Clinical analysis of children Beh?et's disease with gastrointestinal involvement / 中华风湿病学杂志

Objective To summarize the clinical characteristics of Beh?et's disease (BD) in children with gastrointestinal involvement. Methods We retrospectively analyze the children BD with gastrointestinal involvement who were diagnosed in our hospital in recent 10 years. Results Twenty-two children were identified. The average age of onset was(6.1±4.0) years. The time from disease onset to clinical diagnosis was (1.2±2.1) years on average. Fifteen children had abdominal pain, diarrhea and hematochezia. Seven cases had positive endoscopic findings without any gastrointestinal symptoms. Twenty cases received corticosteroids therapy, 13 cases of them were treated with Cyclophosphamide/Methotrexate (CTX/MTX), 3 refractory cases were treated with biologics. Patients were followed up for (28±32) month on average. Eight patients' condition was stable, 7 patients were refractory, 3 patients died, 4 patients were lost to follow-up. At the same term, 5 patients without gastrointestinal involvement who received corticosteroids and CTX/MTX therapy were stable. Conclusion It is difficult to diagnose children BD at early stage. Gastrointestinal involvement may not be found, while the gastrointestinal endoscopy is of great importance in the diagnosis of the disease. Gluco-corticoid combined with immunosuppressive agents are effective. As to refractory patients, biological agent might be used although the recurrence is common. Compared with BD without gastrointestinal involvement, children BD with gastrointestinal involvement have serious condition and poor prognosis.

Lymphoma mimicking Beh?et's disease:case report and literature review / 中华风湿病学杂志

Objective To study the clinical char acteristics and outcome of two lymphoma patients mimicking Beh?et's disease. Methods Lymphoma was diagnosed in two patients mimicking Beh?et's disease referred to our Department in 2015. A search on published similar cases in Chinese database and the Pubmed was also performed and then analyzed. Results Eight patients were indentified in this pooled analysis, six of which were non-Hodgkin lymphoma (NHL). All of the eight cases presented with cutaneous lesion, seven cases with fever, seven cases with oral ulceration and six cases with orogenital ulceration, respectively. Ocular involvement was present in four of the eight cases, two were with a positive pathergy test. Among feverish patients, six were moderate or high fever, four were high fever, one was low-grade fever. Neutropenia was found in four patients, and lymphocytoponia in four of five patients with detailed data. All patients fulfilled the 2014 International Criteria for Beh?et's Disease (ICBD) with an average score of (5.8 ±1.5), ranging from 4 to 8. Survival period ranged from one month to 36 months, with an average of 8 months. Conclusion For patients diagnosed as Beh?et's disease are finally diagnosed as lymphoma. For patients with Beh?et's disease present-ation but also present with mediate to high fever, atypical deepseated ulcer, neutropenia or lymphocytoponia, malignancy especially lymphoma should be investigated.

To demonstrate the change in peripheral blood T lymphocyte subgroups and cytokines of Behet′s syndrome / 中华风湿病学杂志

Objective To demonstrate the change in peripheral blood T lymphocyte subgroup and cytokines of Beh?et′s disease (BD). Methods T lymphocyte subgroup was detected by flow cytometry and serum interleukin (IL)-8, sIL-2R, IL-1?, IL-2, IL-6 and TNF-? were assayed by RIA. Results CD4, CD4/CD8, NK of BD decreased evidently and CD8 incrdased. Serum IL-8, sIL-2R increased compared to normal controls. Change of IL-2, IL-6, tumor necrosing factor (TNF)-? , IL-1? showed no statistical significance compared to normal group. Conclusion Change in peripheral blood T lymphocyte subgroup and serum IL-8, sIL-2R can be one of the activity index of BD.

A Case of Transluminal Stent-Graft for Thoracic Aortic Aneurysm with Behcet's Syndrome

Beh et's syndrome is a multisystem disorder presenting with recurrent oral and genital ulcerations as well as ocular involvement. Vascular complications consist of venous thromboembolism, arterial aneurysms and occlusions, and they develop in about 25% of patients. Weakening of the aortic wall may lead to aneurysms that may rupture and cause life-threatening hemorrhage, but nothing in the surgical and nonsurgical techniques proposed for the treatment for aneurysms in Beh et's syndrome has proved to be satisfactory. The traditional treatment for thoracic aotic aneurysms is the surgical replacement of a prosthetic graft. Although advances in the operative care of patients with thoracic aortic aneurysms have been achieved, the associated morbidity and mortality are considerable, especially in those with coexisting conditions such as advanced age, coronary artery disease and heart failure. Currently, transluminally placed endovascular stent-grafts offer an alternative approach to treatment that is potentially less invasive with a lower risk. We report a 37-year-old male patient with thoracic aortic aneurysm associated with Beh et's syndrome. Transluminal endovascular stent-graft placement was attempted : the stent-graft was introduced through a 22-Fr sheath using a common femoral artery cut down and expanded to 25-30 mm in diamter. There was increased thrombosis of the aneurysm on a follow-up imaging study, and the patient was discharged without complications.

A Case of Transluminal Stent-Graft for Thoracic Aortic Aneurysm with Behcet's Syndrome

Beh et's syndrome is a multisystem disorder presenting with recurrent oral and genital ulcerations as well as ocular involvement. Vascular complications consist of venous thromboembolism, arterial aneurysms and occlusions, and they develop in about 25% of patients. Weakening of the aortic wall may lead to aneurysms that may rupture and cause life-threatening hemorrhage, but nothing in the surgical and nonsurgical techniques proposed for the treatment for aneurysms in Beh et's syndrome has proved to be satisfactory. The traditional treatment for thoracic aotic aneurysms is the surgical replacement of a prosthetic graft. Although advances in the operative care of patients with thoracic aortic aneurysms have been achieved, the associated morbidity and mortality are considerable, especially in those with coexisting conditions such as advanced age, coronary artery disease and heart failure. Currently, transluminally placed endovascular stent-grafts offer an alternative approach to treatment that is potentially less invasive with a lower risk. We report a 37-year-old male patient with thoracic aortic aneurysm associated with Beh et's syndrome. Transluminal endovascular stent-graft placement was attempted : the stent-graft was introduced through a 22-Fr sheath using a common femoral artery cut down and expanded to 25-30 mm in diamter. There was increased thrombosis of the aneurysm on a follow-up imaging study, and the patient was discharged without complications.