ANCA, Anti-neutrophil Cytoplasmic Antibody

ANCA,抗中性粒细胞胞浆抗体
  • 文章类型: Case Reports
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  • 文章类型: Journal Article
    嗜酸性心肌炎是一种炎性心肌病,其特征是嗜酸性粒细胞浸润到心肌组织中。由于心内膜活检的临床局限性,很难确定准确的心肌炎发生率。嗜酸性心肌炎的主要发病机制是嗜酸性粒细胞释放相关物质,导致细胞膜损伤和细胞破坏。然而,证据表明,特定基因在心肌炎的发展中起作用。随着CMR映像可用性的提高,嗜酸性心肌炎的诊断率会增加。心肌炎的诊断主要取决于心内膜活检。糖皮质激素可以缓解患者的症状,但是早期使用类固醇可能会阻止中间疾病阶段的发展(即,血栓坏死和纤维化与壁血栓形成)。抗凝治疗也可能影响疾病的发展。除了常规随访,出院患者应考虑定期进行心肌活检,如果可能的话。
    Eosinophilic myocarditis is a type of inflammatory cardiomyopathy characterized by eosinophilic infiltration into myocardial tissue. The accurate myocarditis incidence rate is difficult to determine because of the clinical limitations of an endomyocardial biopsy. The primary pathogenesis of eosinophilic myocarditis is the release of related substances by eosinophils, leading to cell membrane damage and cell destruction. However, evidence suggests that specific genes play a role in myocarditis development.As CMR imaging availability increases, the diagnosis rate of eosinophilic myocarditis will increase. The diagnosis of myocarditis mainly depends on an endocardial biopsy. Glucocorticoids can relieve patients\' symptoms, but the early use of steroids may prevent intermediate disease stage development (i.e., thrombonecrosis and fibrosis with wall thrombosis). Anticoagulant therapy may also affect disease development. In addition to routine follow-up, a regular myocardial biopsy should be considered for discharged patients, if possible.
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  • 文章类型: Case Reports
    我们介绍了一例二尖瓣肉芽肿性心内膜炎导致肉芽肿性血管炎引起严重瓣膜狭窄的患者。心内膜炎是肉芽肿性多血管炎的罕见并发症,可能被误诊为感染性心内膜炎或,就像我们的情况一样,血栓性病变.(难度等级:中级。).
    We present the case of a patient with granulomatous endocarditis of the mitral valve leading to severe valve stenosis caused by granulomatosis with polyangiitis. Endocarditis is a rare complication of granulomatosis with polyangiitis that may be misdiagnosed as infectious endocarditis or, as in our case, thrombotic lesions. (Level of Difficulty: Intermediate.).
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  • 文章类型: Case Reports
    抗中性粒细胞胞浆抗体(ANCA)相关血管炎(AAV)构成了一组自身免疫起源的血管炎症疾病。髓过氧化物酶(MPO)ANCA与ANCA相关的AAV密切相关。MPO-ANCA阳性AAV患者的肺部受累率很高;然而,只有少数报道的机化性肺炎(OP)病例。尽管经过了整整一个月的抗生素治疗,但由于38°C的发烧,一名78岁的男子被送往我们医院。胸部计算机断层扫描图像显示右肺中叶和左肺上叶可见限制性巩固,这建议了一个OP模式。MPO-ANCA和尿潜血试验阳性。经支气管活检的组织病理学检查显示OP和粘液栓。肾活检的组织学发现显示与AAV有关的坏死性肾小球肾炎。患者被诊断为MPO-ANCA阳性AAV,并接受全身性皮质类固醇治疗,他很快康复了。因此,诊断OP时,应通过订购患者血清ANCA和隐匿性血尿试验来考虑AAV的可能性。
    Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) constitutes a group of blood vessel inflammation diseases of autoimmune origin. Myeloperoxidase (MPO) ANCA is closely related to ANCA associated AAV. The MPO-ANCA positive AAV patients have lung involvement at high rates; however, there are only a few reported cases with organizing pneumonia (OP). A 78-year-old man was presented to our hospital due to a fever of 38 °C despite a whole month of antibiotics treatment. Chest computed tomography image revealed restricted consolidations visible in the middle lobe of the right lung and the upper lobe of the left lung, which suggested an OP pattern. MPO-ANCA and urine occult blood tests were positive. Histopathological examination of the transbronchial biopsy revealed OP and mucus plug. Histological findings on renal biopsy showed necrotizing glomerulonephritis related to AAV. The patient was diagnosed with MPO-ANCA positive AAV and was treated with systemic corticosteroid therapy, from which he recovered rapidly. Thus, when diagnosing OP, the possibility of AAV should be considered by ordering patients\' serum ANCA and occult hematuria tests.
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  • 文章类型: Journal Article
    自身免疫性肝炎(AIH)的急性加重对诊断提出了重大挑战,因为它可以模拟急性病毒性肝炎,尤其是在没有自身抗体和高丙种球蛋白血症的情况下。
    为了确定临床,实验室,AIH急性加重患者的组织病理学特征和治疗反应。
    对8年(2008-2016年)诊断为AIH急性加重的16例患者的回顾性分析。
    在111名诊断为AIH的患者中,16例(14.4%)患者被诊断为AIH急性加重.所有患者均为女性,中位年龄为35岁。9名患者(56%)患有1型AIH,7名(44%)患者被诊断为血清阴性AIH。所有16例(100%)患者在就诊时都有急性病毒性肝炎样疾病。胆红素中位数为4.2mg/dl(范围,2.2-20),天冬氨酸转氨酶为568IU/L(范围,390-908),丙氨酸转氨酶为430IU/L(范围,257-1026)和血清碱性磷酸酶为395IU/L(范围,112-890)在症状期。组织病理学检查显示10例(71.4%)患者有潜在的慢性肝炎,只有2例(14.2)患者的纤维化和2例(14.2%)的肝硬化。所有16例(100%)患者均接受了类固醇和硫唑嘌呤的联合治疗。13例(81%)患者实现了生化完全缓解,3例(19%)患者实现了部分缓解,其中1例(6%)患者因肝硬化并发症而死于疾病。
    在没有阳性病毒标志物的情况下,模拟急性病毒性肝炎的无法解释的急性肝炎患者应考虑AIH的急性加重。在这种情况下,通过免疫血清学标志物和肝活检的评估可以诊断AIH急性加重。
    UNASSIGNED: Acute exacerbation of Autoimmune Hepatitis (AIH) poses a significant challenge for diagnosis as it can mimic acute viral hepatitis especially in absence of autoantibodies and hypergammaglobulinemia.
    UNASSIGNED: To determine the clinical, laboratory, histopathological characteristics and response to treatment in AIH patients with acute exacerbation.
    UNASSIGNED: A retrospective analysis of 16 patients with acute exacerbation of AIH diagnosed over a period of eight years (2008-2016).
    UNASSIGNED: Out of the 111 patients diagnosed with AIH, acute exacerbation of AIH was diagnosed in 16 (14.4%) patients. All patients were females with median age of 35 years. Nine patients (56%) had Type 1 AIH and seven (44%) patients were diagnosed with seronegative AIH. All 16 (100%) patients had acute viral hepatitis like illness at presentation. The median bilirubin was 4.2 mg/dl (range, 2.2-20), aspartate transaminase was 568 IU/L (range, 390-908), alanine transaminase was 430 IU/L (range, 257-1026) and serum alkaline phosphatase was 395 IU/L (range, 112-890) during symptomatic period. The histopathological examination showed underlying chronic hepatitis in 10 (71.4%) patients, only fibrosis in 2 (14.2) patients and cirrhosis with activity in 2 (14.2%). All 16 (100%) patients were treated with a combination of steroids and azathioprine. Thirteen (81%) patients achieved complete biochemical remission and three (19%) patients achieved partial remission out of which one (6%) patient succumbed to illness because of the complications of cirrhosis.
    UNASSIGNED: A suspicion of acute exacerbation of AIH should be considered in patients with unexplained acute hepatitis mimicking acute viral hepatitis in the absence of positive viral markers. Through evaluation with immunoserological markers and liver biopsy can clinch the diagnosis of acute exacerbation of AIH in such cases.
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  • 文章类型: Case Reports
    抗中性粒细胞胞浆抗体(ANCA)相关血管炎(AAV)在儿童中很少见,其特征是主要影响中小型血管的坏死性血管炎。丙硫氧嘧啶(PTU),抗甲状腺药物,与药物诱导的AAV有关。相比之下,川崎病(KD)是一种常见的系统性血管炎,通常在儿童中观察到,这影响了中型船只,包括冠状动脉.描述了一名11岁女孩,她在接受PTU治疗Graves病的同时发展为AAV。她在有2天的发烧病史后入院,宫颈腺病,唇炎和丘疹,3周后增加PTU剂量。尽管停止了PTU和静脉抗生素治疗,她的临床病情恶化,在接下来的两天里,她出现了严重的腹泻,结膜注射和右侧食指肿胀和发红。其他发现包括肝功能障碍,胆囊积水,凝血病和尿液异常,提示肾小球肾炎.她符合KD的诊断标准,并接受了静脉免疫球蛋白(IVIG)联合泼尼松龙,与临床和实验室参数的快速分辨率。在入院的第12天,右食指的剥离变得明显。连续超声心动图显示没有心脏受累的证据。入院时在患者血清中检测到高滴度的髓过氧化物酶ANCA,在恢复期滴度下降。此病例表明,PTU相关AAV的儿童可能具有模仿KD的临床特征,IVIG联合皮质类固醇治疗可有效治疗药物诱导的严重系统性AAV患者。
    Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is rare in children and is characterised as necrotising vasculitis predominantly affecting small and medium-sized vessels. Propylthiouracil (PTU), an antithyroid drug, has been implicated in drug-induced AAV. In contrast, Kawasaki disease (KD) is a common systemic vasculitis, typically observed in children, which affects the medium-sized vessels, including the coronary arteries. An 11-year-old girl who developed AAV while receiving PTU therapy for Graves\' disease is described. She was admitted to hospital following a 2-day history of fever, cervical adenopathy, cheilitis and papular rash, 3 weeks after an increase in the PTU dose. Despite discontinuation of PTU and the administration of intravenous antibiotic therapy, her clinical condition deteriorated and over the next 2 days she developed severe diarrhoea, conjunctival injection and swelling and redness of the right index finger. Additional findings included liver dysfunction, hydrops of the gallbladder, coagulopathy and urine abnormalities, suggesting glomerulonephritis. She met the diagnostic criteria for KD and received intravenous immunoglobulin (IVIG) combined with prednisolone, with rapid resolution of clinical and laboratory parameters. Peeling of the right index fingertip became evident on Day 12 of admission. Serial ultrasound cardiography demonstrated no evidence of cardiac involvement. A high titre of myeloperoxidase ANCA was detected in the patient\'s serum on admission, and the titre decreased during the convalescent stage. This case demonstrates that children with PTU-associated AAV may present with clinical features mimicking KD, and that IVIG along with corticosteroid therapy may be effective in treating patients with drug-induced severe systemic AAV.
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  • 文章类型: Case Reports
    荚膜组织支原体是世界上最常见的地方性真菌病。尽管全球大多数最大的城市枢纽都不在这种生物的流行区域之外,然而,在纽约市或洛杉矶等大都市执业的临床医生必须对组织胞浆菌病保持警惕,因为其医院服务的移民人口众多。H.荚膜感染的范围从无症状的肺部感染到具有扩散的危及生命的弥漫性肺炎。艾滋病流行的早期首次将在以前不熟悉组织胞浆菌病的地区工作的美国临床医生介绍给新的免疫功能低下的患者,这些患者来自最初在本国获得的播散性荚膜H.艾滋病毒预防和治疗方面的改进减少了此类病例的发生频率。在此,我们报告了过去三年在纽约市机构中遇到的三例组织胞浆菌病病例,以强调一线城市临床医生必须意识到这种感染。
    Histoplasma capsulatum is the most common endemic mycosis worldwide. Although most of the globe\'s largest urban hubs fall outside this organism\'s regions of endemicity, clinicians practicing in a metropolis like New York City or Los Angeles must nevertheless remain vigilant for histoplasmosis because of the large immigrant population that is served by its hospitals. H. capsulatum infection ranges from asymptomatic pulmonary infection to life-threatening diffuse pneumonia with dissemination. The early years of the AIDS epidemic first introduced U.S. clinicians working in areas previously unfamiliar with histoplasmosis to newly immunocompromised patients from endemic regions presenting with disseminated H. capsulatum originally acquired in their home countries. Improvement in HIV prevention and therapeutics has reduced the frequency of such cases. Herein we report three cases of histoplasmosis encountered in our New York City institution over the last three years to emphasize that awareness of this infection remains mandatory for the frontline urban clinician.
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  • 文章类型: Journal Article
    Progressive narrowing of the central airways due to diffuse inflammation is a potential life-threatening condition. A number of diseases have been described as possible causes. We present two siblings with severe central airway obstruction. Despite considerable efforts we have not been able to match the clinical appearance of our patients with the diagnostic criteria of any of the disease entities known to cause this condition.
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