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 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
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
<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
Behçet’s disease (BD) is a multi-systemic vasculitis characterized by a triad made up of oral aphthosis, genital aphthosis and uveitis which may or may not be associated with visceral involvement. Other times de...Behçet’s disease (BD) is a multi-systemic vasculitis characterized by a triad made up of oral aphthosis, genital aphthosis and uveitis which may or may not be associated with visceral involvement. Other times described in the ancient silk road, BD is increasingly found in countries south of the Sahara with the mixing of populations. Our objective was to describe the epidemiology and clinical characteristics of BD in an African country. Methodology: This was a five-year retrospective study in the internal medicine department of Donka National Hospital. We used international criteria finding BD. Urinary dipstick was use for finding kidney disease. Result: During the period, five cases of Behçet’s disease were collected. The average age was 35 years old. The male sex was more represented with 3 out of 5 cases. The most common clinical manifestation was oral aphthosis in all patients (100%), followed by genital aphthosis in 4 cases (80%). The other clinical manifestations observed were uveitis in 3 cases (60%), joint manifestations such as arthralgia in 3 cases (60%) and neurological manifestations such as chronic headache in one case (20%). HLA-B51 was found in two cases. The pattergic test done in two patients came back positive (100%). The patients received colchicine and oral corticosteroid therapy. Two patients were lost to sight. We deplored a death probably in a neuro-Behçet table before the rebellious headaches. Conclusion: Behçet’s disease is a multi-systemic vasculitis that must be evoked and managed quickly in a tropical environment, especially since the prognosis, vital and functional prognosis can be engaged.展开更多
Autoimmune diseases are complex clinical conditions that present significant therapeutic challenges due to their intricate immunological mechanisms.Conventional treatment strategies,such as immunosuppressive drugs and...Autoimmune diseases are complex clinical conditions that present significant therapeutic challenges due to their intricate immunological mechanisms.Conventional treatment strategies,such as immunosuppressive drugs and anti-inflammatory therapies,often demonstrate limited efficacy and are associated with considerable side effects.Recently,mesenchymal stem cells(MSCs)have attracted growing interest as a promising therapeutic approach,owing to their immunomodulatory properties and ability to promote tissue repair.However,the direct application of MSCs faces several limitations,including the risk of immunogenicity and difficulties in large-scale production.In this context MSC-derived exosomes(MSC-Exos),nano-sized extracellular vesicles secreted by MSCs,have emerged as a compelling alternative to cell-based therapies.Enriched with proteins,lipids,and nucleic acids,these exosomes exhibit potent anti-inflammatory and immunomodulatory effects.Their primary mechanisms of action include enhancing the population of regulatory T cells,modulating macrophage polarization,and suppressing proinflammatory cytokines such as interleukin-6 and tumor necrosis factor-α.The therapeutic potential of MSC-Exos extends beyond individual conditions,encompassing a wide range of autoimmune diseases.For instance in Behçet’s disease,they have been shown to regulate vasculitis and inflammatory processes by inhibiting proinflammatory cytokines and promoting endothelial cell regeneration.Moreover,MSC-Exos have demonstrated promising immunomodulatory effects in other autoimmune diseases,including systemic lupus erythematosus,rheumatoid arthritis,and multiple sclerosis.Through mechanisms such as inflammation suppression,vascular repair,and the restoration of immune homeostasis,MSC-Exos represent a versatile and innovative approach to autoimmune disease therapy.This review explored the molecular and therapeutic effects of MSCs and MSC-Exos in autoimmune diseases,with particular emphasis on their clinical potential in Behçet’s disease,systemic lupus erythematosus,rheumatoid arthritis,and multiple sclerosis.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.
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<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Behçet’s disease (BD) is a multi-systemic vasculitis characterized by a triad made up of oral aphthosis, genital aphthosis and uveitis which may or may not be associated with visceral involvement. Other times described in the ancient silk road, BD is increasingly found in countries south of the Sahara with the mixing of populations. Our objective was to describe the epidemiology and clinical characteristics of BD in an African country. Methodology: This was a five-year retrospective study in the internal medicine department of Donka National Hospital. We used international criteria finding BD. Urinary dipstick was use for finding kidney disease. Result: During the period, five cases of Behçet’s disease were collected. The average age was 35 years old. The male sex was more represented with 3 out of 5 cases. The most common clinical manifestation was oral aphthosis in all patients (100%), followed by genital aphthosis in 4 cases (80%). The other clinical manifestations observed were uveitis in 3 cases (60%), joint manifestations such as arthralgia in 3 cases (60%) and neurological manifestations such as chronic headache in one case (20%). HLA-B51 was found in two cases. The pattergic test done in two patients came back positive (100%). The patients received colchicine and oral corticosteroid therapy. Two patients were lost to sight. We deplored a death probably in a neuro-Behçet table before the rebellious headaches. Conclusion: Behçet’s disease is a multi-systemic vasculitis that must be evoked and managed quickly in a tropical environment, especially since the prognosis, vital and functional prognosis can be engaged.
文摘Autoimmune diseases are complex clinical conditions that present significant therapeutic challenges due to their intricate immunological mechanisms.Conventional treatment strategies,such as immunosuppressive drugs and anti-inflammatory therapies,often demonstrate limited efficacy and are associated with considerable side effects.Recently,mesenchymal stem cells(MSCs)have attracted growing interest as a promising therapeutic approach,owing to their immunomodulatory properties and ability to promote tissue repair.However,the direct application of MSCs faces several limitations,including the risk of immunogenicity and difficulties in large-scale production.In this context MSC-derived exosomes(MSC-Exos),nano-sized extracellular vesicles secreted by MSCs,have emerged as a compelling alternative to cell-based therapies.Enriched with proteins,lipids,and nucleic acids,these exosomes exhibit potent anti-inflammatory and immunomodulatory effects.Their primary mechanisms of action include enhancing the population of regulatory T cells,modulating macrophage polarization,and suppressing proinflammatory cytokines such as interleukin-6 and tumor necrosis factor-α.The therapeutic potential of MSC-Exos extends beyond individual conditions,encompassing a wide range of autoimmune diseases.For instance in Behçet’s disease,they have been shown to regulate vasculitis and inflammatory processes by inhibiting proinflammatory cytokines and promoting endothelial cell regeneration.Moreover,MSC-Exos have demonstrated promising immunomodulatory effects in other autoimmune diseases,including systemic lupus erythematosus,rheumatoid arthritis,and multiple sclerosis.Through mechanisms such as inflammation suppression,vascular repair,and the restoration of immune homeostasis,MSC-Exos represent a versatile and innovative approach to autoimmune disease therapy.This review explored the molecular and therapeutic effects of MSCs and MSC-Exos in autoimmune diseases,with particular emphasis on their clinical potential in Behçet’s disease,systemic lupus erythematosus,rheumatoid arthritis,and multiple sclerosis.
