Adult ; Behcet Syndrome ; complications ; Female ; Heart Atria ; pathology ; Humans ; Myocardial Infarction ; complications ; Pericardial Effusion ; complications

Behcet's disease (BD) is a chronic, relapsing, multisystem disorder, characterized by recurrent oral ulcer, genital ulcers, eye lesion, and skin lesion. The underlying pathology is nonspecific vasculitis of all vessel sizes, and severe vasculitis can result in fistula formation of neighboring tissues due to a necrotic process. Herein, eleven cases of BD combined with fistula are presented. In the present study, various types of fistula were associated; enterocutaneous fistula in six patients, and rectovaginal fistula in two. The other three patients showed aortoduodenal fistula, urethrovaginal fistula and urethrocutaneous fistula. They were treated with a corrective operation, but the prognoses were poor due to frequent relapses.
Humans ; Fistula/*etiology/surgery ; Female ; Behcet Syndrome/*complications/pathology ; Adult ; Adolescent

Esophageal involvement in Behcet's disease is very rare, and normally is observed as aphtosis and esophagitis, but serious complications such as erosions, perforations, and stenosis may occur. We carried out this prospective study to evaluate the prevalence of esophageal involvement in Behcet's disease and to establish if routine endoscopy and/or manometry are necessary. Twenty-three patients who fulfilled the diagnostic criteria of the international study group for Behcet's disease were enrolled. None were taking drugs or had disease that might produce esophageal abnormalities or alter any existing changes due to the Behcet's disease itself. Twenty- three patients underwent esophagogastroduodenoscopy by a single observer. Esophageal biopsies were performed in thirteen patients and esophageal manometry in twenty. At the beginning of the study, the disease activity was defined by the presence of more than one symptom related to Behcet's disease, and upon the classification of Behcet's disease. Of the twenty three patients enrolled two were excluded from final analysis because of the presence of hiatus hernia. Thus, 13 men and 8 women, ranging in age from 20 to 63 years with a mean age of 36.2 years were included. Ten patients (47.6%) had active disease and four (19%) complained of upper gastrointestinal symptoms at the time of the study. Fourteen patients had endoscopic, manometric and/or microscopic abnormalities. Esophageal manometry was performed in twenty patients and was abnormal in 7 cases (35%). Esophageal biopsies were done in 13 patients and revealed histopathological abnormalities in 5 cases. Microscopic findings showed vasculities in one case, and non-specific inflammatory infiltration mainly consisting of neutrophils in 4 cases. Our results suggest that the prevalence of esophageal involvement in Behcet's disease is rather high and occur even in asymptomatic patients, but that this usually does not result in specific abnormalities.
Adult ; Behcet Syndrome/*complications ; Esophageal Diseases/*epidemiology ; Esophagoscopy ; Esophagus/pathology ; Female ; Human ; Male ; Manometry ; Middle Age ; Prevalence ; Prospective Studies

Ten cases of post-inflammatory aneurysm of the thoracic aorta were studied morphologically using aortic wall and aortic valve tissue resected during the surgical repair of the lesions. Four cases of aortic aneurysm in Behcet's disease showed massive perivascular infiltration of the lymphocytes and plasma cells in adventitia and vasa vasorum. Fibrous obliteration of the vasa vasorum was seen, and neutrophilic infiltration was also associated in two cases with recurrent prosthetic valve failure. Three cases of aortic aneurysm associated with Takayasu's arteritis showed similar histologic features, but giant cells and histiocytic reactions were additional findings. Plasma cell infiltration was more pronounced compared to the Behcet's aortitis. One case of luetic aortitis showed a non-specific chronic inflammatory reaction with florid endothelial cell proliferation. One case of ankylosing spondylitis showed fibrous thickening at the periannular portion of the aortic valve cusps, but this finding was also seen in an aortic valve of a patient with Behcet's disease. One case without any disease association showed similar features of chronic inflammation with granuloma formation. The findings suggest that they have similar histologic features indistinguishable in terms of the clinical disease association, but a granulomatous reaction could be expected in Takayasu's arteritis.
Adult ; Aorta, Thoracic ; Aortic Aneurysm/*etiology/pathology ; Aortitis/*complications/pathology ; Behcet Syndrome/complications/pathology ; Female ; Humans ; Male ; Middle Aged ; Spondylitis, Ankylosing/complications/pathology ; Syphilis/complications/pathology ; Takayasu Arteritis/complications/pathology

Behcet's disease (BD) has been recognized as multi-systemic chronic vasculitic disorder of recurrent inflammation, characterized by the involvement of multiple organs and resulting in orogenital ulcers, uveitis, and skin lesions. Involvement of the central nervous system, vessels, and intestines in BD often leads to a poor prognosis. Digestive manifestations in BD have been reported in up to 1-60% of cases, although the rate varies in different countries. The most frequent extra-oral sites of gastrointestinal involvement are the ileocecal region and the colon. Gastric or esophageal involvement is reported to be very rare. Moreover, there have been no reports on the simultaneous involvement of the esophagus, stomach, ileum, and colon. Here, we present a 55-year-old Korean man with intestinal BD and multiple ileal and colonic ulcerations complicated by perforation, gastric ulcer with bleeding followed by perforation, and esophageal ulcers with bleeding.
Behcet Syndrome/complications/*diagnosis/pathology ; Cecal Diseases/complications/pathology ; Diagnosis, Differential ; Endoscopy, Digestive System ; Gastrointestinal Diseases/complications/*diagnosis ; Gastrointestinal Hemorrhage ; Humans ; Intestinal Perforation/*diagnosis/etiology/pathology ; Male ; Middle Aged ; Peptic Ulcer Perforation/pathology ; Stomach Ulcer/complications/pathology

BACKGROUND: Neuropsychiatric systemic lupus erythematosus (NPSLE) shows some similarities to neuroBehcet's disease (NBD) in that both conditions have some analogous clinical features and they are both pathologically associated cerebral vasculopathy. This study compared the clinical manifestations, brain MRI findings and prognosis of NPSLE and NBD patients. METHODS: Forty three patients with NPSLE (n = 25) or NBD (n = 18), who were monitored at a single center, were enrolled in this study. We retrospectively analyzed the clinical and brain MRI data. The neuropsychiatric manifestations were classified in both groups according to the new American College of Rheumatology nomenclature for NPSLE. RESULTS: The diffuse symptoms that included mood disorders, psychosis, confusion, cognitive dysfunctions, generalized seizures and headaches other than migraine or cluster headaches were more commonly observed in the NPSLE patients, while the frequency of focal diseases such as cranial neuropathy tended to be higher in the NBD patients. The brain MRI revealed that the NBD patients had more abnormalities in the brain stem than did the NPSLE patients. Most of the patients improved, at least partially, after being treated with glucocorticoid and/or immune suppressants. However, the disease course differed significantly between the two groups. There were more episodic cases in the NPSLE group of patients, while there were more remittent cases in the NBD group of patients. CONCLUSION: NPSLE had a tendency to cause diffuse neuropsychiatric manifestations, and it has a different predilection of brain lesions compared with NBD. The NBD patients showed a poorer outcome than did the NPSLE patients, suggesting that different therapeutic strategies for the two diseases need to be considered.
Adult ; Behcet Syndrome/complications/*diagnosis/pathology ; Brain/*pathology ; Female ; Humans ; Lupus Erythematosus, Systemic/complications/diagnosis/pathology ; Lupus Vasculitis, Central Nervous System/complications/*diagnosis/pathology ; *Magnetic Resonance Imaging ; Male ; Prognosis ; Retrospective Studies ; Risk Factors

OBJECTIVES: To assess the clinical features, pathologic findings, postoperative results and the effects of immunosuppressive therapy in patients with Beh et's disease (BD). METHODS: We reviewed the postoperative course of the 9 BD patients who underwent a total of 17 aortic valve replacement procedures with prosthetic valves. RESULTS: Histological examination of the aortic valve commonly revealed diffuse myxoid degeneration (75 percent). Of 17 valve replacement surgeries, 13 surgeries resulted in complications, such as detachment of the prosthetic valve with perivalvular leakage and dehiscence of the sternotomy wound, within an average of 5 months (range from 1 month to 14 months). The rate of prosthetic valve detachment was 76 percent (13 of 17 surgeries). Four of the 9 patients (44 percent) who underwent aortic valve replacement procedures died of heart failure or infection associated with the detachment of the prosthetic valve, and perivalvular leakage within an average of 9 months. Aortic insufficiency associated with dehiscence of the prosthetic valve developed in 11 of 12 surgical cases (92 percent) with a mechanical valve and 2 of 5 surgical cases (40 percent) with tissue valves. Thirteen of 15 surgeries (87 percent) which were not given postoperative immunosuppressive therapy developed complications, while none of 2 surgeries that used postoperative immunosuppressive therapy with prednisolone (1 mg/kg/day) and azathioprine (100 mg/day) had these complications. CONCLUSION: The rates of prosthetic valve detachment in BD involving aortic valve were higher than those of other diseases. Aortic valve involvement was also one of the poor prognostic factors in BD. Intensive postoperative immunosuppressive therapy and surgical methods may be important factors for postoperative results.
Adult ; Aortic Valve/pathology ; Aortic Valve Insufficiency/*etiology/pathology ; Behcet Syndrome/*complications/drug therapy/pathology ; Female ; Heart Valve Diseases/*complications/pathology/surgery ; Heart Valve Prosthesis Implantation/*mortality ; Human ; Immunosuppression ; Male ; Postoperative Complications ; Prosthesis Failure ; Survival Analysis

We report an unusual case of unilateral frosted branch angiitis associated with Behcet's disease, including a review of previously reported cases. A 39-year-old male with history of recurrent oral and genital ulcers presented with visual loss in his left eye. Fundus findings demonstrated occlusive retinal vasculitis resembling acute frosted branch angiitis. Laboratory examinations including viral markers revealed no abnormal findings except positive HLA-B51. The patient was treated with systemic steroid and cyclosporine. Six months after presentation, new oral ulcers and pseudofolliculitis appeared, and he was diagnosed with Behcet's disease following rheumatology consultation. During follow-up, there was no change in visual acuity of hand movement, and disc neovascularization developed even after complete panretinal photocoagulation. Ocular manifestations of Behcet's disease can present as unilateral frosted branch angiitis, and may consecutively involve in both eyes. Early immunosuppressive treatment is recommended.
Adult ; Behcet Syndrome/*complications/diagnosis ; Diagnosis, Differential ; Fluorescein Angiography ; Fundus Oculi ; Humans ; Magnetic Resonance Imaging ; Male ; Retinal Artery/*pathology ; Retinal Vasculitis/diagnosis/*etiology

No abstract available.
Adult ; Behcet Syndrome/complications/*diagnosis ; Colon/blood supply/pathology/surgery ; Colonoscopy ; Gastrointestinal Hemorrhage/*diagnosis/etiology/surgery ; Humans ; Ileum/blood supply/surgery ; Male ; Rectum/blood supply/surgery

No abstract available.
Adult ; Behcet Syndrome/*complications/diagnosis ; Follow-Up Studies ; Humans ; Male ; Optic Disk/*pathology ; Optic Neuritis/diagnosis/*etiology ; Time Factors ; Visual Acuity