Caractéristiques de l’atteinte cardiaque au cours de la granulomatose éosinophilique avec polyangéite

Aim of the studyOur study aimed to identify the characteristics of cardiac involvement in eosinophilic granulomatosis with polyangiitis (EGPA).MethodsWe conducted a retrospective analytic study including EGPA cases diagnosed between 2000 and 2019 in an internal medicine department. Diagnosis was made according to the 1990 American College of Rheumatology criteria and the 2012 Chapel Hill Concensus.ResultsEleven EGPA cases were included, 64% of patients were female. Median age at diagnosis was 52 years [42–58]. Heart damage revealed EGPA in 55% of cases with a significant predominance of women (p = 0.015). The main cardiac manifestations were myocarditis, ischemic cardiomyopathy due to small vessel vasculitis, cardiac tamponade and intracardiac thrombus. Cardiac magnetic resonance imaging (MRI) mainly showed subendocardial hyposignal in early infusion and late enhancement in the same areas, nodular by locations, associated with impaired left ventricle function and micro-infarctions by distal vasculitis. Cardiac damage was associated to ANCA negativity in 83.3% of cases. The median Birmingham Vasculitis Activity Score version3 (BVAS v3) was 16 [10–17]. Under conventional treatment, no relapses had occurred. The median vasculitis damage index (VDI) was 2 [1–2.3] and the mortality rate was zero after a mean follow-up of 43 months.ConclusionCardiomyopathy is a frequent revealing mode of EGPA. A late onset asthma and hypereosinophilia should guide the diagnosis. As ANCA research often turns out to be negative, histological evidence is recommended in this context. The contribution of cardiac MRI in the diagnosis of EGPA remains to be defined.     

Infections at the bottom end of the gastrointestinal tract

This article concentrates on specific infections of the distal gastrointestinal (GI) tract, particularly those that are transmitted sexually, by anal intercourse. Syphilis, gonorrhoea and lymphogranuloma venereum (LGV) are age old and well known diseases, but the features may be unfamiliar to GI histopathologists. Every histopathology trainee will have seen Herpes simplex virus (HSV) infection of squamous epithelium (e.g. oesophagus, vulva and anus), but the HSV pseudotumour of the anus/perineum can present a pitfall to those who have not previously encountered this entity. Another, more recently described, entity that has a predilection for the ano-rectal region is Epstein–Barr Virus (EBV) positive mucocutaneous ulcer, and this also presents a diagnostic trap to the unwary.     

Esophageal perforation in eosinophilic esophagitis: A systematic review on clinical presentation,management and outcomes

There is evidence of an increased fragility in the inflamed esophagus of patients with eosinophilic esophagitis (EoE). We performed a systematic review on presentation, management and outcomes of and surgical interventions for esophageal perforation in these patients, by searching in the MEDLINE, Embase and Scopus databases.Of the 599 references identified, 41 full-papers and 9 abstract met the inclusion criteria. Overall, 76 esophageal perforation episodes in 70 individual patients aged between 9 and 65 years were reported. 51 patients had not been diagnosed with EoE at the time of perforation; 14 patients had an untreated disease and the remaining were non responsive to therapy. Acute or progressive pain after long-lasting dysphagia and food impaction was the most common symptom leading to diagnosis in 42 patients who presented with Boerhaave syndrome. Pushing impacted food into the stomach led to perforation in 5 cases. Eight episodes appeared after dilation. CT scans demonstrated perforation in 82.4% of patients.Conservative management (including esophageal stenting) was used in 67.1% patients. The 25 remaining patients underwent surgery. Recovery was uneventful in the vast majority of patients. No death was reported.Active inflammation due to undiagnosed or untreated EoE was present in most cases of esophageal perforation. Conservative treatment of perforation should always be considered in EoE.     

儿童嗜酸性粒细胞性胃肠炎糖皮质激素治疗的预测因素分析

目的 分析儿童嗜酸性粒细胞性胃肠炎临床特点和治疗方案的关系，探讨糖皮质激素治疗的适用情况，为儿科医师选择治疗方案提供依据。 方法 回顾性收集广州市妇女儿童医疗中心2012年1月至2020年12月收治的182例嗜酸性粒细胞性胃肠炎患儿的临床资料。根据治疗方案中是否使用糖皮质激素分为激素组和对照组，比较分析两组患儿年龄、过敏史、临床症状、实验室检查结果、内镜下表现和胃肠黏膜病理结果，对差异有统计学意义的结果进一步行logistic回归分析。 结果 182例患儿中，36例使用糖皮质激素治疗，占总人数的19.8%。激素组患儿出现血便、贫血，内镜下出现黏膜溃疡/管腔狭窄的比例，以及黏膜嗜酸性粒细胞浸润计数均明显高于对照组（n=146，P<0.05）；激素组血清白蛋白水平明显低于对照组（P<0.05）。多因素logistic回归分析结果显示，内镜下见黏膜溃疡/管腔狭窄（OR=10.830，95%CI：3.090~37.961，P<0.001）和黏膜嗜酸性粒细胞浸润计数升高（OR=0.967，95%CI：0.941~0.993，P=0.015）可提示使用糖皮质激素治疗儿童嗜酸性粒细胞性胃肠炎。 结论 内镜下见到黏膜溃疡/管腔狭窄，病理显示黏膜嗜酸性粒细胞浸润计数明显升高，均提示儿童嗜酸性粒细胞性胃肠炎的治疗方案中可采用糖皮质激素治疗。 引用格式:     

Ⅱ型固有淋巴样细胞在嗜酸性粒细胞性慢性鼻窦炎发病机制中的作用研究进展

慢性鼻窦炎(CRS)是一种常见的慢性非特异感染性鼻部疾病,病理分型主要分为Th2细胞介导的嗜酸性粒细胞性鼻窦炎(ECRS)和Th1细胞介导的非嗜酸性粒细胞性鼻窦炎(非ECRS)2种表型。欧洲国家CRS患病人群主要表现为ECRS,而随着亚洲国家的急剧工业化发展,其ECRS的比例也在急剧增加。ECRS发病机制复杂,涉及多种免疫细胞和相关因子相互作用。其治疗方法包括抗生素、激素药物治疗及手术治疗等,但仍有部分患者症状难以改善或存在复发的风险。Ⅱ型固有淋巴样细胞(ILC2s)是一种非B、非T的新型淋巴细胞,与Th2免疫应答关系密切。文章就ILC2s在ECRS发病机制中的作用进行综述,以期为ECRS的诊断和治疗提供理论参考。     

Importance of eosinophilic structures and its correlation with acid fast bacilli in fine needle aspiration smears of tubercular lymphadenitis

BackgroundThe hallmark of tuberculosis in cytological smears is the presence of epithelioid cell granulomas, necrosis and AFB. In cases, where AFB not detected, diagnosis of tuberculosis can be made by ancillary tests like PCR which requires infrastructure besides being expensive. In India, where majority of population is rural-based there is an imminent need of some morphological change in cytological smears which can point towards diagnosis of tuberculosis in absence of AFB. This study was done to assess the importance of eosinophilic structures (ES) and its correlation with presence of AFB.Material & methodsThis was a retrospective study over a period of one year. Lymph node aspirates reported as granulomatous lymphadenitis, tubercular lesion or suppurative lesion were included. All smears for each case, stained with May Grunwald Giemsa (MGG), Hematoxylin and Eosin (H&E) and ZN stain were retrieved and rescreened for the presence of eosinophilic structure, necrosis, granulomas and AFB.ResultsOur study included 256 cases. Most common age group was 21–30 years with female predominance. Cervical lymph nodes were most commonly involved. In relation to ES and AFB four cytological pictures were seen i.e ES + AFB+ (44.54%) ES + AFB- (5.46%) ES- AFB+ (14.45%) ES - AFB- (35.55%). Chi square test showed a high significant statistical association between ES and AFB(p = 0.00001).ConclusionIn developing countries with very high TB prevalence like India, where new diagnostic modalities are unaffordable by rural population, presence of ES should be included as a morphological parameter in routine cytology reporting of tubercular lesions.     

Disodium cromoglycate treatment reduces TH2 immune response and immunohistopathological features in a murine model of Eosinophilic Esophagitis

Eosinophilic esophagitis (EoE) is an emergent chronic disease of the esophagus. The immunopathological process in EoE is characterized by Th2 immune response and prominent eosinophilic influx, in response to common food allergens. The classical treatment consists of allergen elimination diet and systemic/topical corticosteroid therapy. Nevertheless, patients do not always comply to treatment, and the prolonged corticosteroid therapy can cause side effects, therefore, there is an immediate need for new therapeutic approach for EoE. Disodium cromoglicate (DSCG) is a substance broadly used in allergic asthma treatment, and a well-known mast cell activation stabilizer. However, its effect in EoE have not been evaluated yet. This study aimed to assess the effects of DSCG treatment in an EoE experimental model. Male Balb/C mice were subcutaneously sensitized for five days with OVA, and subsequently orally OVA-challenged, DSCG administration was performed between the OVA-challenges. DSCG treatment not only reduced eosinophilic and mast cell influx, as well as reduced fibrosis. In addition, tslp, GATA_3, IL-5, FoxP₃ and IL-10 mRNA expression were reduced in esophageal mucosa, associated with lower Th2 (CD3⁺CD4⁺GATA3⁺IL4⁺) and B cells (CD19⁺CD40⁺) number in peripheral lymphoid organs. In conclusion, the data demonstrate DSCG treatment was effective in reducing mast cell activation and Th2 immune response, important immunopathological EoE features. Therefore, the use of DSCG as an EoE treatment can be considered a promising therapeutic approach to treat this disease.     

17岁男童反复腹痛、腹腔积液伴嗜酸性粒细胞增多

17岁男童，因腹痛、腹泻伴嗜酸性粒细胞增多3年，加重3 d入院。3年前患儿因进食“老酸奶”后出现腹痛、腹泻，彩超示大量腹腔积液，血常规、骨髓细胞形态学检查、腹水组织学检查可见大量嗜酸性粒细胞；3 d前因腹痛、腹泻再次入院，胃肠镜检查胃角见嗜酸性粒细胞浸润，确诊为嗜酸细胞性胃肠疾病（嗜酸细胞性胃肠炎），给予糖皮质激素及饮食规避治疗后好转，随访1年未反复。对于因腹痛、腹泻等消化道症状就诊的患儿，如伴外周血嗜酸性粒细胞增多，需考虑嗜酸细胞性胃肠疾病的可能，内镜活检胃肠道组织中见嗜酸性粒细胞浸润且计数异常为诊断的关键。 ［引用格式:中国当代儿科杂志，2021，23（11）：1169-1173］