Diagnostic dilemma between intestinal Behet disease and inflammatory bowel disease with pyoderma gangrenosum

TO THE EDITOR I have read with great interest the very recent article rifled ＂Intestinal Behcet＇s disease with pyoderma gangrenosum： A case report＂ of Nakamura T et al that was published in your journal. The authors stated that they presented a very rare case of intestinal Behcet＇s disease with pyoderma gangrenosum in a 16-year old patient. However, I would like to make some important contributions and suggestions to the presented case and have a few questions to ask the authors.

Beh et's disease complicated by multiple aseptic abscesses of the liver and spleen

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.

Muscular atrophy and weakness in the lower extremities in Behçet’s disease: A case report and review of literature

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.

Right Ventricular Thrombus and Tricuspid Valve Dysfunction in a Patient with Behçet’s Disease

Background: Behçet’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. Case Presentation: We present the case of a young patient with vascular-Behçet 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. Conclusion: Intracardiac thrombosis is a rare but early manifestation of Behçet’s disease, which is difficult to diagnose and has a poor prognosis.

英夫利西单抗治疗儿童肠白塞病的疗效分析

目的分析英夫利西单抗治疗儿童肠白塞病的疗效。方法回顾性分析首都医科大学附属北京儿童医院风湿科2017年1月至2020年12月收治的10例肠白塞病患儿的临床资料,均接受英夫利西单抗治疗>3次。探讨肠白塞病患儿消化道及其他系统受累情况、激素及免疫抑制剂治疗情况、英夫利西单抗应用情况以及随访情况等。结果男性7例,女性3例,起病年龄为(8.18±3.66)岁,病程中位时间为3(1~72)个月。10例患儿均有消化道症状,其中腹泻6例、腹痛5例、血便3例、呕吐1例、腹胀1例,3例肠穿孔。消化道超声和内镜检查提示回盲部溃疡最常见。3例合并血管受累。英夫利西单抗的剂量3~5 mg/(kg·次)。英夫利西单抗治疗时间为(20.9±9.5)个月。10例患儿随访中位时间为17.5(14~54)个月。9例患儿予甲泼尼龙冲击治疗。泼尼松初始剂量为1.6~2.0 mg/(kg·d)。8例使用甲氨蝶呤,5例使用环孢素A,5例使用沙利度胺,1例使用吗替麦考酚酯。治疗后所有患儿均无临床症状,炎症指标及消化道超声无异常,病情稳定。6例停用英夫利西单抗。英夫利西单抗输注过程中无过敏反应及活动性感染。结论英夫利西单抗治疗对儿童肠白塞病是一种耐受性良好且有效的治疗方法。

肠白塞病发病机制研究进展

白塞综合征(Behcet’s syndrome,BS)是一种慢性、周期性复发的全身性血管炎疾病,其典型临床表现为反复发作的口腔与生殖器溃疡。其中,肠白塞病作为BS的一种亚型,主要以肠溃疡为临床特征。肠白塞病的临床表现多样,可能并发严重病症,从而增加死亡风险,加重经济负担,且显著降低患者的生活品质。虽然肠白塞病的发病机理尚未彻底明了,但现有研究揭示其可能与遗传素质、环境因素、免疫调节失衡及肠道微生物群的变化密切相关。对这些病因的深入理解对于提高早期诊断精准度和开发更有效治疗手段具有重大意义。当前,关于BS的病理生理学的研究已较为充分,然而,针对肠白塞病亚型的研究文献相对较少。本文将侧重对肠白塞病涉及的遗传背景、免疫机制、环境因素和肠道微生态失衡等方面的研究进展进行综述。

生物制剂治疗以心脏瓣膜病变为主要表现的白塞病8例

目的探讨生物制剂治疗以心脏瓣膜病变为主要表现的白塞病患者的疗效。方法收集北京协和医院2016年6月至2018年9月以心脏瓣膜病变为主要表现并应用生物制剂治疗的8例患者的临床资料,回顾性分析其临床表现、超声心动图资料、手术情况及应用生物制剂后的疗效。结果 8例均为男性,平均年龄(39.9±7.7)岁,平均病程(11.8±6.0)年。瓣膜病变均以主动脉瓣受累为主,超声心动图均表现为重度主动脉瓣关闭不全。8例均在围手术期应用了生物制剂,包括英夫利昔单抗、阿达木单抗和托珠单抗。8例患者共行18例次手术,其中6例手术≥2次,先后出现瓣周漏10例次,均为单纯主动脉瓣置换术后,且术前未用激素/免疫抑制剂治疗者8例次;经围手术期积极治疗(激素联合免疫抑制剂及生物制剂),改用Carbrol术、Bentall术及瓣周漏封堵后,随访平均13(6~24)个月无明显术后并发症。应用生物制剂后,8例患者激素用量显著下降[从(41.3±15.3)mg降至(14.7±3.9)] mg(P<0.05)。结论白塞病瓣膜病变诊治困难,应加强激素和免疫抑制剂治疗,注意改良手术方式,对疗效欠佳或需要尽快手术的患者加用生物制剂能快速控制炎症,尽快创造手术时机,并减少术后并发症的发生。

神经白塞综合征诊治进展

白塞综合征是一种慢性系统性血管炎,当累及神经系统时称为神经白塞综合征(NBS),目前其病因和发病机制尚未明确。NBS较为罕见,中枢神经系统受累时可分为实质型、非实质型和混合型,临床表现有头痛、发热、偏瘫、构音障碍、共济失调等。在NBS急性期磁共振成像可见T2高信号病灶,慢性期的特征表现为脑干萎缩。NBS的诊断主要依靠临床及影像学检查,实验室检查无特异性抗体,治疗则多根据临床经验及专家意见,药物多选择糖皮质激素和免疫抑制剂,预后较差。

阿普米司特在皮肤病中的应用进展

阿普米司特(apremilast)是一种口服的小分子药物,可特异性抑制磷酸二酯酶4(phosphodiesterase-4,PDE-4)的活性,通过调节免疫系统,增加细胞内环磷酸腺苷的水平,进一步调控肿瘤坏死因子和其他炎性细胞因子的表达,从而有效控制炎症反应。它被美国食品药品监督管理局(FDA)批准用于治疗银屑病、银屑病关节炎(PsA)和贝赫切特综合征(BD)口腔溃疡。最近阿普米司特已被超适应症用于治疗全身性糖皮质激素或免疫抑制剂无效的多种皮肤病,如斑秃(AA)、扁平苔藓(LP)、化脓性汗腺炎(HS)、白癜风、特应性皮炎(AD)、慢性皮肤结节病(CCS)、慢性光化性皮炎(CAD)和慢性口疮口臭。现将该药对上述疾病的临床作用进行综述,以供临床参考。