Chinese consensus on diagnosis and treatment of intestinal Beh?et′s disease / 中华消化杂志

Beh?et′s syndrome is a kind of chronic systemic vasculitis with involvement of multiple organs. Intestinal involvement of Beh?et′s syndrome is presently named as intestinal Beh?et′s syndrome. Recently, there is considering another kind of disease type with only typical intestinal ulcers. Since it is difficult to differentiate intestinal Beh?et′s syndrome from Crohn′s disease, intestinal tuberculosis, intestinal lymphoma, and intestinal manifestations of many other autoimmune diseases, and there is limited evidence for the therapy of intestinal Beh?et′s syndrome, proposing diagnosis and treatment recommendations for intestinal Beh?et′s syndrome through evidence-based judgment will be of great significance for clinical practice.

Clinical characteristics of 47 cases of intestinal Beh?et′s disease / 中华消化杂志

Objective:To summarize the clinical features of intestinal Beh?et′s disease, so as to provide reference for the diagnosis of the disease.Methods:From April 1 2014 to January 31 2019, the clinical data of 47 patients diagnosed as intestinal Beh?et′s disease at the Second Hospital of Hebei Medical University were retrospectively analyzed, which included initial symptoms, gastrointestinal symptoms, complications, erythrocyte sedimentation rate (ESR), the levels of C reactive protein (CRP), hemoglobin, serum albumin, results of acupuncture test, gastrointestinal involved site and ulcer shape. At the same time, gender differences of clinical manifestations were compared. Chi-square test was used for statistical analysis.Results:Among 47 patients with intestinal Beh?et′s disease, the initial symptoms of 26 (55.3%) cases were gastrointestinal symptoms. Abdominal pain was the most common symptom, the others were diarrhea, anorexia, abdominal distension and perianal abscess, and the incidence rate was 80.9%(38/47), 46.8% (22/47), 42.6% (20/47), 36.2% (17/47) and 2.1% (1/47), respectively. The main complications were gastrointestinal bleeding, perforation and obstruction, and the incidence rates was 40.4% (19/47), 4.3% (2/47) and 4.3% (2/47), respectively. Thirty-seven (78.7%) patients had different degrees of hypoalbuminemia (serum albumin<35 g/L). The CRP level of 36(76.6%) patients increased. The ESR of 36 (76.6%) patients increased. Twenty-two (46.8%) patients had mild anemia (hemoglobin<90 g/L). The acupuncture test was positive in 25 (53.2%) patients. The involved sites of gastrointestinal tract were terminal ileum and ileocecal junction, colon, esophagus, duodenum and jejunum, stomach, and rectum, the proportion was 57.4% (27/47), 27.2% (13/47), 23.4% (11/47), 23.4% (11/47), 17.0% (8/47) and 8.5% (4/47), respectively. All 47 (100.0%) patients had oral ulcers. 62.1%(18/29) patients presented with multiple ulcers under endoscope. The shape of ulcer was round ulcer, irregular ulcer, and longitudinal ulcer, the proportion was 48.3% (14/29), 34.5% (10/29) and 17.2 (5/29), respectively. The incidence rate of genital ulcer of female patients with intestinal Beh?et′s disease was higher than that of male patients with intestinal Beh?et′s disease (85.7%, 18/21 vs. 30.8%, 8/26), and the difference was statistically significant ( χ2=14.189, P<0.01). There were no significant differences between the female group and the male group in the incidence rate of oral ulcer, abdominal pain, diarrhea, and positive rate of acupuncture test (100.0%, 21/21 vs. 100.0%, 26/26; 85.7%, 18/21 vs. 76.9%, 20/26; 42.9%, 9/21 vs. 50.0%, 13/26; 52.4%, 11/21 vs. 58.3%, 14/26, all P>0.05). Conclusions:The common clinical symptoms of intestinal Beh?et′s disease are oral ulcers, abdominal pain, diarrhea and genital ulcer. Female patients with intestinal Beh?et′s disease are more likely to develop genital ulcer than male patients with intestinal Beh?et′s disease. Multiple ulcers are more common under endoscopy, which are round ulcer, irregular ulcer and longitudinal ulcer. The most common sites are the terminal ileum and ileocecal junction, followed by colon, esophagus and other parts.

Clinical analysis of 25 cases of Beh?et′s disease complicated with pulmonary hypertension / 中华全科医师杂志

Objective To understand the clinical features of Behcet′s Disease( BD) with pulmonary hypertension( PH) .Methods The etiology, clinical features, treatment and prognosis of 25 patients with Bahcet′s disease ( BD) complicated with pulmonary hypertension ( PH) admitted in Peking Union Medical College Hospital from January 2000 to August 2015 were retrospectively reviewed.Results Total 912 BD patients were hospitalized during the same period, among whom 25 cases were complicated with PH accounting for 3%.There were 15 males (60%) and 10 females (40%), with the mean age of (33 ±12) years (range 19 to 66 years).The median interval from the diagnosis of BD to the onset of PH was 1 year (range 0 to 40 years).The most common cause of PH was heart valve disease (n=10, 40%), followed by pulmonary arterial stenosis or occlusion (n=6, 24%), pulmonary arterial aneurysm with thrombus (n=1, 4%), pulmonary thrombosis (n=1, 4%), cardiomyopathy (n=1, 4%);there were no causes identified in 6 cases (24%).Pulmonary arterial pressures estimated by echocardiography were 40 to 117 mmHg (1 mmHg=0.133 kPa) with a mean pressure of (60 ±22) mmHg.When the PH developed, elevated erythrocyte sedimentation rate ( ESR ) and increased hypersensitive C-reactive protein ( hs-CRP ) were founded in 48% ( 11/23 ) and 82% ( 14/17 ) of patients, respectively.After treated with glucocorticoid (96%,24/25), immunosuppressive agents(92%, 23/25), anticoagulation or thrombolysis(36%,9/25) and specific targeted vasodilator(32%,8/25), the levels of ESR and hs-CRP declined in 91% (10/11) and 71%(10/14) of patients, respectively; and pulmonary arterial systolic pressure declined in 50% of cases (8/16).Among 9 patients followed for 2 to 96 months, 4 died, 1 aggravated, 2 kept stable and 1 improved.Conclusion PH is an uncommon complication in disease.Heart valve disease, pulmonary artery involvement are the major causes of PH.The therapeutic effect and prognosis are poor.

A Case of Brain Stem Neuro-Behcet's Disease Presenting as Paroxysmal Headache With Autonomic Features

Migraine without aura was significantly frequent in Beh et's disease patients. While, the relationship of TAC(trigeminal autonomic cephalalgia) and Beh et's disease was not known. And, it is thought that pathogenesis of proxysmal headache with autonomic features in neuroBehcet's disease as our case may be different from those of clust headache and TAC. A 45-year-old male admitted with left sided periobital poxysmal headache accompained by ipsilateral conjunctival injection and lacrimination with autonomic features. T2WMR image showed about 1.5x2.6x2.6 cm sized mass with high signal intensity surrounded by a rim of low signal intensity in pons. Enhanced MR image showed enhancing mass-like lesion inside the T2 high signal intensity. CSF study revealed neutrophilic pleocytosis with normal pressure, protein and glucose concentration. No malignant cell were seen. Antiphospholipid antibody and cryoglobuline was significantly increased. 3 days after hospitalvisit, oral ulcerations appeared in the patient. He was treated with high-dose steroid for a week and his headache and abnormal findings of CSF study became fully recovered. Two weeks later, the mass was nearly reduced in the pons on follow-up MR image. We suggest to include a careful interviewfor 2ndary TAC as the first neurologic symptom in the diagnostic work-up of Behet's disease.

Clinical analysis of incomplete Beh et′s disease / 中华眼底病杂志

Objective To investigate the clinical diagnosis and treatment of incomplete Beh et′s disease. Methods The clinical data of 52 patients (83 eyes ) with incomplete Beh et′s disease were retrospectively analysed. Visual acuity and results of examination of slit-lamp microscope, ocular fundus, and fundus fluorescein angiography (FFA) were observed and analysed. All of patients were treated by local cycloplegic, systemic treatment with glucocorticoid and cytoxan. The followed-up period was 1 year or more. Results Recurrent hypopyon or empyema of anterior chamber angle, and hyperaemia of optic papilla was found in 54 and 41 eyes, respectively. The results of FFA showed that abnormal changes were found in all of the eyes. There was vascular leakage and staining of optic papilla at the early and late stage in 83 eyes (100%), diffuse leakage of retinal capillary vessel in 83 eyes (100%), and leakage of affected branch of retinal vessel and staining of the vascular wall at the late stage in 71 eyes (85.54%).After the treatment, the disease recovered completely in 20 patients (26 eyes), recovered partly in 20 (33 eyes), and alleviated in 12 (24 eyes). No patients suffered from hypopyon or empyema of anterior chamber angle and hyperemia of optic papilla. None of the patients had oral ulcer, genital ulcer or arthritis until the last day of the follow-up. Conclusions Recurrent hypopyon, papillitis, and retinal vasculitis were the main clinical manifestations of incomplete Beh et′s disease. FFA redounds to the diagnosis of the disease and timely application of cycloplegic and systemic application of glucocorticoid and cytoxan may inhibit the aggravation of the disease effectively.

Preliminary observation of the therapeutic effects of vitrectomy on chronic and refractory Beh et′s disease / 中华眼底病杂志

Objective To evaluate the therapeutic effects of vitrectomy on chronic and refractory Beh et′s diseases. Methods The clinical data of 8 patients (10 eyes) with Beh et′s diseases from 0.5 to 3 years after vitrectomy were retrospectively analyzed. Pre-and post-operative visual acuity, control of inflammation after the surgery, and the recurrence were observed and analyzed. Both of the eyes were involved in all of the patients, including complete Beh et′s diseases in 5 and incomplete in 3. Results Vitreous opacity and liquefaction were found in all of the involved eyes, dark retina and thin retinal vessels were seen in most of the eyes, and vascular obstruction or sheath-like changes in different degrees were detected in some eyes. Improvement of visual acuity was observed in all of the eyes which had undergone the treatment after the operation. There was significant difference between the visual acuity before the surgery and at the 1st and 2nd week and the 1st and 6th month after the surgery. During the follow-up period, recurrence of the inflammation was found in 3 eyes within 1 month and in 6 eyes within 6 months; no recurrence was found in 4 eyes after the operation. No acute recurrence of inflammation was found after operation in the involved eyes. Conclusion Vitrectomy for chronic and refractory Beh et′s diseases may improve the visual acuity of the involved eyes, and the surgery is safe and effective.

A Case of Myelodysplastic Syndrome with Behcet's Disease / 대한혈액학회지

Myelodysplastic syndrome (MDS) is a hematologic disorder characterized by peripheral cytopenia and histologic feature of hematologic dysplasia. MDS has rarely been reported in association with Behcet's disease. We describe a patient with MDS associated Behcet's disease and a review of the literature.

Association of human leukocyte angtigen-B51 and Behet′s disease with uveitis / 中华眼底病杂志

0.10). Conclusions HLA-B51 might be a susceptible gene for BD, and there was a weak association between HLA-B51(HLA-B*5101) and BD patients with uveitis.

Molecular genetics (HLA) of Behcet's disease

Behcet's disease (BD) has been known to be strongly associated with the human leukocyte antigen (HLA) B51. This B51 association has been confirmed in many different ethnic groups between the Middle East and Japan, and it has been proposed that BD is prevalent in those ethnic groups along the old Silk Route. The hypothesis could be made that B51 molecules are primarily involved in BD development through specific antigen presentation. However, polymorphic analyses of the TNFB gene and Tau-a microsatellite between the HLA-B and TNF genes indicate that the pathogenic gene of BD is not the HLA-B51 gene itself but another gene located around the HLA-B gene. HLA-C genotyping by the PCR-SSP method also suggests that the BD pathogenic gene is not the HLA-C gene itself but other gene located near the HLA-B gene. Recently we sequenced a single contig of 236,822 bp from the MICA gene (58.2 kb centromeric of HLA-B) to 90.8 kb telomeric of HLA-C and identified 8 novel genes designated NOB1-8 (NOB: new organization associated with HLA-B). During the course of the genomic sequence analysis we clarified the genetic structure of the MICA (MHC class I chain-related gene A) gene and found a triplet repeat microsatellite polymorphism of (GCT/AGC)n in the transmebrane (TM) region. Furthermore, the microsatellite allele consisting of 6 repetitions of GCT/AGC (MICA A6 allele) was present at a significantly higher frequency in the BD patient group than in the control group and a significant fraction of B51-negative patients were positive for this MICA A6 allele. These results suggest the possibility of a primary association of BD with MICA rather than HLA-B.

Streptococcal infection in the pathogenesis of Behcet's disease and clinical effects of minocycline on the disease symptoms

Although the precise pathoetiology of Behcet's disease (BD) remains obscure, patients with BD have a high incidence of chronic infectious foci, indicating an enhanced susceptibility to chronic tonsillitis, and dental caries. Sometimes, clinical symptoms appear after treatment of these foci in BD patients. It is believed that BD might be related to an allergic reaction to a bacterial infection in view of the many clinical symptoms, especially the presence of aphthous and genital ulcerations. An attempt to obtain cutaneous responses to bacterial antigens has been carried out using various vaccines developed from bacteria isolated from the ulcerative lesions and oral cavities of BD patients. BD patients often show intense hypersensitivity to various strains of streptococci, not only by their cutaneous reactions but also by in vitro testing. In this report, we describe our previous studies on the correlation between streptococcal antigens and the pathogenesis of BD and also discuss the recent reports of other authors. The intense hypersensitivity to streptococcal antigens acquired after streptococcal infection is thought to play an important role in the appearance of symptoms in BD patients since the production of pro-inflammatory cytokines by peripheral blood mononuclear cells (PBMC) was enhanced when stimulated with streptococcal antigen in a culture system. Minocycline, an antibiotic to which certain strains of streptococci are sensitive, reduced the frequency of clinical symptoms in BD patients as well as the production of pro-inflammatory cytokines by BD-PBMC stimulated with streptococcal antigen.