BACKGROUND In Behçet’s disease(BD),very few cases of muscular involvement have been reported previously.The natural history and therapeutic protocol for muscular involvement in BD are obscure due to the low inci...BACKGROUND In Behçet’s disease(BD),very few cases of muscular involvement have been reported previously.The natural history and therapeutic protocol for muscular involvement in BD are obscure due to the low incidence of peripheral neuropathy or myopathy in BD.The purpose of our study was to report a rare case of BD with chronic,focal forms of neuromyopathy and review the relevant literature.CASE SUMMARY We herein report the case of a 54-year-old man who presented with progressive muscular atrophy and weakness of both thighs 2 years after the presentation of the cardinal symptoms of BD.The past medical history,electrophysiological study,neurological examination,blood tests,magnetic resonance imaging study,and histological exam were performed for the differential diagnosis.Relevant literature on muscular involvement in BD was reviewed.Neurological examination revealed that muscular involvement was predominantly localized in the proximal parts of the lower extremities.Heterogeneous enhancement of several thigh muscles was observed on magnetic resonance imaging,which corresponded with the clinical manifestations.Histological study of one of the enhanced muscles showed denervation atrophy of the muscle with superimposed myopathic changes,while electrophysiological studies only suggested denervation.CONCLUSION To our knowledge,this is the first case of neurogenic muscular atrophy with a specific set of clinical,radiological,electrophysiological,and histological findings reported in BD.展开更多
BACKGROUND Gastrointestinal involvement in Behçet's disease(GIBD)and Crohn’s disease(CD)are inflammatory diseases sharing a considerable number of similarities.However,different from CD,the operative and pos...BACKGROUND Gastrointestinal involvement in Behçet's disease(GIBD)and Crohn’s disease(CD)are inflammatory diseases sharing a considerable number of similarities.However,different from CD,the operative and postoperative management of GIBD remains largely empirical because of the lack of comprehensive treatment guidelines.AIM To compare surgical patients with GIBD and those with CD in a medical center and identify notable clinical features and effective postoperative treatment for surgical patients with GIBD.METHODS We searched patients diagnosed with CD and GIBD who underwent operations for gastrointestinal complications from 2009 to 2015 at West China Hospital of Sichuan University.A total of 10 surgical patients with GIBD and 106 surgical patients with CD were recruited.Information including demographic data,medication,and operative and postoperative parameters were collected and analyzed.As the incidence of surgical GIBD is low,their detailed medical records were reviewed and compared to previous studies.Moreover,the prognoses of CD and GIBD were evaluated respectively between groups treated with biological and non-biological agents.RESULTS Indication for first surgery was often acute intestinal perforation for GIBDpatients (7/10 vs 0/106, P < 0.001), whereas intestinal fistulae (0/10 vs 44/106, P =0.013) and ileus (0/10 vs 40/106, P = 0.015) were the indications for surgical CDpatients. Approximately 40% of patients with GIBD and 23.6% of patients withCD developed postoperative complications, 50% of patients with GIBD and 38.7%of patients with CD had recurrence postoperatively, and 40% (4/10) of patientswith GIBD and 26.4% (28/106) of patients with CD underwent reoperations. Theaverage period of postoperative recurrence was 7.87 mo in patients with Behçet'sdisease (BD) and 10.43 mo in patients with CD, whereas the mean duration fromfirst surgery to reoperation was 5.75 mo in BD patients and 18.04 mo in CDpatients. Surgical patients with GIBD more often used corticosteroids (6/10 vs7/106, P < 0.001) and thalidomide (7/10 vs 9/106, P < 0.001) postoperatively,whereas surgical patients with CD often used infliximab (27/106), azathioprine,or 6-mercaptopurine (74/106) for maintenance therapy.CONCLUSIONPatients suffering GIBD require surgery mostly under emergency situations,which may be more susceptible to recurrence and reoperation and need moreaggressive postoperative treatment than patients with CD.展开更多
BACKGROUND Splenic rupture associated with Behçet’s syndrome(BS)is extremely rare,and there is no consensus on its management.In this case report,a patient with BSassociated splenic rupture was successfully trea...BACKGROUND Splenic rupture associated with Behçet’s syndrome(BS)is extremely rare,and there is no consensus on its management.In this case report,a patient with BSassociated splenic rupture was successfully treated with splenic artery embolization(SAE)and had a good prognosis after the intervention.CASE SUMMARY The patient was admitted for pain in the left upper abdominal quadrant.He was diagnosed with splenic rupture.Multiple oral and genital aphthous ulcers were observed,and acne scars were found on his back.He had a 2-year history of BS diagnosis,with symptoms of oral and genital ulcers.At that time,he was treated with oral corticosteroids for 1 month,but the symptoms did not alleviate.He underwent SAE to treat the rupture.On the first day after SAE,the patient reported a complete resolution of abdominal pain and was discharged 5 d later.Three months after the intervention,a computed tomography examination showed that the splenic hematoma had formed a stable cystic effusion,suggesting a good prognosis.CONCLUSION SAE might be a good choice for BS-associated splenic rupture based on good surgical practice and material selection.展开更多
<strong>Background:</strong> Beh<span style="white-space:nowrap;">ç</span>et’s disease (BD) is a multisystemic, chronic inflammatory disorder with a broad range of manifes...<strong>Background:</strong> Beh<span style="white-space:nowrap;">ç</span>et’s disease (BD) is a multisystemic, chronic inflammatory disorder with a broad range of manifestations including within the cardiovascular system. Cardiac involvement like intracardiac thrombus (ICT) and valvular involvement in BD are rarely seen entities and often associated with poor prognosis. <strong>Case Presentation: </strong>We present the case of a young patient with vascular-Beh<span style="white-space:nowrap;">ç</span>et diagnosed by the presence of intracardiac thrombus in the right ventricle, unresponsive to medical treatment. Even though the intracardiac lesion was successfully treated by surgical excision, he presented a severe tricuspid dysfunction some years later due to the extension of fibrosis with no surgical therapeutic option. <strong>Conclusion:</strong> Intracardiac thrombosis is a rare but early manifestation of Beh<span style="white-space:nowrap;">ç</span>et’s disease, which is difficult to diagnose and has a poor prognosis.展开更多
Aseptic abscesses are an emergent entity and have been described in inflammatory bowel disease,especially in Crohn's disease,and in other diseases.However,aseptic abscesses associated with Beh et's disease are...Aseptic abscesses are an emergent entity and have been described in inflammatory bowel disease,especially in Crohn's disease,and in other diseases.However,aseptic abscesses associated with Beh et's disease are extremely rare.We report a Japanese male diagnosed with an incomplete type of Beh et's disease who developed multiple aseptic abscesses of the spleen and liver.In 2002,the spleen abscesses were accompanied by paroxysmal oral aphthous ulcers and erythema nodosum.As the patient's response to antibiotic treatment was inadequate,a splenectomy was performed.Severe inflammatory cell infiltration,largely of polymorphonuclear neutrophils,was observed without evidence of bacterial or fungal growth.Although the patient had no history of ocular symptoms or genital ulcers,a diagnosis of incomplete Beh et's disease was made according to the Japanese diagnostic criteria because of the presence of paroxysmal arthritis and epididymitis since 2002.In 2005,multiple liver abscesses developed with right hypochondrial pain and seemed to be attributed to Beh et's disease because the abscesses yielded negative results during a microbiologic investigation and failed to go into remission under antibiotic therapy.Oral prednisone(15 mg/d) was started in May 2006,and the abscesses dramatically disappeared 4 wk after treatment.Although the patient had a relapse of the liver abscesses in association with the tapering of prednisone,the augmentation of prednisone dosage yielded a response.The abscesses of the liver and spleen were strongly suggested to be attributed to Beh et's disease.Clinician should be aware of the existence of aseptic abscesses as uncommon manifestations of Beh et's disease.展开更多
Behet’s disease(BD)is a chronic inflammatory disease affecting multiple organ systems,such as the skin,joints,blood vessels,central nervous system,and gastrointestinal tract.Intestinal BD is characterized by intest...Behet’s disease(BD)is a chronic inflammatory disease affecting multiple organ systems,such as the skin,joints,blood vessels,central nervous system,and gastrointestinal tract.Intestinal BD is characterized by intestinal ulcerations and gastrointestinal symptoms.The medical treatment of intestinal BD includes corticosteroids and immunosupressants.There have been several reports of tumor necrosis factor-α (TNF-α)blockers being successful in treatment of refractory intestinal BD.Here,we report on a patient who was diagnosed with intestinal BD despite treatment with the fully humanized TNF-α blocker(adalimumab)for underlying ankylosing spondylitis.This patient achieved clinical remission and complete mucosal healing through the addition of a steroid and azathioprine to the adalimumab regimen.展开更多
Behet's disease is a chronic, relapsing, systemic vasculitis of unknown aetiology. Patients present manifestations of gastrointestinal complications, including mouth lesions, small and large intestinal lesions, ...Behet's disease is a chronic, relapsing, systemic vasculitis of unknown aetiology. Patients present manifestations of gastrointestinal complications, including mouth lesions, small and large intestinal lesions, and vascular lesions in the abdomen. In some cases, the intestinal ulcers of patients with Behet's disease are indistinguishable from those of Crohn's disease, tuberculosis, vasculitis and other diseases. In this article, we present a case of atypical Behet's disease with a complicated medical history and multisystem damage, for the purpose of better management of this disease.展开更多
Purpose: To analyze the clinic manifestation and prognosis of Behet disease. Method: Twenty patients requiring inpatient treatment with Beh?觭et disease were retrosp-ectively analyzed. Results: The morbidity of Beh?...Purpose: To analyze the clinic manifestation and prognosis of Behet disease. Method: Twenty patients requiring inpatient treatment with Beh?觭et disease were retrosp-ectively analyzed. Results: The morbidity of Beh?觭et disease is 5.5/100 000. In the systemic damage, stom-atocace and skin lesion are 95%, eye lesion and genital ulcer 50%, joint lesion 45%, gastrointestinal lesion 35%, Uveitis is the major disease in eye lesion, and followed in order by retinal vasculitis and obstruction of retinal artery. Attack age average 30.3 years old. Diagnosis age average 34.8 years old. The patients stay in hospital for 41 days on the average. Cure rate is 55%, improvement rate 40%, blinding rate of eye lesion is 36%. Conclusions: Beh?觭et disease is a multisystem lesion disease. Stomatocace and skin lesion is the major lesion, the next in common occurrence are eye and genital lesions. Repeated attack of uveitis, complicated cataract and secondary glaucoma are the major causes of blindness.展开更多
Introduction:Behçet disease is a chronic multisystem vasculitis disease,however generalized polymorphous cutaneous lesions are uncommon.Here,we reported a case of Behçet disease with multiple parts of the bo...Introduction:Behçet disease is a chronic multisystem vasculitis disease,however generalized polymorphous cutaneous lesions are uncommon.Here,we reported a case of Behçet disease with multiple parts of the body and complex lesions,which may lead to misjudging in clinical diagnosis.Case report:A 69-year-old man presented with a seven-year history of recurrent generalized polymorphous mucocutaneous lesions(erythematous papules,nodules,ulcers,and necrosis)over his entire body,and the lesions had been painful for the past three years.Based on the past medical history,clinical presentation,histological examination excluded other diseases,and the 2014-amended International Criteria for Behget disease,the patient had a score of 6 points and was diagnosed as Behçet disease.Discussion:The common clinical feature in patients with Behçet syndrome is the presence of recurrent and usually painful mucocutaneous ulcers.Other clinical manifestations of this disorder are more variable among different patients.A diagnosis of generalized polymorphous cutaneous lesions should remain on the list of differential diagnosis of Behçet disease after excluding other diseases.Conclusion:The mucocutaneous lesions of Behçet disease are often preceded by other manifestations,and timely diagnosis may benefit early treatment and prognosis.展开更多
目的系统评价白塞病(BD)患者血浆同型半胱氨酸水平与动静脉血栓形成间的关联性。方法检索1950年1月至2014年10月Pubmed、EMBASE、Cochrane Library、Web of Science、CNKI等中外文数据库上所有关于BD合并动静脉血栓形成的研究。按照纳...目的系统评价白塞病(BD)患者血浆同型半胱氨酸水平与动静脉血栓形成间的关联性。方法检索1950年1月至2014年10月Pubmed、EMBASE、Cochrane Library、Web of Science、CNKI等中外文数据库上所有关于BD合并动静脉血栓形成的研究。按照纳入和排除标准纳入文献,并采用Rev Man5.2软件进行Meta分析。结果最终纳入11个研究,分析结果显示同型半胱氨酸血症在血栓组较无血栓组更普遍(比值比2.61,95%置信区间1.46~4.67,P=0.001),血栓组同型半胱氨酸的平均水平显著高于无血栓组(均数差=2.99,95%置信区间1.81~4.16,P【0.00001]。结论血浆高同型半胱氨酸增加BD患者的动静脉血栓形成风险。展开更多
文摘BACKGROUND In Behçet’s disease(BD),very few cases of muscular involvement have been reported previously.The natural history and therapeutic protocol for muscular involvement in BD are obscure due to the low incidence of peripheral neuropathy or myopathy in BD.The purpose of our study was to report a rare case of BD with chronic,focal forms of neuromyopathy and review the relevant literature.CASE SUMMARY We herein report the case of a 54-year-old man who presented with progressive muscular atrophy and weakness of both thighs 2 years after the presentation of the cardinal symptoms of BD.The past medical history,electrophysiological study,neurological examination,blood tests,magnetic resonance imaging study,and histological exam were performed for the differential diagnosis.Relevant literature on muscular involvement in BD was reviewed.Neurological examination revealed that muscular involvement was predominantly localized in the proximal parts of the lower extremities.Heterogeneous enhancement of several thigh muscles was observed on magnetic resonance imaging,which corresponded with the clinical manifestations.Histological study of one of the enhanced muscles showed denervation atrophy of the muscle with superimposed myopathic changes,while electrophysiological studies only suggested denervation.CONCLUSION To our knowledge,this is the first case of neurogenic muscular atrophy with a specific set of clinical,radiological,electrophysiological,and histological findings reported in BD.
基金National Natural Science Foundation of China,No.81270447.
文摘BACKGROUND Gastrointestinal involvement in Behçet's disease(GIBD)and Crohn’s disease(CD)are inflammatory diseases sharing a considerable number of similarities.However,different from CD,the operative and postoperative management of GIBD remains largely empirical because of the lack of comprehensive treatment guidelines.AIM To compare surgical patients with GIBD and those with CD in a medical center and identify notable clinical features and effective postoperative treatment for surgical patients with GIBD.METHODS We searched patients diagnosed with CD and GIBD who underwent operations for gastrointestinal complications from 2009 to 2015 at West China Hospital of Sichuan University.A total of 10 surgical patients with GIBD and 106 surgical patients with CD were recruited.Information including demographic data,medication,and operative and postoperative parameters were collected and analyzed.As the incidence of surgical GIBD is low,their detailed medical records were reviewed and compared to previous studies.Moreover,the prognoses of CD and GIBD were evaluated respectively between groups treated with biological and non-biological agents.RESULTS Indication for first surgery was often acute intestinal perforation for GIBDpatients (7/10 vs 0/106, P < 0.001), whereas intestinal fistulae (0/10 vs 44/106, P =0.013) and ileus (0/10 vs 40/106, P = 0.015) were the indications for surgical CDpatients. Approximately 40% of patients with GIBD and 23.6% of patients withCD developed postoperative complications, 50% of patients with GIBD and 38.7%of patients with CD had recurrence postoperatively, and 40% (4/10) of patientswith GIBD and 26.4% (28/106) of patients with CD underwent reoperations. Theaverage period of postoperative recurrence was 7.87 mo in patients with Behçet'sdisease (BD) and 10.43 mo in patients with CD, whereas the mean duration fromfirst surgery to reoperation was 5.75 mo in BD patients and 18.04 mo in CDpatients. Surgical patients with GIBD more often used corticosteroids (6/10 vs7/106, P < 0.001) and thalidomide (7/10 vs 9/106, P < 0.001) postoperatively,whereas surgical patients with CD often used infliximab (27/106), azathioprine,or 6-mercaptopurine (74/106) for maintenance therapy.CONCLUSIONPatients suffering GIBD require surgery mostly under emergency situations,which may be more susceptible to recurrence and reoperation and need moreaggressive postoperative treatment than patients with CD.
文摘BACKGROUND Splenic rupture associated with Behçet’s syndrome(BS)is extremely rare,and there is no consensus on its management.In this case report,a patient with BSassociated splenic rupture was successfully treated with splenic artery embolization(SAE)and had a good prognosis after the intervention.CASE SUMMARY The patient was admitted for pain in the left upper abdominal quadrant.He was diagnosed with splenic rupture.Multiple oral and genital aphthous ulcers were observed,and acne scars were found on his back.He had a 2-year history of BS diagnosis,with symptoms of oral and genital ulcers.At that time,he was treated with oral corticosteroids for 1 month,but the symptoms did not alleviate.He underwent SAE to treat the rupture.On the first day after SAE,the patient reported a complete resolution of abdominal pain and was discharged 5 d later.Three months after the intervention,a computed tomography examination showed that the splenic hematoma had formed a stable cystic effusion,suggesting a good prognosis.CONCLUSION SAE might be a good choice for BS-associated splenic rupture based on good surgical practice and material selection.
文摘<strong>Background:</strong> Beh<span style="white-space:nowrap;">ç</span>et’s disease (BD) is a multisystemic, chronic inflammatory disorder with a broad range of manifestations including within the cardiovascular system. Cardiac involvement like intracardiac thrombus (ICT) and valvular involvement in BD are rarely seen entities and often associated with poor prognosis. <strong>Case Presentation: </strong>We present the case of a young patient with vascular-Beh<span style="white-space:nowrap;">ç</span>et diagnosed by the presence of intracardiac thrombus in the right ventricle, unresponsive to medical treatment. Even though the intracardiac lesion was successfully treated by surgical excision, he presented a severe tricuspid dysfunction some years later due to the extension of fibrosis with no surgical therapeutic option. <strong>Conclusion:</strong> Intracardiac thrombosis is a rare but early manifestation of Beh<span style="white-space:nowrap;">ç</span>et’s disease, which is difficult to diagnose and has a poor prognosis.
文摘Aseptic abscesses are an emergent entity and have been described in inflammatory bowel disease,especially in Crohn's disease,and in other diseases.However,aseptic abscesses associated with Beh et's disease are extremely rare.We report a Japanese male diagnosed with an incomplete type of Beh et's disease who developed multiple aseptic abscesses of the spleen and liver.In 2002,the spleen abscesses were accompanied by paroxysmal oral aphthous ulcers and erythema nodosum.As the patient's response to antibiotic treatment was inadequate,a splenectomy was performed.Severe inflammatory cell infiltration,largely of polymorphonuclear neutrophils,was observed without evidence of bacterial or fungal growth.Although the patient had no history of ocular symptoms or genital ulcers,a diagnosis of incomplete Beh et's disease was made according to the Japanese diagnostic criteria because of the presence of paroxysmal arthritis and epididymitis since 2002.In 2005,multiple liver abscesses developed with right hypochondrial pain and seemed to be attributed to Beh et's disease because the abscesses yielded negative results during a microbiologic investigation and failed to go into remission under antibiotic therapy.Oral prednisone(15 mg/d) was started in May 2006,and the abscesses dramatically disappeared 4 wk after treatment.Although the patient had a relapse of the liver abscesses in association with the tapering of prednisone,the augmentation of prednisone dosage yielded a response.The abscesses of the liver and spleen were strongly suggested to be attributed to Beh et's disease.Clinician should be aware of the existence of aseptic abscesses as uncommon manifestations of Beh et's disease.
文摘Behet’s disease(BD)is a chronic inflammatory disease affecting multiple organ systems,such as the skin,joints,blood vessels,central nervous system,and gastrointestinal tract.Intestinal BD is characterized by intestinal ulcerations and gastrointestinal symptoms.The medical treatment of intestinal BD includes corticosteroids and immunosupressants.There have been several reports of tumor necrosis factor-α (TNF-α)blockers being successful in treatment of refractory intestinal BD.Here,we report on a patient who was diagnosed with intestinal BD despite treatment with the fully humanized TNF-α blocker(adalimumab)for underlying ankylosing spondylitis.This patient achieved clinical remission and complete mucosal healing through the addition of a steroid and azathioprine to the adalimumab regimen.
基金Supported by Major Foundation of Xiamen Gastroenterology Center,No.200702
文摘Behet's disease is a chronic, relapsing, systemic vasculitis of unknown aetiology. Patients present manifestations of gastrointestinal complications, including mouth lesions, small and large intestinal lesions, and vascular lesions in the abdomen. In some cases, the intestinal ulcers of patients with Behet's disease are indistinguishable from those of Crohn's disease, tuberculosis, vasculitis and other diseases. In this article, we present a case of atypical Behet's disease with a complicated medical history and multisystem damage, for the purpose of better management of this disease.
文摘Purpose: To analyze the clinic manifestation and prognosis of Behet disease. Method: Twenty patients requiring inpatient treatment with Beh?觭et disease were retrosp-ectively analyzed. Results: The morbidity of Beh?觭et disease is 5.5/100 000. In the systemic damage, stom-atocace and skin lesion are 95%, eye lesion and genital ulcer 50%, joint lesion 45%, gastrointestinal lesion 35%, Uveitis is the major disease in eye lesion, and followed in order by retinal vasculitis and obstruction of retinal artery. Attack age average 30.3 years old. Diagnosis age average 34.8 years old. The patients stay in hospital for 41 days on the average. Cure rate is 55%, improvement rate 40%, blinding rate of eye lesion is 36%. Conclusions: Beh?觭et disease is a multisystem lesion disease. Stomatocace and skin lesion is the major lesion, the next in common occurrence are eye and genital lesions. Repeated attack of uveitis, complicated cataract and secondary glaucoma are the major causes of blindness.
文摘Introduction:Behçet disease is a chronic multisystem vasculitis disease,however generalized polymorphous cutaneous lesions are uncommon.Here,we reported a case of Behçet disease with multiple parts of the body and complex lesions,which may lead to misjudging in clinical diagnosis.Case report:A 69-year-old man presented with a seven-year history of recurrent generalized polymorphous mucocutaneous lesions(erythematous papules,nodules,ulcers,and necrosis)over his entire body,and the lesions had been painful for the past three years.Based on the past medical history,clinical presentation,histological examination excluded other diseases,and the 2014-amended International Criteria for Behget disease,the patient had a score of 6 points and was diagnosed as Behçet disease.Discussion:The common clinical feature in patients with Behçet syndrome is the presence of recurrent and usually painful mucocutaneous ulcers.Other clinical manifestations of this disorder are more variable among different patients.A diagnosis of generalized polymorphous cutaneous lesions should remain on the list of differential diagnosis of Behçet disease after excluding other diseases.Conclusion:The mucocutaneous lesions of Behçet disease are often preceded by other manifestations,and timely diagnosis may benefit early treatment and prognosis.
文摘目的系统评价白塞病(BD)患者血浆同型半胱氨酸水平与动静脉血栓形成间的关联性。方法检索1950年1月至2014年10月Pubmed、EMBASE、Cochrane Library、Web of Science、CNKI等中外文数据库上所有关于BD合并动静脉血栓形成的研究。按照纳入和排除标准纳入文献,并采用Rev Man5.2软件进行Meta分析。结果最终纳入11个研究,分析结果显示同型半胱氨酸血症在血栓组较无血栓组更普遍(比值比2.61,95%置信区间1.46~4.67,P=0.001),血栓组同型半胱氨酸的平均水平显著高于无血栓组(均数差=2.99,95%置信区间1.81~4.16,P【0.00001]。结论血浆高同型半胱氨酸增加BD患者的动静脉血栓形成风险。
