肺血管炎及其诊治概述

1肺血管炎的概念和分类血管炎系病理学名词,指以血管壁炎症和坏死为特征的病理改变。血管炎可以累及动脉、静脉和毛细血管。临床上,血管炎是指病理主要表现为血管炎的一组异质性疾病。根据血管炎的病因可将其分为原发性血管炎和继发性血管炎。病因不明的血管炎称为原发性血管炎     

抗中性粒细胞胞浆抗体与血管炎

讨论Ｈｉｌｂｅｒｔ空间上标型谱算子的基本性质。推广关于次正规算子不变子空间存在性的Ｂｒｏｗｎ定理以及关于本质正规算子本质本等价的Ｂｒｏｗｎ－Ｄｏｕｇｌａｓ－Ｆｉｌｌｍｏｒｅ定理。     

肺小血管炎的研究进展

肺小血管炎(SVVL)是原发性小血管炎累及肺脏血管的一组疾患.因本病与抗中性粒细胞胞浆抗体(ANCA)密切相关,故称为抗中性粒细胞胞浆抗体相关性小血管炎(AAVS).主要包括:韦格纳肉芽肿病(WG)、显微镜下多血管炎(MPA)、变应性肉芽肿性血管炎(CSS)[1],常可累及多个系统.肺是原发性小血管炎累及频率最高的脏器之一,据报道肺脏受累占75%,仅次于肾脏,且是造成死亡的主要原因.其临床表现复杂多变,缺乏特异性,常常被误诊为其它疾病,延误治疗.而经积极治疗,其生存率明显上升.因此有必要提高对SVVL的认识.     

呼吸系统抗中性粒细胞胞浆抗体相关性血管炎的预后和危险因素

目的评价呼吸系统抗中性粒细胞胞浆抗体(ANCA)相关性血管炎(AAV)的预后,并分析与预后相关的危险因素。方法回顾性收集45名就诊于中国医科大学呼吸疾病研究所的AVV患者的临床资料。计算患者的1年死亡率,并对可能与这一预后相关的因素进行逻辑回归分析。结果患者的1年死亡率为17.8%(8/45)。主要死亡原因为肺部感染、弥漫性肺泡出血和呼吸衰竭。年龄>65岁的患者1年内死亡风险显著增高,OR值为6.63(P=0.025,95%CI 2.56⁵5.11),显微镜下多血管炎的OR值为1.6(P=0.03,95%CI 1.0855.11),显微镜下多血管炎的OR值为1.6(P=0.03,95%CI 1.08⁴.98),呼吸衰竭的OR值为5.5(P=0.044,95%CI 1.94.98),呼吸衰竭的OR值为5.5(P=0.044,95%CI 1.9⁴5.72),急性肾衰竭的OR值为8.14(P=0.032,95%CI 3.2245.72),急性肾衰竭的OR值为8.14(P=0.032,95%CI 3.22⁶1.14)。结论即使接受积极的治疗,呼吸系统AAV患者的死亡率仍然很高,高龄、显微镜下多血管炎、呼吸衰竭和急性肾衰竭是死亡的主要危险因素。     

抗中性粒细胞胞浆抗体相关性血管炎的诊治进展

抗中性粒细胞胞浆抗体（ANCA）相关性血管炎（ANCA-associated vasculitis,AAV）是一类病因不明的慢性自身免疫性系统性疾病,病理学特征为小血管（包括小动、静脉和毛细血管）的纤维素样坏死性血管炎.AAV的共同特征为血清中可检测到ANCA,肺和肾脏累及.根据2012年Chapel Hill会议[1]（CHCC）对血管炎分类标准的定义,ANCA相关性血管炎包括以下3类：显微镜下多血管炎（MPA）;肉芽肿性多血管炎（GPA）,原名为韦格纳肉芽肿;嗜酸细胞性肉芽肿性多血管炎（EGPA）,又名为Churg-Strauss综合征.由于AAV的临床表现多样且少有特异性,在临床工作中极易被漏诊或误诊,以致延误患者病情.     

抗中性粒细胞胞浆抗体相关性血管炎的诊治进展

正华中科技大学同济医学院附属同济医院邢瑞凃巍*,武汉430030     

28例ANCA阳性肺血管炎临床分析

目的总结我科28例ANCA阳性肺血管炎的临床表现,提高对该病认识。方法回顾性分析我科2011-2015年ANCA阳性肺血管炎患者,总结其临床资料、实验室和肺部CT检查表现。结果 28例患者纳入分析,性别比例1∶1.15;平均年龄64.11±13.51岁(36~81岁);大部分患者表现为发热、咳嗽、咳痰;25例患者表现为P-ANCA阳性(89.28%);所有检测ESR的患者均有不同程度升高(21/21),96.30%的患者CRP升高(26/27);17例患者合并有肾受累表现,主要以血尿为主(17/28,60.71%);74.19%的患者有不同程度的贫血,78.57%有中性粒细胞比例升高。肺部CT提示以斑片状渗出影最常见(67.86%),其次为间质病变(28.57%),其他病变包括结节样病变(17.86%),胸腔积液(25.00%),磨玻璃样病变(7.14%)。与单纯肺受累组相比,合并肾受累时实验室检查和肺部影像学改变并无明显差异。结论 ANCA阳性肺血管炎临床和相关检测缺少特异性,诊断上需仔细甄别。     

肺血管炎误诊为肺结核5例临床分析及误诊原因探讨

目的探讨肺血管炎误诊为肺结核的原因及预防方法。方法通过对5例肺血管炎误诊为肺结核的临床分析，重点分析肺血管炎误诊原因，探讨诊治方法。结果5例肺血管炎患者均误诊为肺结核，在明确诊断前均进行了不适当的检查和治疗，延误病情，5例中有3例好转出院，2例死亡。结论应提高对肺小血管炎的认识，当患者病情经相应治疗未见好转，应及时检查血周围型抗中性粒细胞胞浆抗体（ANCA），如果有条件应积极进行开胸肺活检或通过胸腔镜活检，争取明确诊断。     

抗中性粒细胞胞浆抗体小血管炎24例临床分析

目的对我院24例抗中性粒细胞胞浆抗体小血管炎患者的临床资料进行临床分析。方法对确诊为抗中性粒细胞胞浆抗体小血管炎患者的临床表现、实验室检查、肾脏病理、诊治及预后进行分析。结果24例患者均为抗中性粒细胞胞浆抗体（ANCA）阳性,均有肾脏受累,同时还伴有肺、胃肠道、眼、耳、鼻、声带、皮肤、关节、甲状腺及外周神经受累。临床表现复杂多样,平均确诊时间为（9±4）个月。治疗以糖皮质激素加用环磷酰胺为主。总缓减率为78％。结论抗中性粒细胞胞浆抗体小血管炎为多系统受累疾病,漏诊误诊率高,早期诊治能提高生存率,但部分患者预后不佳。     

Update in the diagnosis and management of pulmonary vasculitis

The term vasculitis encompasses a number of distinct clinicopathologic disease entities, each of which is characterized pathologically by cellular inflammation and destruction of the blood vessel wall, and clinically by the types and locations of the affected vessels. While multiple classification schemes have been proposed to categorize and simplify the approach to these diseases, ultimately their diagnosis rests on the identification of particular patterns of clinical, radiologic, laboratory, and pathologic features. While lung involvement is most commonly seen with the primary idiopathic, small-vessel or antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides of Wegener granulomatosis, microscopic polyangiitis, and Churg-Strauss syndrome, one should remember that medium-vessel vasculitis (ie, classic polyarteritis nodosa), large-vessel vasculitis (ie, Takayasu arteritis), primary immune complex-mediated vasculitis (ie, Goodpasture syndrome), and secondary vasculitis (ie, systemic lupus erythematosus) can all affect the lung. However, for the purpose of this review, we will focus on the ANCA-associated vasculitides.     

Classification,diagnosis and treatment of ANCA-associated vasculitis

Diagnosis of anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis is usually not difficult in patient with systemic disease, including lung and kidneys involvement, and laboratory signs of inflammation. The presence of ANCA and the results of histological investigation confirm diagnosis of ANCA-associated vasculitis. Cyclophosphamide/azathioprine in combination with high dose steroids are used to induce and maintain remission of systemic vasculitis. The clinical trials also showed efficacy of rituximab that induces depletion of B-cells. Our understanding and management of ANCA-associated vasculitis improved significantly over the last decades but there is still a lot of debate over its classification, diagnostic criteria, assessment of activity and optimum treatment.     

The complications of vasculitis and its treatment

Survival following a diagnosis of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) has improved since the introduction of cyclophosphamide-based immunosuppressive regimens and is now almost 80% at 5 years. However, mortality remains 2.6 times greater in the population with AAV than in an age- and sex-matched general population. The greatest risk of harm for patients with AAV is during the first year of diagnosis and from the adverse events associated with treatment rather than with active vasculitis. Infection, cardiovascular disease (CVD) and malignancy are the most common causes of death during follow-up. New regimens including rituximab, although with an efficacy similar to that of cyclophosphamide, have not yet shown a clear reduction in adverse events. Therapy for AAV must currently encompass a much greater focus on preventing harm from treatment through vaccination, Pneumocystis jirovecii pneumonia (PJP) prophylaxis, CVD risk assessment and bone protection measures.     

老年肺肉芽肿性血管炎的诊治(一)

肺部肉芽肿性疾病原因很多,其中部分伴有血管炎,称为肺肉芽肿性血管炎（pulmonary granulomatous vasculitis）或肺血管炎和肉芽肿病（pulmonary angiitis and granulomatosis）,1973年由Liebow首先界定,主要包括韦格内肉芽肿（Wegener＇s granulomatosis,WG）、变应性血管炎和肉芽肿病（allergic angiitis and granulomatosis,Churg-Strauss syndrome,CSS）、     

Pulmonary embolism: diagnosis and treatment

Pulmonary embolism is a condition with a potentially high mortality that often goes unrecognized. Prompt diagnosis and treatment effectively reduce the mortality rate. Clinical judgment alone is insufficient for diagnosis; objective testing is needed. Once the diagnosis is made, effective treatment should be instituted. Treatments differ, based on several factors including disease severity, contraindications to treatment and pre-existing diseases. The major thrust of training should be to teach medical care workers how to prevent deep vein thrombosis and its most serious complication, pulmonary embolism.     

Pulmonary aspergilloma, diagnosis and treatment

Pulmonary aspergilloma is a saprophytic form of aspergillosis, and the diagnosis is usually based on radiological findings such as thickened cavitary wall and fungus ball, and on positive serum antibody. Up to 58% of the patients with aspergilloma in Japan have medical history of tuberculosis. Serum anti-Aspergillus antigen is almost always positive in aspergilloma patients but aspergillus antigen is usually negative. Massive hemoptysis can be a fatal complication of aspergilloma, and the most common complication was respiratory failure according to our study. Surgical resection is the only promising intervention to cure the aspergilloma, however, low pulmonary function does not allow operation. Antifungal treatment is chosen for those who are out of operation indication, but the efficacy of antifungal treatment against aspergilloma is controversial. Some patients with aspergilloma show progressive form, and we define such aspergillosis as CNPA, chronic necrotizing aspergillosis, although the original entity of CNPA by Binder et al. is different. We make a diagnosis of CNPA only if all the following entity meets, 1; progressive shadows in radiological findings regardless of the presence of aspergilloma, 2; have some symptoms such as cough, sputum, hemosputum, hemoptysis or fever, 3; proof of Aspergillus attribution by mycological or pathological examination, 4; positive systemic inflammatory reaction, 5; neglect of other etiology of pulmonary diseases. Since CNPA is usually progressive, patients with CNPA should be treated with antifungals.     

ANCA-associated vasculitis: diagnosis, clinical characteristics and treatment

The primary systemic vasculitides are a group of diseases characterized by an inflammatory process of the vessel walls and classified according to the smallest vessels involved. Small vessel vasculitides comprise the largest subgroup divided into diseases with a pauci-immune vasculitis and ANCA and diseases with deposition of immunoglobulin without ANCA. ANCA-associated systemic vasculitides include Wegener's granulomatosis, microscopic polyangiitis comprising renal-limited vasculitis and Churg-Strauss syndrome. Diagnosis is based on clinical manifestation, ANCA-testing and histology. Beside the role of ANCA as a diagnostic marker many studies and animal models have focused on the pathogenic role. The treatment of ANCA-associated vasculitis has changed from a standardized "Fauci-protocol" to an individualized less toxic strategy taking into consideration disease severity) organ manifestation, age of the patient and individual risk factors (e.g. increased bone marrow toxicity in patients with renal insufficiency). For remission induction patients are sub-grouped according to limited or generalized disease with moderate or severe renal involvement. Thus cyclophosphamide is only used in patients with generalized disease or - regarding Churg-Strauss-syndrome - patients with risk factors. For maintenance of remission azathioprine should be used in most of the patients.     

Acute fatal pulmonary embolism: its prevention, diagnosis and treatment

Acute fatal pulmonary embolism is one cause of sudden death which should be guarded against. It is the most often missed diagnosis in sudden death cases within the hospital. Clinical pictures of 10 patients with acute fatal pulmonary embolism proved by autopsy were examined to elucidate the problems of diagnosis, and to look for an effective treatment, and a method of prevention. Common risk factors were old age and immobility due to stroke or postoperative state. Common past histories were hypertension, diabetes mellitus, obesity, atrial fibrillation and hyperlipidemia. Electrocardiogram and echocardiogram showed that in these patients there was definite evidence of acute right ventricular overload. High doses of intravenous urokinase should be given whenever acute cardiovascular collapse develops in such high risk patients. Emergent pulmonary angiogram and pulmonary embolectomy could be life-saving in patients with acute massive pulmonary embolism. Prevention is, however, the best treatment. In addition to anticoagulation medication, frequent change of body position and early mobilization are important precautions to prevent fatal pulmonary embolism developing in such patients.