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Objective This study aims to investigate the frequency and characteristics of vascular involvement in Beh(c)et's disease (BD).Methods We enrolled patients who were hospitalized at Changhai Hospital affiliated Naval Medical University and who had been diagnosed with BD at discharge from January 1999 to July 2017.Patientswere divided into two groups,Vascular Beh(c)et's disease (VBD) group and non-VBD group,according to vascular involvement or not.We recorded and compared the demographicinformation,disease activity scores Beh(c)et’s Disease Current Activity Form (BDCAF),other systemic involvement and laboratory test results between the two groups.The clinical features of vascular involvement were analyzed.The measureddata were statistically analyzed with Student's t/t’ test or Mann-Whitney U test.The numerical data were statistically analyzed with x2 test or continuity correction x2 test.Results The total numbers of BD patients were 224,including 120 males and 104 females.Vascular involvements were found in 41 (18.3％) cases,including 34 males and 7 females.VBD was more common in males (28.3％ vs 6.7％,x2=17.388,P＜0.01).Of 41 VBD patients,11 cases (26.8％) had single vascular lesions,and 30 cases (73.2％) had multiple vascular lesions.Moreover,24 cases (58.5％) had arterial lesions,and 25 cases (61.0％) had venous lesions.Eight (19.5％) patients had both arterial and venous lesions.Compared to the non-VBD group,the VBD group had a higher frequency of cardiac involvement (22.0％ vs 3.8％,x2=16.592,P＜0.01),a higher disease activity index score (BDCAF) [3.0(2.0,4.0) score vs 2.0(2.0,3.0) score,U=2 609.5,P=0.001],higher levels of C-reactiveprotein (CRP) (14.45(4.97,64.08) mg/L vs 9.02(3.16,26.53) mg/L,U=2 809,P=0.046) and plasma homocysteine (Hcy) [(17.6±7.7) μmol/L vs (7.1±2.1) μmol/L,t'=7.894,P＜0.01].Conclusion VBD mainly affectsmales.It mainly presentsas multiple lesions.Moreover,patients in the VBD group havehigher frequency of cardiac complications,higher disease activity index scores (BDCAF),higher levels of CRP and Hcy than the non-VBD group.These results may have great valueto predict and diagnoseof VBD.
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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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Objective Endothelial microparticles (EMPs) are direct indicator of endothelial cell activation or apoptosis,and may also reflect endothelial inflammation,increased coagulation,and vascular tone.The aim of this study is to investigate whether EMPs would be able to evaluate systemic involvement and be a new indicator of disease activity in Beh(c)et's disease (BD).Methods Thirty-nine consecutive BD patients (who fulfilled the modified International Study Group on BD in 1990 or International Criteria for BD in 2006) and 67 age and sex-matched healthy controls were enrolled (Including 37 patients with hypertension and 30 healthy subjects).The plasma levels of EMPs were measured by flow cytometry utilizing specific labels for endothelial MPs (CD31+ and CD42b-).The measurement data of each group were expressed as-x±s,and the comparison data betwen groups were analyzed by independent sample t test and analysis of variance,Spearman/Pearson correlation analysis,P＜0.05 was statistically significant.Results The levels of circulating EMPs (CD31 + and CI42b-) were significantly elevated in the case group compared with the healthy control group and hypertension (F=6.845,P＜0.05).Moreover,BD patients plasma EMPs were positively correlated with active BD (r=0.802,P＜0.05).Vascular involvement in BD patients was higher than in patients without vascular EMPs,t=4.707,P＜0.05.Gastrointestinal involvement in BD patients was more frequent than that in patients without Gastrointestinal involvement,t=2.673,P＜0.05.Conclusion Levels of circulating EMPs are elevatedd in BD patients and correlated with disease activity in BD.Elevated EMPs may be a potential indicator to predict disease activity of BD.The plasma level of EMPs is increased,which indicats increased risk of vascular and digestive tract involvement in BD.
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Objective To discuss the characteristics of in-patients with Beh(c)et's disease in Xinjiang Uygur Autonomous region.Methods Clinical data of 146 cases with Behcet's disease admitted to Xinjiang Uygur Autonomous region people's hospital were analyzed.The demographic data and clinical manifestations were analyzed retrospectively.The categorical data was compared using x2 test or Fisher's exact test.Results ① Of the 146 patients with BD,99 cases were Han,47 cases were uyghurs.Oral ulcer were observed in 146 cases (100％).Genital ulcer occurred in 115 cases (78.8％),of which 76 were Han (76.8％),39 (82.9％) were uyghurs,the difference was not significant (P=0.391).The initial presentation was oral ulcer in 83 cases (56.8％),of which 60 cases (60.6％) were Han,23 cases (48.9％) were the uyghurs.The second most common presentation was oral ulcer and genital ulcer,which occurred in 42 cases (28.8％),of which 23 cases (23.2％) were Han,19 cases (40.4％) were the uyghurs.More Han patients initially presented with both oral and genital ulcers than uyghur (P=0.032).② Skin lesions were recorded in 66 cases (45.2％),of which 45 cases (45.4％) were Han,21 cases (44.7％) were the uyghurs,the difference was not significant (P=0.930).③ Joint damage occurred in 40 cases (27.4％),of which 22 cases (22.2％) were Han,18 cases (38.3％) were the uyghurs.Joint damage wasmore frequently observed in Han than in uyghur (P=0.042).④ Eye lesion happened in 21 cases (14.4％),ofwhich 9 cases (9.1％) were Han,12 cases (25.5％) were the uyghurs,ie,eye lesion of Uyghur was higher than Han (P=0.008).⑤ Vascular lesions were detected in 8 cases (5.5％),of which the 5 cases were Han,3 cases were uyghurs,the difference was not significant (P=0.712).⑥ Nervous lesions were observed in 2 cases (1.37％),1 was Han and one was uyghur,the difference was not significant (P=0.542).Conclusion The most common clinical presentations of BD in Xinjiang region are oral and genitalulcers.Skin,joints,eye,vascular,nervous system can also be affected.Joint damage in Han is higher than Uyghur,but eye lesion of Uyghur is more common than Han.
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Objective To investigate the clinic manifestations of intestinal involvement of Beh(c)et's disease (BD).Methods Medical data of patients with intestinal involvement of BD admitted to Peking Union Medical College Hospital (PUMCH) from January 1994 to August 2013 were retrospectively reviewed.Clinic manifestations,laboratory tests and endoscopic characteristics were analyzed,and the influence of sex on the clinic manifestations was investigated.Numerical data and categorical data comparisons were analyzed using t-test,x2 test respectively.Results Sixty-four patients with intestinal involvement accounted for 10.5 percent (64/611) of total hospitalized BD patients during the same period.Among these 64 patients,31 were male and 33 were female.The median age at the onset of intestinal involvement was 34 years old.Gastrointestinal symptoms were presented after other systemic symptoms of BD in 91％ patients (58/64).Abdominal pain (88％,56/64) was the most common clinical manifestations of intestinal involvement,and severe complications such as gastrointestinal bleeding (31％,20/64),intestinal obstruction (22％,14/64),intestinal perforation (11％,7/64) and intestinal fistula (16％,10/64) could also occur in some patients.Lesions usually located at ileocecum portion of the gastrointestinal tract.The common endoscopic manifestation was deep ulcers.Female sex was associated with higher C reactive protein level and lower serum albumin level [(52±46) vs (27±36) mg/L,t=2.287,P=0.026; Serum albumin:(34±6) vs (37±5) mg/L,t=2.237,P=0.029].After treated with glucocorticoid,im-munosuppressant and TNF-α blockers,62 patients achieved clinical remission while 21 cases were operated.Conclusion Most of intestinal involvement of BD patients are young adults,and the gastrointestinal symp-toms usually present after the presence of other systemic symptoms.Abdominal pain is the most common manifestations,while severe complications such as gastrointestinal bleeding and intestinal obstruction can also occur.The most common endoscopic findings are deep ulcers,which often located in the ileocecal region.Female patients are more likely to have severe clinical course.Glucocorticoid,immunosup-pressant and TNF-α antagonist therapy are effective,but some patients still need surgical intervention.
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Objective To investigate the clinical characteristics of Beh(c)et's disease (BD) associated with thrombosis.Methods Medical records at Peking Union Medical College Hospital from 1993 to 2012 were reviewed to identify patients who were diagnosed as BD associated with thrombosis.Comparisons between groups were tested by t-test and x2 test.Results Seventy-six BD patients developed thrombosis which accounted for 11.5％ of 659 BD patients hospitalized during the same period.Among these 76 patients,64 were male,12 were female.The average age was (34±10) years old (range 16-66 years).The most common site of thrombosis was extremity veins (87％) including deep vein thrombosis (n=65) and superficial thrombophlebitis (n=3).The thrombosis associated with BD in a descending frequency of order was pulmonary thromboem-bolism (16 cases,21％),Venae Cavae (15 cases,20％),cerebral venous sinus (10 cases,13％),intracardiac thrombus (7 cases,9 ％),renal veins (3 cases,4％),and superior mesenteric vein (2 cases,3％).Only four patients with single site thrombosis,all the others had two or more sites of thrombosis.Extremity vein thrombosis showed a male preponderance,while cerebral venous sinus thrombosis had a female preponderance.BD patients always exhibited active disease during emergence of thrombotic events.Conclusion The incidence of thrombosis in BD patients is high and tends to occur in patients with active disease.More attention should be paid to these patients.
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Objective To investigate the clinical and pathological characteristics of renal involvement in Beh(c)et's disease (BD).Methods A retrospective analysis was carried out in BD patients complicated with renal damage who were admitted to Peking Union Medical College Hospital from June 1998 to July 2012.Results Twenty patients with renal involvement constituted 3.2％ of all the 618 hospitalized BD patients.The presentation of renal disease was chronic glomerulonephritis in 6 patients (1 with nephrotic syndrome),renal tubular acidosis in 1 patient,renal artery stenosis in 7 patients,renal vein thrombosis in 1 patient,and chronic renal failure of unknown etiology in 5 patients.Kidney biopsy was performed in 5 patients,3 of them revealed glomerular minor lesion,mild mesangial proliferative glomerulonephritis and chronic tubularinterstitial nephropathy,respectively.The other 2 patients underwent a second biopsy,one with glomerular minor lesion transforming into IgA nephropathy of grade Ⅲ on Lee's glomerular grading system after 6 years,and the other with IgA nephropathy of grade Ⅱ progressing to grade Ⅳ after 2 years.After the diagnosis of renal BD,one patient with uremia underwent peritoneal dialysis,while the remaining 19 patients received immunosuppressant (or with combination of glucocorticoid,angiotensin converting enzyme inhibitors and angiotensin Ⅱ receptor blockers.etc.) therapy.Among the 8 patients with renal vascular involvement,2 underwent surgery,and several received anticoagulant therapy.During the follow-up of 13 patients,the urine protein quantifications were reduced,and renal functions remained relatively stable.Conclusion Renal damage is relatively uncommon in BD patients.There are various clinical spectrums for renal BD.Routine screening with urinalysis,serum creatinine and imaging studies should be carried out for the early diagnosis of renal BD.