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Objective To explore the characteristics of diagnosis and treatment of Behset's disease (BD) with meningeal thickening and to improve doctors" awareness of such diseases.Methods We reported a missed diagnosis of a patient with BD complicated with diffuse meningeal thickening,and reviewed the related literature.Results A 25-year-old young man,manifested mainly as recurrent headache,fever,recurrent oral ulcer,erythema skin nodules and folliculitis,and his cranial radiology revealed diffuse meningeal thickening and intracranial venous sinus thrombosis.We diagnosed him as neuropathy associated with BD.After the treatment with steroids,cyclophosphamide,infliximab and anti-coagulants,his symptoms improved rapidly.Conclusion In clinical,BD complicated with meningeal thickening is rare,which is easily misdiagnosed or miss-diagnosed.For the patients with unexplained meningeal thickening,the symptoms of BD,such as recurrent oral or genital ulcers,ophthalmitis and skin lesions,should be acquired in detail.In addition to steroids and immunosuppressive agents,anti tumor necrosis factor (TNF)-alpha can also be used in the treatment of BD with meningeal thickening.
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Objective To explore the use of biological agents in Neuro-Beh(c)et's disease (NBD).Methods We retrospectively reviewed the clinical data of five NBD patients treated with biological agents who were in-patients at Peking Union Medical College Hospital between June 2009 and June 2016.The continuous variables were analyzed by t test.Results All five cases (4 male and 1 female) were severe and/or refractory patients with parenchymal involvement (pNBD).Their age at neurological onset was (31±12) years old.Four cases presented with multiple lesions.The brainstem,spinal cord,cerebral hemisphere and cerebellum involvement were presented in 4,3,3 and 2 patients,respectively.The Rankin score at the onset of NBD was (4.0±0.7).The biological agents were administrated when corticosteroids and immunosuppressant were ineffective.Three cases received tumor necrosis factor (TNF)-α inhibitors therapy,among whom one patient had gastrointestinal ulceration.One patient with refractory retinal vasculitis received interferon-α therapy.One patient treated with tocilizumab [interleukin (IL)-6R inhibitor] had high level IL-6 in the cerebrospinal fluid.All patients achievcd clinical improvements and the Rankin score significantly decreased to (2.2±0.8) when compared with the baseline (t=4.81,P＜0.01) after the treatment with biological agents.The corticosteroid dose was tapered in all cases and the numbers of immunosuppressants were reduced in most cases,indicating a potential steroid and immunosup-pressant-sparing effect.No serious adverse events were observed during the follow-up.Conclusion Neurological involvement is a severe complication of Behqet's disease.We can take appropriate biological agents such as TNF-α inhibitors or interferon-α into consideration when patients have severe/refractory pNBD.
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Trisomy 8 in myelodysplastic syndrome (MDS) plays an important role in concurrent intestinal Beh?et's disease (BD) pathogenesis.Here,we reported a case of intestinal BD combined with MDS involving trisomy 8.A 48-year-old woman who has had a 20-year history of recurrent oral ulcer,perineal ulcer and iris,was diagnosed as MDS with trisomy 8 four years ago.She developed high fever and acute abdominal pain.Multiple ulcerative perforations in ileum and colon were found by endoscopy,meeting the criteria for intestinal BD.The patient was successfully treated with immunomodulatory drugs.
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Trisomy 8 in myelodysplastic syndrome (MDS) plays an important role in concurrent intestinal Beh?et's disease (BD) pathogenesis.Here,we reported a case of intestinal BD combined with MDS involving trisomy 8.A 48-year-old woman who has had a 20-year history of recurrent oral ulcer,perineal ulcer and iris,was diagnosed as MDS with trisomy 8 four years ago.She developed high fever and acute abdominal pain.Multiple ulcerative perforations in ileum and colon were found by endoscopy,meeting the criteria for intestinal BD.The patient was successfully treated with immunomodulatory drugs.
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Ocular Beh(c)et disease,which usually presents as panuveitis,with characteristic of vision impairment and relapse,is one of the most refractory uveitis entities.Usually,one or more conventional immunosuppressive agents,such as glucocorticoid,ciclosporin,chlorambucil,cyclophosphamide,etc,are needed in disease management.However,long-term use of immunosuppressive agents would result in severe adverse effects.New biologics,such as interferon,antitumor necrosis factor agents,interleukin or its receptor antagonists,provide new therapeutic approaches to the treatment for this disease.This paper reviewed the conventional therapies and biologics applied in ocular Beh(c)et disease.
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Objective To investigate the clinical characteristics of Beh(c)et's disease (BD) complicated with cerebral venous sinus thrombosis (CVST).Methods In patients data at Peking Union Medical College Hospital between February 2004 and April 2013 were reviewed retrospectively to identify patients who were diagnosed as BD complicated with CVST.SPSS 19.0 software was used for statisticals analysis.Data of normal distribution was expressed by x±s (standard deviation) and that of abnormal distribution by median and range.Results Of 601 inpatients with BD during that period,12 patients (2％,6 male and 6 female) developed CVST,and their age was (28±10) years old (range 15 to 52 years old).Most of patients (92％,11/12)suffered neurological symptoms after other initial systemic symptoms of BD in mean (56±35) months.The most frequent neurological signs were headache (11 patients,92％),papilledema (9 patients,75％),nausea/vomiting (8 patients,67％) and blurred vision (6 patients,50％),with progression as the main mode of onset (9/11,82％).Eight out of 10 patients (80％) were found elevated cerebrospinal fluid (CSF) pressure by lumbar puncture.Occlusions of multiple sinuses (8 patients,67％) were more frequent than single occlusion (4 patients,33％).The most frequent locations of CVST were the transverse sinus (9 patients,75％),superior sagital sinus (8 patients,67％) and sigmoid sinus (7 patients,58％).Other than CVST,8 patients were complicated with other sites of thrombosis (7 cases in lower extremity veins and 1 in pulmonary artery).In addition,cerebral infarction was found in 3 patients,subarachnoid hemorrhage and right temporal lobe hemorrhage in 1 patient,respectively.After glucocorticoid,immunosuppressant,as well as anticoagulant and dehydration therapy,with a median follow-up of 8 months (0.7 to 88.8 months),10 patients achieved clinical improvements (83％),2 patients relapsed (17％).Conclusion CVST is a relatively rare complication of Beh(c)et's disease,with headache,papilledema,nausea/vomiting and blurred vision as the common clinical manifestations.By early identification,timely diagnosis and active treatment,the prognosis of most patients is good.
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Background Interleukin (IL)-1a-889C/T,IL-1β-S11C/T,+ 3962C/T and IL-1Ra-2 are different morphology of single nucleotide of interleuki-1 (IL-1).The potential relationship of IL-1 polymorphisms with Beh(c)et disease has been studied in several populations and groups.However,study outcomes are controversial for a long duration.Objective This Meta-analysis was to determine whether the IL-1 gene polymorphisms is associated with the pathogenesis and development of Beh(c)et disease.Methods The literature of the assoiation between IL-1 polymorphisms and Beh(c)et disease was retrieved from Medline,EMBASE,Cochrane Library,Web of knowledge,Google Scholar,Wanfang and CNKI databases.All the published original articles (case-control studies) were reviewed with the deadline May 31,2013,and the languages were limited to English and Chinese.Included reports were evaluated based on Newcastle-Ottawa Scale (NOS) score.The potential influence of IL-1α-889TT gene,IL-1β-3962C,IL-1β-511T and IL-1Ra-2 polymorphisms on Beh(c)et disease were analyzed.RevMan5.0,the Cochrane collaboration software program,was used to prepare and complete this review.The effect size was assessed using fixed effect model in the index with lower heterogeneity (I2 ＜50％) or randomized effect model in the index with higher heterogeneity (I2＞50％).Results The literature search resulted a total of 370 cases-controlled studies,and 7 studies met the included criteria,with the NOS scores ≥ 8.The total patients were 499 and normal controls were 708.Meta-analysis was performed on several populations.Overall,the polymorphisms of IL-1β-3962C allele increased the susciptibility of Beh(c)et disease (OR =1.41,95％ CI:1.06-1.88,P =0.02),and IL-1α-889TT genotype reduced the risk of Beh(c)et disease (OR =0.61,95 ％ CI:0.40-0.92,P =0.02).However,There was no significant association between variants of IL-1β-511T allele (OR =0.84,95％ CI:0.58-1.23,P=0.38) or IL-1RA-2 allele (OR =1.25,95％ CI:0.50-3.14,P=0.63) and Beh(c)et disease susciptibility.Conclusions These results suggest that Beh(c)et disease is associated with the IL-1 gene polymorphisms at the locations α-889C/T and β-3962C/T.A larger sample size clinical data still are need to confirm this conclusion.
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Objective To explore the key points of diagnosis and treatment of Behcet's disease with venous sinus thrombosis.Methods The diagnosis and treatment of a 25 year-old female patient with recurrent headaches was analyzed and discussed.Results Her headache was due to Behcet's disease with venous sinus thrombosis and her symptoms were gradually relieved.Conclusion Physician should be asked about the history in patients with persistent headache and the correct diagnosis can be made by MRI.
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· AIM: To investigate the expression and the possible implication of CD40/CD40L costimulatory molecules in erythema nodosum of patients with Beh(c)et's disease.· METHODS: Sampling was done from erythema nodosum of 5 patients with Beh(c)et's disease and normal skin of 2 healthy individuals. Immunohistochemical staining was performed to examine the expression of CD4, CD8, CD19, CD68, HLA-DR,CD40 and CD40L molecules in the obtained tissues.· RESULTS: Approximately 90% of epidermic cells in erythema nodosum expressed CD40 molecule. In the dermis and subcutaneous tissue, a significantly increased number of CD4+Tcells, CD8+Tcells, CD19+cells, CD68+cells, HLA-DR+cells,CD40L+cells, and CD40+cells were observed in the erythema nodosum as compared with that in normal skin. Double staining showed that CD40L molecules were expressed on 45% of CD4+T cells. CD40 molecules were expressed on 100% CD68+ cells and 59.2% of HLA-DR+cells respectively.· CONCLUSION: A number of CD40/CD40L costimulatory molecules are upreguiated in the erythema nodosum of patients with Behcet's disease.