy (SpA) complex. It was undertaken on 58 patients with BD (BD group), 56 patients with SpA (SpA group), and 3 patients who concurrently satisfied the criteria for BD and SpA (BDSpA group). The clinical parameters and known susceptible hla antigens were compared between BD group and SpA group. In addition, 3 patients in BDSpA group were reviewed. The prevalence of definitive sacroiliitis (SI) in BD group and SpA group was 46.4% and 5.2%, respectively. However, none had a definitive SI in healthy controls. Enthesitis was observed in 3.4% of BD group and in 50% of SpA group. The patterns of eye involvement were different between these two groups. HLA-B27 was negative in all 49 patients of BD group, whereas it was positive in 67.9% of SpA group. The prevalence of HLA-B51 was 51.7% in BD group, and that in SpA group was 21.4%. One patient in BDSpA group was considered to have concurrent BD and ankylosing spondylitis (AS). Another patient was closer to AS, and the third to BD. Conclusively, it seems that BD could not be classified into the SpA complex.
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3/17. Cytotoxic T-cell lymphoma arising in Behcet disease.

    The case of a 49-year-old man with peripheral T-cell lymphoma arising in Behcet disease (BD) is reported. A diagnosis of incomplete BD was made, and the patient was treated with immunosuppressive agents for 9 months. A left perirenal mass emerged, and a computed tomography-guided needle biopsy of the tumor revealed the infiltration of small- and medium-sized lymphoma cells. The cells were positive for CD3, CD8, CD45RO, CD43, granzyme B, and T-cell intracellular antigen-1. A diagnosis of non-Hodgkin's lymphoma (diffuse medium, T-cell) was made. A left orbital mass also appeared. Standard combination chemotherapy diminished the perirenal and orbital lesions. lymphoma cell infiltration in the esophagus was detected after chemotherapy, and the patient died of massive bleeding from the gastrointestinal tract. Non-Hodgkin's lymphoma is rarely associated with BD, and only 7 cases have been reported in the literature. We have summarized the published case reports of malignant lymphoma arising in BD. To our knowledge, this case report is the first to describe cytotoxic T-cell lymphoma arising in Behcet disease.
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4/17. An unusual case of Behcet's syndrome: triggered by typhoid vaccination?

    A case of Behcet's syndrome in a 32-year-old woman occurring shortly after her third vaccination against typhoid fever is described. scleritis and pyoderma gangrenosum were unusual manifestations of BS that occurred in this case. Treatment benefit was provided by mycophenolate mofetil and etanercept. As bacterial antigens have been proposed as potential triggers for the onset of BS, it is possible that the syndrome was precipitated by typhoid vaccination in this patient.
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5/17. The immune complex pathogenesis of glomerulonephritis and pulmonary vasculitis in Behcet's disease.

    A kidney and lung biopsy were performed on a patient with active Behcet's disease with renal and pulmonary involvement. Histologic, immunohistochemical and electron microscopic studies of the kidney biopsy specimen revealed a focal segmental necrotizing glomerulonephritis characterized by the presence of numerous subendothelial and occasional intramembranous deposits containing immunoglobulin g (IgG), the third component of complement (C3), the fourth component of complement (C4) and fibrin(ogen). Histologic and immunohistochemical studies of the lung biopsy specimen showed an acute venulitis and septal capillaritis associated with the presence of identical deposits within the walls of affected vessels. Circulating immune complexes were detected in the patient's serum by Raji cell assay. The findings indicate that the glomerulonephritis and pulmonary vasculitis occasionally occurring in Behcet's disease are due to the deposition of circulating antigen-antibody complexes. In addition, they strongly suggest that the majority of the major and minor manifestations of the disease, such as uveitis, cutaneous vasculitis, synovitis and meningoencephalitis, are a result of vascular immune complex deposition.
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6/17. Behcet's disease revealed by double peripheral arterial involvement.

    We report the case of a 62-year-old man with Behcet's disease revealed by double aneurysmal involvement of the posterior tibial and popliteal arteries. The diagnosis was suggested because of repeated phlebitis, buccal aphthous ulcerations, and results of human leukocyte antigen gene typing. The different arguments leading to the diagnosis of Behcet's disease and appropriate treatment are presented.
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7/17. A case of possible Behcet's disease, preceded by recurrent knee arthropathy for 5 years, effectively treated with oral colchicine.

    We report a case of possible Behcet's disease (BD) preceded by arthropathy for 5 years. A 29-year-old man experienced right knee swelling and pain since April 1999. Thorough examinations were performed, yet a diagnosis was not established. In December 2004 he experienced recurrent aphtha, following folliculitis-like skin lesions. A human leukocyte antigen examination revealed a positive B51 allele and his illness was diagnosed as possible Behcet disease. colchicine was administered and his symptoms were ameliorated.
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8/17. Human leukocyte antigen in Sweet's syndrome and its relationship to Behcet's disease.

    A 41-year-old man with Sweet's syndrome (SS) had symptoms similar to Behcet's disease (BD). To study the relationship of the two diseases, human leukocyte antigen (HLA) typing was performed on 28 patients with SS and 49 patients with BD. Of the 28 patients with SS, seven had BD symptoms. The frequencies of both HLA-B51 and -DQw3 were significantly higher in patients with BD. However, the frequencies of the two hla antigens in the 28 patients with SS and the 21 patients with SS without BD symptoms were not significantly different from the controls. The frequency of HLA-Bw54 was significantly increased in both groups of patients with SS. Taken together, these data indicate that SS is a genetically distinct disease entity from BD, although their symptoms are similar and the incidence of SS among patients with BD is high in japan.
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9/17. Induction of Behcet's disease symptoms after dental treatment and streptococcal antigen skin test.

    Severe symptoms of Behcet's disease were induced after dental treatment in 2 patients with stable Behcet's disease. Similar symptoms were probably induced by the streptococcal antigen skin test in 4 patients. These observations suggest a possible role of the streptococcus in the pathogenesis of Behcet's disease.
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10/17. hla antigens in a family with Behcet's syndrome.

    A family is described in which 3 of 12 members suffered from Behcet's syndrome. Four members, including the three patients with Behcet's syndrome suffered from recurrent aphthous stomatitis. These four family members possessed the HLA-haplotype HLA A2, B15, Cw3, DR4. However, one relative had inherited the same HLA-haplotype without showing any symptoms of the disease. Genetic influence from the actual HLA-haplotype may interact in manifestation of the syndrome.
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