Elevated Serum Levels of Vascular Endothelial Growth Factor in Behcet's Syndrome

Hong, Ji Hyun,Kim, Hyun Sook,Kim, Hae Rim,Park, Mi Kyung,Yoon, Chong Hyeon,Lee, Sang Heon,Kim, Ho Youn,Park, Sung Hwan 대한류마티스학회 2005 대한류마티스학회지 Vol.12 No.3

OBJECTIVE: Vascular endothelial growth factor (VEGF) is a potent endothelium-specific cytokine and stimulates inflammation and angiogenesis. Vascular endothelial dysfunction is one of the characteristic features of Behcet's syndrome. We previously demonstrated the possible involvement of proinflammatory cytokines (TNF-alpha, IL-15, MIF) and chemokines (IL-8, MCP-1, MIP-1alpha) in the etiopathogenesis of Behcet's syndrome and its association with disease activity. In this study, we assesed VEGF levels in patients with Behcet's syndrome and its relation with disease activity. METHODS: Serum VEGF levels of 65 patients with Behcet's syndrome and 35 healthy control volunteers were measured by enzyme-linked immunosorbent assay. The level of MIF and IL-8 were also measured in 41 pateints. Among the 65 patients, 18 patients were in active state of disease which was classified according to clinical finding. RESULTS: The mean serum VEGF levels were higher in patients of Behcet's syndrome than control subjects (332.7 pg/mL vs. 234.4 pg/mL, p=0.004) and patients with active disease had significantly higher level of VEGF than inactive disease (377.8 pg/mL vs. 265.0 pg/mL, p<0.05). There is no difference in the mean serum level of VEGF between Behcet's syndrome with vascular complication and without vascular complication. Serum level of VEGF showed strong positive correlation with MIF (r=0.57, p=0.001). CONCLUSION: Serum VEGF levels are elevated in patients with Behcet's syndrome, particularly in active state and have positive correlation with MIF. These results suggest that the increased VEGF levels in serum of Behcet's syndrome patients may participate, like other proinflammatory cytokines and chemokines, in the pathogenesis of Behcet's syndrome.

베체트 증후군 환자에서 혈관내피성장인자(Vascular Endothelial Growth Factor, VEGF)의 상승

홍지현 ( Ji Hyun Hong ),김현숙 ( Hyun Sook Kim ),김해림 ( Hae Rim Kim ),박미경 ( Mi Kyung Park ),윤종현 ( Chong Hyeon Yoon ),이상헌 ( Sang Heon Lee ),김호연 ( Ho Youn Kim ),박성환 ( Sung Hwan Park ) 대한류마티스학회 2005 대한류마티스학회지 Vol.12 No.3

Objective: Vascular endothelial growth factor (VEGF) is a potent endothelium-specific cytokine and stimulates inflammation and angiogenesis. Vascular endothelial dysfunction is one of the characteristic features of Behcet`s syndrome. We previously demonstrated the possible involvement of proinflammatory cytokines (TNF-α, IL-15, MIF) and chemokines (IL-8, MCP-1, MIP-1α) in the etiopathogenesis of Behcet`s syndrome and its association with disease activity. In this study, we assesed VEGF levels in patients with Behcet`s syndrome and its relation with disease activity. Methods: Serum VEGF levels of 65 patients with Behcet`s syndrome and 35 healthy control volunteers were measured by enzyme-linked immunosorbent assay. The level of MIF and IL-8 were also measured in 41 pateints. Among the 65 patients, 18 patients were in active state of disease which was classified according to clinical finding. Results: The mean serum VEGF levels were higher in patients of Behcet`s syndrome than control subjects (332.7 pg/mL vs. 234.4 pg/mL, p=0.004) and patients with active disease had significantly higher level of VEGF than inactive disease (377.8 pg/mL vs. 265.0 pg/mL, p<0.05). There is no difference in the mean serum level of VEGF between Behcet`s syndrome with vascular complication and without vascular complication. Serum level of VEGF showed strong positive correlation with MIF (r=0.57, p=0.001). Conclusion: Serum VEGF levels are elevated in patients with Behcet`s syndrome, particularly in active state and have positive correlation with MIF. These results suggest that the increased VEGF levels in serum of Behcet`s syndrome patients may participate, like other proinflammatory cytokines and chemokines, in the pathogenesis of Behcet`s syndrome.

베체트 증후군 환자의 말초혈액단핵구에서 종양괴사인자(Tumor Necrosis Factor)-α의 자극에 의한 Macrophage Migration Inhibitory Factor의 생산 증가

곽승기 ( Seung Ki Kwok ),박성환 ( Sung Hwan Park ),박미경 ( Mi Kyung Park ),조미라 ( Mi La Cho ),서수홍 ( Soo Hong Seo ),주지현 ( Ji Hyeon Ju ),윤종현 ( Chong Hyeon Yoon ),김호연 ( Ho Youn Kim ) 대한류마티스학회 2007 대한류마티스학회지 Vol.14 No.2

Objective: To investigate the effect of tumor necrosis factor (TNF)-α on the production of macrophage migration inhibitory factor (MIF), which might have important roles in the immune mediated inflammatory response of Behcet`s syndrome. Methods: Sixty two patients with Behcet`s syndrome and thirty healthy controls were included in this study. The concentrations of TNF-α in sera were determined by enzyme-linked immunosorbent assay (ELISA). Peripheral blood mononuclear cells (PBMCs) from eleven patients with Behcet`s syndrome were cultured for 48 hours with various concentration of TNF-α. The concentrations of MIF in sera and culture supernatants were determined by ELISA. Results: Serum levels of TNF-α were significantly higher in patients with Behcet`s syndrome than in healthy controls. TNF-α dose-dependently increased MIF production from PBMCs in patients with Behcet`s syndrome. Serum levels of TNF-α tended to correlate with serum levels of MIF, although did not reach statistical significance. Conclusion: Upregulation of MIF production by increased levels of TNF-α in patients with Behcet`s syndrome might be related to the pathogenesis of Behcet`s syndrome.

악성혈액질환이 동반된 베체트병에서 동종골수이식을 시행한 2예

권남희,전경만,박세훈,김동희,이순일,차훈석,이홍기 대한조혈모세포이식학회 2001 대한조혈모세포이식학회지 Vol.6 No.2

골수 이식 후 자가면역질환의 관해는 수 차례 보고 되었다. 그러나 베체트병과 악성혈액질환이 동반된 예에서 골수 이식을 시행한 경우는 보고된 바가 없다. 저자 등은 재생불량성빈혈 혹은 골수이형성 증후군과 각각 동반된 베체트병에서 동종 골수 이식을 시행하여 베체트병의 임상 증상이 호전된 후 양호한 임상 경과를 보이는 한 예와 정확한 분석이 어려운 한 예를 경험하였다. 현재까지 보고된 다른 자가면역질환에서와 같이 베체트병에서도 혈액질환이 동반되어 있는 경우 동종 골수 이식을 새로운 치료법으로 사용할 수 있으나, 장기적인 경과 및 치료 성적은 정리되지 않은 상태이다. 따라서 향후 더 많은 증례를 분석하여 그 의미를 찾아보아야 할 것이다. Allogeneic or autologous bone marrow transplantation (BMT) in autoimmune disease is a new approach for treating the patients who either do not respond to the standard treatments or require long-term administration of toxic drugs to maintain a clinical remission. We report two cases of allogeneic BMT in patients with Behcet"s disease which was accompanied with either aplastic anemia or myelodysplastic syndrome. A 36-year old woman who had abdominal pain, oral ulcer, and vaginal ulcer was diagnosed to have Behcet"s disease and severe aplastic anemia. Prednisolone, colchicine, and sulfasalazine were administered for the treatment of Behcet"s disease; however, patient had the persistent symptoms and signs without improvement. She underwent allogeneic BMT for the treatment of severe aplastic anemia. Following the transplantation, she recovered completely from Behcet"s disease as well as aplastic anemia. In the second case, a 33-year old woman was diagnosed to have Behcet"s disease, which was based on her recurrent oral ulcer, genital ulcer, and erythema nodosum. In the meantime, she was diagnosed to have myelodysplastic syndrome (RARS). Although her Behcet"s disease responded partially to colchicine, sulfasalazine, prednisolone, and dapsone, she required these drugs in high dosages for a long time. We performed allogeneic BMT for her myelodysplastic syndrome. She had successful engraftment after transplantation, and the symptoms and signs associated with Behcet"s disease temporarily disappeared. In conclusion, allogeneic BMT can be a promising curative treatment for Behcet"s disease accompanied with hematologic malignancies.

Incidence, prevalence, and mortality of Behcet’s disease in Korea: a nationwide, population-based study (2006-2015)

( Young Bok Lee ),( Soo Young Lee ),( Jin Young Choi ),( Ji Hyun Lee ),( Jin-wou Kim ),( Dong Soo Yu ) 대한피부과학회 2017 대한피부과학회 학술발표대회집 Vol.69 No.2

Background: The epidemiology of Behcet’s disease varies among ethnic populations worldwide. Trends in the incidence of Behcet’s disease have not been investigated based on the Korean National Health Insurance database. Objectives: This study investigated the incidence and mortality of Behcet’s disease by age using nationwide population data in Korea. Methods: A nationwide population-based cohort study was performed using the Korean National Health Insurance Claims Database from 2006 to 2015. Results: The annual incidence of Behcet’s disease per 100,000 person-years was 3.976 (2.587 for males and 5.373 for females) from 2006 to 2015. The incidence of Behcet’s disease peaked among people in their 40s (6.561 per 100,000 person-years). Incidence was significantly higher in subjects with comorbid metabolic conditions, such as diabetes mellitus, hypertension, and dyslipidemia. The mortality rate per 1,000 person-years increased with age in Behcet’s disease patients. Conclusion: This study showed the incidence, prevalence, and mortality of Behcet’s disease. Metabolic conditions increased the risk of Behcet’s disease among Koreans.

다발성 폐경색, 상대정맥 증후군 및 종격동 임파선 종대를 동반한 베체트 증후군 1예

장성훈,손호성,정연순,김진구,오세진,박무인,박선자,구자영 고신대학교 의학부 1999 高神大學校 醫學部 論文集 Vol.14 No.1-2

Behcet's syndrome is a multisystemic disease involving mucocutaneous, cardiovascular, central nervous system, renal, articular, gastrointestinal, skin, pulmonary and lymphatic system. Intrathoracic manifestations of Behcet' s syndrome are thromboembolism of vena cava, arterial aneurysm, vasculitis, pulmonary infarct, pulmonary hemorrhage, pleural effusion, and rarely mediastinal or hilar lymphadenopathy. We report a case of Behcet's syndrome manifested as pulmonary infarct, superior vena cava syndrome, and mediastinal lymphadenopathy.

Anti-Tumor Necrosis Factor Therapy in Intestinal Behcet’s Disease

( Jihye Park ),( Jae Hee Cheon ) 대한소화기학회 2018 Gut and Liver Vol.12 No.6

Intestinal Behcet’s disease is a rare, immune-mediated chronic intestinal inflammatory disease; therefore, clinical trials to optimize the management and treatment of patients are scarce. Moreover, intestinal Behcet’s disease is difficult to treat and often requires surgery because of the failure of conventional medical treatment. Administration of anti-tumor necrosis factor-α, a potential therapeutic strategy, is currently under active clinical investigation, and evidence of its effectiveness for both intestinal Behcet’s disease and inflammatory bowel diseases has been accumulating. Here, we review updated data on current experiences and outcomes after the administration of anti-tumor necrosis factor-α for the treatment of intestinal Behcet’s disease. In addition to infliximab and adalimumab, which are the most commonly used agents, we describe agents such as golimumab, etanercept, and certolizumab pegol, which have recently been shown to be effective in refractory intestinal Behcet’s disease. This review also discusses safety issues associated with anti-tumor necrosis factor-α, including vulnerability to infections and malignancy. (Gut Liver 2018;12:623-632)

다발성 폐경색, 상대정맥 증후군 및 종격동 임파선 종대를 동반한 베체트 증후군 1예

장성훈,손호성,정연순,김진구,오세진,박무인,박선자,구자영 고신대학교(의대) 고신대학교 의과대학 학술지 1999 고신대학교 의과대학 학술지 Vol.14 No.1

Behcet's syndrome is a multisystemic disease involving mucocutaneous, cardiovascular, central nervous system, renal, articular, gastrointestinal, skin, pulmonary and lymphatic system. Intrathoracic manifestations of Behcet's syndrome are thromboembolism of vena cava, arterial aneurysm, vasculitis, pulmonary infarct, pulmonary hemorrhage, pleural effusion, and rarely mediastinal or hilar lymphadenopathy. We report a case of Behcet's syndrome manifested as pulmonary infarct, superior vena cava syndrome, and mediastinal lymphadenopathy.

回盲腸 潰瘍을 同伴한 完全型 Behcet 症候群 1例 보고 : A case of complete form of Behcet's syndrome with multiple ileocecal ulcerations

崔秀全,林慶浩 인제대학교 1983 仁濟醫學 Vol.4 No.2

Behcet's syndrome has been known as multisystemic disease involving oral, genital and eye lesions with vascular system, kidney, joints, skin, central nervous system and gasrointestinal system. Recently authors experienced a case of complete from of Bthcet's syndrome with intestinal manifestation in volving multiple ulcerations on ileocecal area and with improvement of cell mediated immunity by Levamisole.

골수이형성증 환자에서 발생한 베체트병

민정아 ( Jung Ah Min ),김정은 ( Jung Eun Kim ),오신택 ( Shin Taek Oh ),조석구 ( Seok Goo Cho ),조백기 ( Baik Kee Cho ),박현정 ( Hyun Jeong Park ) 대한피부과학회 2009 대한피부과학회지 Vol.47 No.11

Behcet`s disease (BD) is a multisystemic inflammatory disease that affects various organs and it causes mucocutaneous lesions. BD is only rarely associated with leukemia or other hematologic disorders. Many cases of BD associated with myelodysplastic syndrome (MDS) have recently been reported and these cases are mainly in East Asia and the Mediterranean. We herein report on a case of a 34-year-old man who developed Behcet`s disease 8 years after the onset of MDS. He had two, firm, subcutaneous nodules on both shin and recurrent oral ulcer for 2 months. He also had a retinal vasculitis. Skin biopsy from the left shin showed the septal panniculitis, which was consistent with erythema nodosum. He had multiple ileal ulcers observed on the colonoscopy exam, so he was diagnosed with intestinal Behcet`s disease. Yet the chromosomal analysis revealed nonspecific abnormality and not trisomy 8. This is the first reported case of intestinal Behcet`s disease associated with MDS in the Korean dermatologic literature. (Korean J Dermatol 2009;47(11):1309~1313)