Fatal thoracic aortic aneurysm in a patient with childhood-onset vasculo-Behcet's disease: an autopsy report. (73/725)

A 33-year-old man died following sudden unexpected rupture of a saccular thoracic aortic aneurysm. The patient had been treated as suspicious Behcet's disease (BD) for 9 years, however, the medication was discontinued at the age of 24. Autopsy disclosed multiple aneurysms at the descending thoracic and abdominal aorta. Histopathologically, infiltration of inflammatory cells and destruction with loss of elastic and muscle fibers of the aortic medial wall were seen. Taking the clinical course into account, all the postmortem findings were compatible with those of vasculo-BD. It is important that medical follow-up should be implemented even for childhood-onset patients who do not satisfy criteria of BD.  (+info)

Association of the MIC-A gene and HLA-B51 with Behcet's disease in Arabs and non-Ashkenazi Jews in Israel. (74/725)

BACKGROUND: Behcet's disease is known to be strongly associated with HLA-B51 in many different ethnic groups. Recently, it was suggested that MIC-A (major histocompatibility complex class I related gene A) is the pathogenic gene after strong association was found between the MIC-A A6 allele of the transmembrane region and the disease in Japanese and Greek patients, although in Greek patients this association was found to be due to linkage disequilibrium with HLA-B51. OBJECTIVES: To investigate microsatellite polymorphism in Arab and non-Ashkenazi Jewish (NAJ) patients in Israel, to determine whether this association occurs in these groups with Behcet's disease, and elucidate the associated HLA allele of the disease. METHODS: Forty four Israeli patients with Behcet's disease, including 20 Arabs and 24 NAJ, and 130 ethnically matched healthy controls were examined for MIC-A microsatellite polymorphism of the transmembrane region using polymerase chain reaction, autoradiography, and sequence analysis. RESULTS: The MIC-A A6 allele was significantly more frequent in the Arab patient group (19/20 (95%)) than in healthy Arab controls (25/42 (60%)) (p(corr)=0.015, OR=12.92), but not in the NAJ patients (16/24 (67%)) compared with NAJ healthy controls (48 /88 (55%)) (p(corr)=1.02, OR=1.667). In stratification analysis of the Arab subgroup, on the confounding effect of MIC-A A6 on HLA-B51 association and vice versa, Behcet's disease was distinctly associated only with HLA-B51. CONCLUSIONS: These results imply strong association between the MIC-A A6 allele and the disease in Israeli Arabs, but not in Israeli NAJ patients. The stratification analysis indicates that this association results secondarily from a strong linkage disequilibrium with HLA-B51, and the real disease susceptibility gene which plays a part in the development of Behcet's disease is most probably the HLA-B51 allele itself.  (+info)

Combination therapy including pentoxifylline for entero-Behcet's disease. (75/725)

Behcet's disease is a chronic relapsing vasculitis characterized by recurrent aphthous oral and genital ulcerations with uveitis. Multiple organs can be involved. Entero-Behcet's disease is often uncontrollable, relapsing, and can cause acute intestinal bleeding or perforation. We utilized a combination therapy including 600 mg of pentoxifylline per day, in two doses, to treat three female patients and observed the subsequent changes in clinical symptoms, serum C reactive protein levels, and endoscopic findings. In all three patients, clinical symptoms as well as serum C reactive protein levels improved immediately. Endoscopically, lower intestinal lesions were significantly reduced or healed in all of them. Combination therapy including pentoxifylline appears to be clinically effective in the patients with entero-Behcet's disease.  (+info)

The incidence and pathology of conjunctival ulceration in Behcet's syndrome. (76/725)

AIMS: To describe the results of a study of the incidence of conjunctival ulceration and its pathology as a manifestation of Behcet's syndrome. METHODS: The authors reviewed retrospectively medical charts of 152 patients with Behcet's syndrome seen at the uveitis service of Okayama University Hospital from January 1995 to December 1999. Criteria used to diagnose Behcet's syndrome in this study required the presence of oral aphthous ulceration plus any two of three other manifestations-genital ulceration, eye lesions such as iridocyclitis and uveoretinitis, or skin lesions such as erythema nodosum. The presence of conjunctival ulceration was determined in patients with Behcet's syndrome through standardised ophthalmological examinations. A biopsy of the conjunctival ulceration was performed in one patient. The incidence of conjunctival ulceration in patients with Behcet's syndrome was calculated and the clinical and pathological features of this ulceration were examined. RESULTS: Four of 152 patients (2.6%) developed conjunctival ulceration in concordance with exacerbation of their systemic symptoms. Histopathological examinations of a conjunctival biopsy specimen in one patient revealed intraepithelial and perivascular infiltration with neutrophils and lymphocytes, consistent with Behcet's syndrome. The conjunctival ulcerations of the study patients showed good response to topical corticosteroids or topical steroids plus oral colchicine. CONCLUSIONS: Conjunctival ulceration should be noted as an uncommon but possible manifestation of Behcet's syndrome. Accordingly, routine examination of the conjunctiva is recommended in patients with Behcet's syndrome, and Behcet's syndrome should be included in a list of differential diagnoses for patients with conjunctival ulcerations.  (+info)

Prevalence of Behcet's disease in an Arab community in Israel. (77/725)

OBJECTIVE: To evaluate the prevalence of Behcet's disease (BD) in an Israeli Arab town (Taibe). METHODS: Questionnaires about the occurrence and prevalence of aphthous ulcers were distributed randomly to the parents of children attending a paediatric centre in Taibe. The parents were asked whether they or any of their children aged between 10 and 20 years had recurrent aphthous stomatitis. Any who had had more than four aphthous episodes (each episode lasting more than seven days) during the previous year were invited for an extensive interview and examination by a rheumatologist or a paediatrician. RESULTS: A total of 4876 subjects were included in this survey, of whom six (one male, five female) were diagnosed as having BD. Of these six, two were siblings (a brother and a sister). Five had skin lesions, four had visual involvement, and all had genital ulcers and joint symptoms; one in two patients had a positive pathergy test. Five of the six carried HLA-B5 antigens. The results showed a prevalence of 12/10,000 in Taibe. CONCLUSION: The prevalence of BD found in our survey is high and concurs with that found in other Mediterranean and Asian countries.  (+info)

Cryoglobulins in Behcet's syndrome and recurrent oral ulceration: assay by laser nephelometry. (78/725)

The presence of cryoglobulins was investigated in ninety patients with recurrent oral ulcers (ROU) and sixty-one patients with Behcet's syndrome (BS). The immunodiffusion method was compared with Laser nephelometry for the analysis of IgG, IgM, IgA and C3 in cryoglobulins. Although the two methods of assessment showed a very significant agreement. Laser nephelometry was more sensitive than the double diffusion precipitation method and was used for quantitative analysis of cryoglobulins. The prevalence of any type of cryoglobulins was 64% in ROU and 75% in BS, as compared with controls (15%). In ROU significant levels of IgA were found in minor (P = 0.0196) and major (P = 0.0114) aphthous ulcers and to a lesser extent in herpetiform ulcers (P = 0.0624). Among the four types of BS signficant increases in C3 were found in the arthritic type (P = 0.0068) and ocular type (P = 0.0275), whereas IgM (P = 0.0031) and IgG (P = 0.0369) were increased only in the muco-cutaneous type. Sequential studies showed that disease remissions or exacerbations were correlated with a decrease or increase in IgM or IgG classes of cryoglobulins. However, the converse was found with IgA which may inhibit some functions of polymorphonuclear leucocytes, and this may be responsible for the failure to remove damaging IgG, IgM and C3 complexes from the circulation.  (+info)

Humoral and cell mediated immune response to cow's milk proteins in Behcet's disease. (79/725)

OBJECTIVE: To investigate the humoral and cellular immune response against cow's milk proteins in Behcet's disease and to distinguish any behaviour during active or inactive disease. METHODS: Peripheral blood mononuclear cells from 16 patients and from eight normal controls were cultured in the presence of phytohaemagglutinin (PHA), beta-casein, beta-lactoglobulin, or chicken egg albumin. Interferon gamma (IFNgamma) and interleukin 4 (IL4) were measured in the culture supernatants by enzyme linked immunosorbent assay (ELISA). Serum samples from 46 patients with Behcet's disease and from 37 healthy subjects were also studied for antibody detection. Antibodies to beta-casein, beta-lactoglobulin, and chicken egg albumin were determined by ELISA. RESULTS: High IFNgamma but not IL4 levels were found in the supernatants of lymphocytes from patients with active disease cultured in the presence of cow's milk proteins. Levels were comparable with those obtained in cultures stimulated with PHA. A significantly higher level of anti-beta-casein and anti-beta-lactoglobulin IgG and IgA antibodies was found in patients with active Behcet's disease. No relation was found between their occurrence and the age of the patients, the duration of disease, or the presence of gastrointestinal abnormalities. Antibodies to chicken albumin were detected at low levels and with a prevalence similar to that of healthy subjects. CONCLUSION: The results indicate that an active immune response occurs in Behcet's disease. This response involves an increased frequency of antibodies to cow's milk protein and a strong Th1 polarisation after exposure to these antigens. The occurrence of these abnormalities supports a putative role for cow's milk proteins immune response in the pathogenesis of Behcet's disease.  (+info)

Multiple pulmonary arterial aneurysms in Behcet's disease: clinical and radiologic remission after cyclophosphamide and corticosteroid therapy. (80/725)

Behcet's disease (BD) with pulmonary arterial aneurysm is rare and often associated with a poor prognosis. But there is also a chance that the aneurysm may completely resolve with medical therapy. A 39-year-old man presented with fever, malaise, bilateral chest pain, recurrent oral ulcers, and hemoptysis. The chest radiograph showed a round opacity in the right hilum. Computed tomography and magnetic resonance angiography (MRA) further revealed multiple, bilateral pulmonary arterial aneurysms consistent with the diagnosis of BD. The patient was started on a course of cyclophosphamide and corticosteroid therapy that resulted in cessation of his symptoms and complete resolution of radiologic findings. The chest radiograph and MRA reverted to normal on long-term follow-up. He is still alive and symptom-free 20 months after diagnosis.  (+info)