原发性干燥综合征肾损害的临床病理分析

目的：探讨原发性干燥综合征患者肾员害的临床表现、免疫学特点和肾脏病理改变。方法：回顾性分析14例原发性干燥征伴肾损害患者的临床表现、实验室指标、肾活检病理及免疫病理、肾组织肾小球及小管间质病变程度采用半定量积分表示。结果：（1）育龄期妇女发病多见，肾脏受累以低渗尿和肾小管酸中毒（RTA）最为常见（92．9％），其中I型RTA8例，Ⅲ型RTA2例，混合型RTA2例，不完全型RTA1例。尿蛋白阳性的患者均表现为小管源性蛋白尿，镜下血尿少见。（2）免疫系统异常，13例患者有多种自身抗体阳性，冷球蛋白阳性率为50％（6／12），血清球蛋白升高71．4％，IgG异常升高71．4％（8／11），IgM异常升高9．09％（1／11），IgA异常升高18．2％（2／11）。（3）肾活检病理改变11例为慢性间质性肾炎，3例肾小管间质基本正常，肾小球均有系膜增生，部分有C3（50％）、IgM(21.4%),IgG(14.3%)沉积。小管基膜和间质血管壁均无免疫球蛋白沉积。结论：干燥综合征伴肾损害以尿浓缩功能减低和肾小管酸中毒最为多见，肾小管间质病变为慢性间质性肾炎，血管病变发生率低，高球蛋白血症和小管间质的功能和形态改变有一定联系。     

CT引导下经皮肺活检48例临床分析

目的：探讨肺部外周性病变的诊断准确性。方法：对痰细胞学阴性纤支镜不能确诊的48例肺部外周性病变患者，在胸部CT扫描定位下，用20G活检针经皮肺穿刺活检取材，行病理学诊断。结果：48例患者共行穿刺53次，阳性（43／48）占89．6％，阴性（5／48）占10．4％，经手术后病理、临床证实，其恶性病变诊断正确率93．3％（28／30），良性病变诊断正确率83．3％（15／18），误诊率10．4％。术后小量咯血2例，未发生气胸及死亡病例。结论：以CT引导下经皮肺活检是一种安全、有效的检查手段，对肺部周围性病变具有较高的诊断价值。     

有机溶剂相关性肾病综合征(一种新发现的中毒性肾病?)

目的：分析有机溶剂相关性。肾病综合征患者的流行病学、临床表现及肾组织病理特点。万法：回顾性分析2004年10月至2008年4月在解放军肾脏病研究所住院经临床及肾活检诊断为有机溶剂相关性。肾病综合征18例患者临床资料，分析其接触有机溶剂的种类、临床表现、实验室检查、肾组织病理特点及初步随访结果。结果：（1）18例有机溶剂相关性肾病综合征患者多见于青年男性，其中男性15例，女性3例，平均年龄为（26．1±11．9）岁，所有患者均表现为中-重度水肿，大量混合性蛋白尿，平均尿蛋白（9．67±2．85）g／24h，其中4例患者入院时出现血压升高。（2）肾小管损伤标记物尿NAG酶及RBP均明显升高，其中尿NAG酶平均（67．5±40．4）u／g·cr。所有患者均无镜下血尿、糖尿及氨基酸尿。（3）18例患者血白蛋白均明显降低，平均（17．5±2．5）g／L，总胆固醇水平均明显升高，平均（11．5±2．87）mmol／L。9例患者入院时出现血肌酐升高，平均（130．8±73．7）μmoL／L。16例患者血免疫球蛋白IgG显著降低，平均为（3．93±2．61）g/L。（4）所有患者肾活检病理示肾小球病变轻，表现为轻度节段系膜增生性病变或无系膜增生性病变，但均可见足细胞数目减少，13例患者出现节段壁层上皮细胞增生，16例患者出现肾小管返流。所有患者均存在近端肾小管上皮细胞刷状缘脱落，但无间质炎细胞浸润。（5）18例患者对免疫抑制剂治疗均无明显疗效，随访时间1～43月，2例（11．1％）患者死亡，7例（38．89％）患者出现肌酐倍增，其中3例（16．67％）患者进入ESRD期。结论：有机溶剂相关性肾病综合征是一种新发现的肾脏疾病，多见于中青年男性，临床表现为超大量蛋白尿伴近端小管损伤，表现为尿NAG酶，尿RBP升高，混合性蛋白尿，尿渗量正常，无糖尿及氨基酸尿；肾活检病理表现为足细胞数目减少，节段壁层上皮细胞增生，肾小管返流以及近端小管病变；该病对免疫抑制剂治疗反应不佳，预后较差。     

胃上皮内瘤变内镜活组织检查与内镜切除标本病理结果差异分析

目的 分析比较我国胃镜活组织检查（简称活检）和内镜下切除病理诊断胃上皮内瘤变的可靠性。 方法 回顾性分析2010年1月至2015年3月北京协和医院胃镜活检病理诊断为胃上皮内瘤变的98例患者,包括20例低级别上皮内瘤变（LGIN）,65例高级别上皮内瘤变（HGIN）和13例早期胃癌（EGC）。患者均行内镜下切除,结合患者临床资料,对活检病理与内镜下切除病理差异率、临床特征和差异因素进行分析。 结果 20例活检病理为LGIN的患者,内镜下切除后有12例病理结果较活检病理进展,其中7例HGIN（差异率35.0％,7／20）,5例EGC（25.0％,5／20）。活检病理诊断HGIN的65例患者,内镜切除后38例诊断为EGC（58.5％,38／65）,4例诊断为LGIN（6.2％,4／65）。13例活检诊断为EGC者切除后病理维持原诊断。活检病理和内镜切除病理诊断的总体差异率为55.1％（54／98）。病变直径＞2 cm,病变表面充血是活检和内镜切除病理差异的主要因素（P＜0.05）。 结论 内镜活检病理诊断胃上皮内瘤变的可靠性欠佳,内镜下切除不仅是治疗胃上皮内瘤变的主要手段,也是明确组织学诊断的一个重要方法。     

胃原发性恶性淋巴瘤22例临床分析

目的探讨胃原发性恶性淋巴瘤临床及内镜表现。方法分析总结22例患者临床、胃镜及病理。结果22例患者临床表现无特异性，上腹痛最常见(22／22)，其次为上消化道出血(16／22)、反酸嗳气(6／22)等。胃镜表现：病变发生2个以上部位多见(10／22)，其次为胃体(6／22)、胃窦(3／22)等；病变直径多大于2cm(占17／22)；病变形态以溃疡型多见(11／22)，其次为弥漫浸润型(7／22)；胃镜活检加免疫组化确诊率为63．63％(14／22)；Hp阳性检出率86．36％(19／22)。结论胃原发性恶性淋巴瘤临床表现无特异性；其镜下形态多种多样，其中累及2个以上部位者多见，病变范围较大。胃镜活检加免疫组化病理检查是诊断本病的重要手段，Hp感染与胃原发性恶性淋巴瘤的发生具有相关性。     

经支气管镜腔内超声非实时引导下行肺活检对菌阴肺结核的诊断价值

目的评价经支气管镜腔内超声（endobronchial ultrasonography,EBUS）非实时引导下行肺活检对菌阴肺结核的诊断价值和安全性。方法对2014年12月至2016年12月在福州肺科医院就诊、且行常规电子支气管镜检查未能发现病变的46例菌阴肺结核患者,行EBUS非实时引导下的肺活检,观察诊断阳性率、不同部位肺部病灶发现率及安全性。结果通过EBUS非实时引导下经支气管镜肺活检,46例菌阴肺结核患者中,40例肺部周边发现病变,病变总体发现率为86．96％（40／46）。40例通过EBUS技术发现病灶的患者中,32例经支气管镜肺活检明确诊断,诊断阳性率为80．00％（32／40）;6例通过EBUS技术未发现病灶的患者,再次行常规支气管镜肺活检,常规支气管镜肺活检诊断阳性者3例（3／6）;其余的11例患者,7例通过CT引导下肺穿刺病理检查明确诊断,4例通过胸腔镜手术病理检查明确诊断。40例通过EBUS技术发现病灶的患者中,病灶位于右上叶者诊断阳性11例（11／13）,病灶位于右中叶者诊断阳性2例（2／3）,病灶位于右下叶者诊断阳性8例（8／10）,病灶位于左上叶者诊断阳性7例（7／8）,病灶位于左下叶者诊断阳性4例（4／6）。46例患者支气管镜肺活检后的主要并发症为咯血（39．13％,18／46）和胸痛（13．04％,6／46）,未出现气胸等并发症;均未进行特殊处理,白行缓解。结论EBUS非实时引导下经支气管镜肺活检诊断率高,并发症少,适用于菌阴肺结核的诊断。     

CT引导下经皮肺活检的临床应用

目的探讨CT引导下经皮肺穿刺活检对肺部周围型病变的诊断价值。方法对痰细胞学阴性及纤维支气管镜不能确诊的58例肺部周围型病变患者，在胸部CT定位下，用活检针经皮肺穿刺活检取材，行病理学诊断。结果58例患者的诊断阳性率为94．8％（55／58），阴性率5．2％（3／58），术后气胸7例，小量咯血2例。结论CT引导下经皮肺穿刺活检是一种安全的检查手段，对周围型肺部病变具有较高的诊断价值。     

超声内镜在胰岛细胞瘤定位诊断中的作用

目的 探讨内镜超声在胰岛细胞瘤定位诊断中的作用。方法 对临床症状及生化检查诊断胰岛细胞瘤的患者15例分别行腹部B超、CT、选择性动脉造影（DSA）和内镜超声检查（EUS），通过与手术和病理结果对照，分析各影像学检查在胰岛细胞瘤定位诊断中的意义。结果 经手术及病理证实为胰岛细胞瘤15例（包含16个病灶），各检查诊断准确率分别为：腹部B超20.0％（3／15），CT33．3％（5／15）。DSA64．3％（9／14）和EUS86．7％（13／15）。EUS发现的14个病灶中，表现低回声者10个，中等回声者1个，中等偏强回声者3个，病变边界均清楚，回声均匀。EUS发现病变小于2cm者10个，最小为0．5cm。EUS误判断的2例中，1例位于胰尾与脾门交界处，另1例胰腺内有2个病变，而EUS仅发现了1个。结论 EUS对胰岛细胞瘤定位诊断的敏感性明显优于B超、CT、DSA等。     

白塞病合并肾脏损害三例分析并文献复习

目的 分析白塞病合并肾脏病变的临床和肾脏活组织检查（简称：肾活检）组织学特点。方法 分析3例白塞病患者肾脏表现的临床、实验室检查及肾脏活检表现，对相关文献进行复习并与本研究结果对比。结果 3例患者出现肾脏病变时均伴有全身病变活动。2例表现为无症状蛋白尿，肾活检为肾小球病变；另1例表现为肾小管酸中毒和蛋白尿，肾脏活检为肾小管间质病变，3例均有不同程度的肾间质病变。所有3例的肾脏病变均较轻，经糖皮质激素和免疫抑制剂联合治疗后临床表现减轻。结论 白塞病肾脏受累在临床上不多见，肾脏损害以蛋白尿多见。病理上肾小球、肾小管及肾间质均可以受累，而肾血管则均未累及。本研究报道的3例患者均有肾间质病变，肾脏组织学损害较轻微。     

儿童原发性肾病综合征并发特发性急性肾衰竭12例临床分析

回顾性分析12例原发性肾病综合征（PNS）并发特发性急性肾衰竭（IARF）患者的临床资料。结果显示，12例均为突然发生少尿或无尿，血肌酐及尿素氮进行性升高，肾活检病理检查示微小病变7例（58．3％）、系膜增生性肾炎3例（25．0％）、局灶节段性肾小球硬化2例（16．7％），其共同病理变化是小管间质病变广泛；经泼尼松、血液透析等对症治疗肾功能均迅速改善。认为PNS并发IARF多见于肾小球微小病变者，早期行综合治疗者预后较好。     

Hypereosinophiles Syndrom und Churg-Strauss-Syndrom

Churg-Strauss syndrome and the hypereosinophilic syndrome share many clinical features, particularly in the early disease stages. Beside blood and tissue eosinophilia, peripheral neuropathies, cutaneous manifestations, eosinophilic alveolitis and gastroenteritis are frequently found. In contrast to the hypereosinophilic syndrome, Churg-Strauss syndrome is defined by the presence of systemic vasculitis. However, frequently symptoms related to eosinophilia are (mis)interpreted as indirect signs of vasculitis. New treatment modalities and diagnostic methods render the early differentiation between Churg-Strauss syndrome and the hypereosinophilic syndrome increasingly clinically important. Patients with hypereosinophilic syndrome should be tested for the presence of the FIP1L1-PDGRFA-mutatition in order to identify patients that could benefit from a treatment with a tyrosine kinase inhibitor such as Imatinib. At present, immunosuppression is still the treatment of first choice for Churg-Strauss syndrome. Novel treatment modalities for both diseases include immunomodulation with interferon alpha and biologics such as antibodies against interleukin 5.     

Inhaled corticosteroids and Churg-Strauss syndrome: a report of five cases.

Churg-Strauss syndrome is an eosinophil-associated, small vessel granulomatous vasculitis, characterized by late onset asthma, upper airways disease, eosinophilia, and clinical manifestations of systemic vasculitis. Several cases of Churg-Strauss syndrome have been recognized in patients treated with cysteinyl leukotriene-receptor antagonists and weaned off systemic corticosteroids. These cases have led to a general warning on the possible development of Churg-Strauss syndrome after taking cysteinyl leukotriene-receptor antagonists. The authors report five cases of Churg-Strauss syndrome in severe steroid dependent asthmatics in whom inhaled corticosteroids allowed systemic corticosteroid withdrawal. It is concluded that physicians should monitor patients carefully when severe asthma is controlled with any substance allowing withdrawal from (or even avoidance) of systemic corticosteroids. Case-control studies should identify more precisely the risk factors of Churg-Strauss syndrome.     

Churg-Strauss综合征肺损害1例并文献复习

目的 加深对Churg-Strauss综合征又名变应性肉芽肿血管炎(CSS)的认识.方法 通过对1例CSS病例的诊断、治疗及相关文献进行研究学习,分析Churg-Strauss综合征的病因、临床症状、实验室检查、影像学表现、诊断、鉴别诊断及治疗.结果 CSS病因不清,临床表现无特异性,可累及多系统器官,最常见的临床表现是哮喘及血管炎引起的肺部表现,特征性的病理表现为外周血嗜酸粒细胞增多,目前治疗首选激素,预后较好.结论 Churg-Strauss综合征是一种少见的系统性血管炎,临床缺乏特异性表现,易被误诊,当患者表现为哮喘、外周血嗜酸性粒细胞增多及肉芽肿性血管炎时,应高度怀疑此病.     

Allergic angiitis with granulomatosis: Churg-Strauss syndrome. Retrospective study of 16 cases

Sixteen patients with Churg-Strauss syndrome (CSS), a disorder characterized by hypereosinophilia and systemic vasculities which complicate preexisting asthma, were analyzed. The mean duration of asthma before CSS was 8 years; peripheral blood eosinophilia was always greater than 1,900/microliters and exceeded 5,000/microliters in 14 cases. The clinical manifestations were the following: 16 in generally poor condition with fever; 12 peripheral neuropathies; 11 cutaneous lesions; 9 pericardial or myocardial involvement; 9 digestive disorders; 9 muscular or articular diseases; 5 renal involvement, all associated with the vasculitis; 7 upper respiratory tract disorders. Chest radiographs showed pleuropulmonary or cardiac anomalies in 11 patients. The diagnosis was confirmed histologically in 11 cases, however, no clinical, biological or evolutive differences were observed between these patients and those with negative biopsies (5). Follow-up for 6.35 +/- 5.55 years was characterized by relapses always preceded by increased eosinophilia. Fourteen patients were successfully treated with corticosteroids, associated with cyclophosphamide in 7 of them. Five-year survival was 87%. Four deaths occurred, all CSS-associated, two because of a poorly adapted therapeutic regimen. The need for rapid and effective treatment must be stressed. The diagnosis can be made based on clinical manifestations alone before histological confirmation can be obtained.     

Churg-Strauss Syndrome: An Update

Churg-Strauss syndrome is an uncommon disease of unknown cause described initially by Churg and Strauss in 1951. Even though it was initially thought to be a variant of polyarteritis nodosa, its pathological, clinical, and laboratory features show that it is related to the small vessel vasculitides, and it is now classified as an antineutrophil cytoplasmic antibody-associated vasculitis. The presence of asthma, usually of adult onset, along with other allergic symptoms, peripheral and tissue eosinophilia, is specific to this disease. These features usually help clinicians distinguish it from other types of small vessel vasculitis and should alert clinicians about its presence. Two different clinical subtypes defined by the presence of antineutrophil cytoplasmic antibodies recently have been recognized. Recent advances in the treatment and pathophysiology of Churg-Strauss syndrome are reviewed in this article.     

Churg-Strauss syndrome

Churg-Strauss syndrome is a systemic ANCA-associated vasculitis arising almost exclusively in patients with a pre-existent asthma. Common clinical manifestations are marked blood eosinophilia, asthma, chronic sinusitis, cardiomyopathy, pulmonary infiltrates, gastrointestinal complaints and a multiplex neuropathy. The morphological substrate is an eosinophilic necrotizing vasculitis.Other eosinophilic disorders such as parasitic diseases, allergies and idiopathic hyper-eosinophilic syndrome have to be excluded. The mainstay of therapy is high-dose corticosteroids with the addition of cytotoxic drugs in patients with poor prognosis.     

Das Churg-Strauss-Syndrom

Churg-Strauss syndrome is a systemic ANCA-associated vasculitis arising almost exclusively in patients with a pre-existent asthma. Common clinical manifestations are marked blood eosinophilia, asthma, chronic sinusitis, cardiomyopathy, pulmonary infiltrates, gastrointestinal complaints and a multiplex neuropathy. The morphological substrate is an eosinophilic necrotizing vasculitis. Other eosinophilic disorders such as parasitic diseases, allergies and idiopathic hyper-eosinophilic syndrome have to be excluded. The mainstay of therapy is high-dose corticosteroids with the addition of cytotoxic drugs in patients with poor prognosis.     

Churg-Strauss syndrome (two case reports)

Churg-Strauss syndrome is a necrotizing vasculitis with multiple organ involvement characterized by asthma, peripheral blood eosinophilia, eosinophilic tissue infiltration and extravascular granulomas. A 35 years-old male with 6-months history of asthma and a 43 years-old female with 4-years history of asthma, were further examined due to clinical worsening and lesions on chest radiographs. They were finally diagnosed as Churg-Strauss syndrome. Clinical and radiological response to oral corticosteroid therapy was obtained.     

Parasitic eosinophilic lungs

The eosinophilic lung is a term used to identify a heterogeneous group of parasitic diseases leading to common manifestations including alveolar or tissular pulmonary eosinophilia and, classically, radiological visible lesions of the lung. The different clinical presentations--L?ffler's syndrome, larva migrans syndrome and tropical pulmonary eosinophilia are analyzed in this article and the particular features of the different causal parasites are discussed. Positive and differential diagnostic procedures in patients with pulmonary eosinophilia due to mycoses, drugs, vasculitis or an unknown cause are detailed.