变应性肉芽肿性血管炎1例报告并文献复习

1 病例摘要 患者女性,40岁,因反复咳嗽、气喘2年余到我院住院治疗.患者2004年3月开始出现着凉后咳嗽、咳痰、气喘,经抗炎、平喘治疗后症状缓解.此后在当地多个诊所就诊,诊断"支气管炎、支气管哮喘、过敏性肺炎",经抗炎并间断服用强的松治疗后,症状可缓解,但停药后症状又反复.     

肉芽肿性多小血管炎一例

患者,女,38岁.因＂间断咳嗽,咳痰5个月,浮肿伴纳差、恶心1个月＂入院.5个月前受凉后出现低热,畏寒,咳嗽,咳带血丝黄痰,胸闷,伴活动后气喘,周身关节酸痛.先后就诊于多家医院,行抗感染治疗后发热、咳嗽、咳痰仍反复发作.病程中曾出现视物模糊（眼科诊断为结膜炎）.4个月后患者出现恶心、呕吐,呕吐物为胃内容物,咯血,尿量渐减少（约300 ml/d）,查血肌酐1100 μmol/L,行连续性肾脏替代治疗（CRRT）,病程中一度出现神志不清.患者既往无特殊病史.人院查体：眼睑浮肿,双肺呼吸音粗,双下肢中度对称凹陷性水肿.     

变应性肉芽肿性血管炎误诊2例分析

目的总结变应性肉芽肿性血管炎(Churg-Strauss syndrome,CSS)的诊治经验及误诊原因。方法回顾性分析近年来本院收治诊断的2例被误诊的CSS患者的临床表现、发病机制、影像学表现、诊断和治疗的特点。结果 2例患者中1例被误诊为难治性哮喘,1例被误诊肺结核,提示CSS患者的临床表现早期可能与相关疾病很多相似之处,容易漏诊、误诊而延误诊治。结论临床医师对本病的认识不足是误诊的主要原因,提高对本病的认识程度,熟悉其病理生理,临床特点,影像学特征是减少误诊的关键。     

长期误诊的变应性肉芽肿性血管炎一例

患者,女性,39岁。因反复咳嗽、气喘20年,加剧伴腹痛腹泻2周,于2002年10月11日入院。患者19岁开始出现反复咳嗽,咳少量白色粘液痰,伴间歇性气喘,每次发作数天到数十天不等,无明显季节性,有时能自行缓解。2001年后症状加重,体力下降,曾在外院按“慢支、肺气肿,过敏性支气管炎,哮喘,肺结核,心脏病”等进行治疗,病情无改善。     

嗜酸性肉芽肿性多血管炎并多发脑梗死一例

患者,男,52岁,因“反复咳嗽、咳痰、喘息3年,间断痰中带血半年,加重1个月”于2018年10月26日入住华中科技大学同济医学院附属同济医院呼吸与危重症医学科。患者于2015年8月无明显诱因出现咳嗽、喘息,于当地医院诊断为哮喘,给予对症治疗后症状缓解,出院后长期规律吸入布地奈德福莫特罗和噻托溴铵,并间断口服孟鲁司特钠、多索茶碱片控制病情,但上述症状仍反复发作,且发作频率增加,急性加重时外院多次查嗜酸性粒细胞(EOS)计数升高(家属代诉,未见报告)。     

以胆脂瘤型中耳乳突炎为首发症状的肉芽肿性血管炎1例并文献复习

肉芽肿性血管炎(Granulomatosis with polyangiitis,GPA)以前也被称为韦格纳氏肉芽肿病(Wegener granulomatosis,WG),是一种系统性坏死性血管炎,与细胞质ANCA相关,属多系统受累的自身免疫疾病。该病常累及小、中、偶尔累及大动脉,导致呼吸系统、循环系统、肾脏和中枢、周围神经系统的损害。但是以胆脂瘤型中耳乳突炎为首发症状者则较少见。现将临床诊治的一例胆脂瘤型中耳乳突炎合并肺部肉芽肿性疾病的病例及文献复习报告如下。     

肉芽肿性多血管炎合并颅内动脉瘤

目的总结肉芽肿性多血管炎(granulomatosis with polyangiitis,GPA)并发颅内动脉瘤临床特征、治疗及预后。方法报道1例GPA并发颅内动脉瘤病例。结果患者行双侧大脑中动脉瘤夹闭术,术后恢复良好。结论及时完善相关检查可GPA并发颅内动脉瘤为患者的治疗争取时间,明显改善预后。治疗上应积极应用大剂量皮质激素和环磷酰胺,必要时予手术处理。     

脑肉芽肿性血管炎误诊脑肿瘤一例

原发性中枢神经系统血管炎 (ｐｒｉｍａｒｙａｎｇｉｉｔｉｓｏｆｃｅｎｔｒａｌｎｅｒｖｏｕｓｓｙｓｔｅｍ ,ＰＡＣＮＳ)是局限于中枢神经系统的少见的小血管炎症性疾病 ,组织学显示 :不同程度的肉芽肿性和非肉芽肿性小血管炎 ,可有小血管实质性出血和血栓形成。其中肉芽肿性血管炎的临床表现和影像学特征与肿瘤极为相似 ,如不进行组织病理学检查 ,容易造成误诊。现将我院诊治的1例原发性脑肉芽肿性血管炎患者的情况介绍如下。患者 :女性 ,35岁。 1997年 7月 3日神经内科以“双基底节、颞叶占位病变”收入院。表现为无诱因头痛 1个月 ,多…     

显微镜下多血管炎相关肺纤维化3例及文献复习

显微镜下多血管炎（microscopic polyangiitis,MPA）是一种主要累及小血管的系统性坏死性血管炎,多见于中老年患者,肾脏及肺为其最常受累及的器官。肺受累表现为肺泡出血、坏死性气道炎症、间质炎症及纤维化,其影像表现不具有特征性。     

显微镜下多血管炎和肉芽肿性多血管炎死因及临床特点

目的研究并比较肉芽肿性多血管炎(granulomatosis with polyangiitis,GPA)和显微镜下多血管炎(microscopic polyangiitis,MPA)死亡病例的死因及临床特点。方法回顾性分析北京协和医院因MPA或GPA住院的病例,评估疾病活动度,并统计分析两组死因及疾病特点。结果 455例诊断为GPA或MPA的住院患者中,死亡49例(GPA 15例,MPA 34例),MPA病死率明显高于GPA(14.6%vs.6.8%,P0.05)。GPA死亡患者的平均年龄明显低于MPA[(47.2±17.7)岁vs.(69.4±9.3)岁,P0.001]。MPA生存时间中位数明显低于GPA(3.85个月vs.10.00个月,P0.05)。起病3个月内死亡的MPA患者明显高于GPA(44.1%vs.6.7%,P0.05)。MPA患者五因子评分明显高于GPA[(3.2±1.0)vs.(1.5±0.5),P0.001]。GPA患者首要死因是原发病活动导致出血(53.4%),而MPA首要死因是感染(55.9%)。两组死因累及的首要系统均为呼吸系统,MPA占82.4%,GPA占66.7%。MPA组合并肺间质病变明显高于GPA(44.1%vs.13.3%,P0.05);而合并耳鼻喉表现明显低于GPA组(2.9%vs.66.7%,P0.001)。肺部感染病原分析显示,最常见为细菌感染,其次是真菌感染;且MPA组合并真菌感染(58.8%vs.26.7%,P0.05)及混合感染(44.1%vs.13.3%,P0.05)的发生率均高于GPA组。结论与GPA相比,MPA病死率高,且MPA患者病情重,进展快,易发生严重感染。     

家族性特发性肺纤维化五例临床特征分析

目的 探讨家族性特发性肺纤维化(FIPF)的临床特点,提高临床诊治水平.方法 回顾性分析河南省人民医院2006年5月至2007年8月诊断的2个家族中5例特发性肺纤维化(IPF)患者的临床、影像学和病理学资料.结果 5例(男3例,女2例)分属于2个家族,家族1有3例,家族2有2例.诊断时平均年龄为(55±12)岁.主要表现为咳嗽和进行性呼吸困难,双肺底Velcro 啰音,杵状指.高分辨率CT示网状模糊影和广泛蜂窝状改变.肺功能检查示通气功能正常,弥散功能降低.1例男性患者经开胸肺活检证实为普通型间质性肺炎.家族1中1例男性患者为体检中被发现,其影像学特征符合IPF特点.5例中有3例给予糖皮质激素和免疫抑制剂治疗,效果差,2例死亡.结论 FIPF的临床表现与IPF相似,高分辨率CT检查有助于早期发现家族中其他无症状的IPF患者.     

Relapsing subarachnoid hemorrhage as a clinical manifestation in microscopic polyangiitis: a case report and literature review

Clinical Rheumatology - Microscopic polyangiitis (MPA) is a systemic small-vessel vasculitis associated with anti-neutrophil cytoplasmic antibody (ANCA) and predominantly causes kidney and...     

Esophageal involvement in microscopic polyangiitis: a case report and review of literature

A 72-year-old man with cough and sputum showed esophageal wall thickening and pneumonia in chest computed tomography (CT) scan. Following endoscopy, we diagnosed reflux esophagitis and subscribed proton pump inhibitor. The esophageal lesion, however, was intractable. We diagnosed microscopic polyangiitis (MPA) because of vasculitis symptoms, cytoplasmic antineutrophil cytoplasmic antibodies (cANCA) in blood and no granulomatous change in the esophagus. We adopted pulse therapy of cyclophosphamide and oral prednisolone; the symptoms and esophageal lesion were markedly improved. We concluded that the esophageal lesion was an aspect of MPA. To our knowledge, this is the first report of esophageal involvement in MPA.     

胺碘酮致肺间质纤维化呼吸衰竭1例并文献复习

目的 提高对胺碘酮所致肺间质纤维化的认识,提高其诊断及治疗水平.方法 回顾性分析我院1例因误服过量胺碘酮致肺间质纤维化呼吸衰竭病例并进行相关文献复习.结果 患者,男,60岁,因“咳嗽、乏力伴畏寒2d”于2010年11月13日入院,入院前曾因“阵发性室上速”,给予胺碘酮150mg静注2次、300mg静脉滴注,后改为口服600 mg/d,3次/d,后患者未遵医嘱按时减量,至入院时共使用胺碘酮50 d总量30 g.入院时患者呼吸急促,心率快,口唇及甲床发绀,双侧中、下肺部闻及广泛湿哕音及爆裂音.实验室检查示:白细胞11.18×109/L,中性粒细胞0.87,谷丙转氨酶70 U/L,白蛋白26.3 g/L,总蛋白55.9 g/L,C反应蛋白102.50 mg/L,pH值7.441,PO234.80 mm Hg,PCO229 mm Hg,SaO268.9％,肺部CT示双肺呈间质性改变,以右肺尤著.肺功能检查提示:重度混合性通气功能障碍,肺弥散功能重度降低.诊断为胺碘酮所致肺间质纤维化合并感染,呼吸衰竭Ⅰ型.停用胺碘酮,面罩吸氧,先后给予左氧氟沙星、头孢哌酮/舒巴坦、万古霉素、氟康唑以及甲泼尼龙和乙酰半胱氨酸治疗后逐步好转,激素和乙酰半胱氨酸总疗程为7个月,停药后随访7个月,患者活动后无明显胸闷气促,肺CT间质纤维化改变吸收.结论 肺间质纤维化为胺碘酮常见药物不良反应之一.使用胺碘酮前应进行利益/风险评价,长期用药应使用最小有效剂量,定期检测肺功能,以减少和及时发现肺毒性.一旦临床诊断为肺毒性,应及时减量和停药,多数患者停药后可缓解,严重者需使用糖皮质激素治疗.     

以呼吸系统症状为首发表现的间变性大细胞淋巴瘤一例及文献复习

目的 提高对以呼吸系统症状为首发表现的间变性大细胞淋巴瘤（ALCL）的临床、实验室检查、胸部影像学和病理改变的认识。方法 对1例经纤维支气管镜肺活检和骨髓活检证实的ALCL病例的临床表现、实验室检查、影像学和病理资料并结合相关文献进行回顾性分析。结果 以呼吸系统症状为首发表现的ALCL症状主要表现为咳嗽、胸痛、咳血痰、气促、发热;实验室检查可有三系血细胞减少、脾大、骨髓噬血细胞增多;胸部X线和高分辨率CT（HRCT）早期可表现双肺多个小点状结节影,后可出现肺不张、肺团块影和纵隔淋巴结肿大,晚期可出现大量胸腔积液。ALCL的病理表现为肿瘤细胞体积大,核大而不规则,细胞弥散或巢样沿淋巴窦或滤泡间浸润,几乎所有的瘤细胞均表达间变细胞CD30（Ki-1）抗原。结论 以呼吸系统症状为首发表现的ALCL非常罕见,其临床表现、影像学检查无特异性,而诊断需要依靠病理,CD30（ki-1）阳性是诊断ALCL的有力依据。     

Microscopic polyangiitis complicated with bilateral brachial plexopathy: a case report and review of the literature

Microscopic polyangiitis is a small-vessel necrotizing vasculitis associated with anti-neutrophil cytoplasmic antibodies and presents itself with glomerulonephritis and hemorrhagic pulmonary capillaritis. Peripheral nervous system involvement is common in anti-neutrophil cytoplasmic antibodies-associated vasculitis, but brachial plexopathy is unusual. We present the case of a 22-year-old man with known microscopic polyangiitis who was under maintenance therapy with prednisolone and cyclophosphamide and developed cough, dyspnea, and hemoptysis which increased in 6 days accompanying pain and paresthesia in the upper limbs. His physical examination revealed hypoesthesia, absence of deep tendon reflexes, and decreased muscle strength in the upper limbs. His chest computed tomography scan showed ground glass pattern in the lower and middle lobes. Electromyography and nerve conduction study showed bilateral brachial plexopathy with involvement of all of the cervical roots that were more severe in the lower roots and left side.     

Eosinophilic granulomatosis with polyangiitis complicated by cholecystitis: a case report and review of the literature

The objectives are to report an atypical case of eosinophilic granulomatosis with polyangiitis (EGPA) associated with cholecystitis and to review the main clinical features, therapy, and prognosis of it. We present a 49-year-old male with non-classic clinical manifestations of EGPA and EGPA-related cholecystitis. EGPA was diagnosed by histology of the gallbladder after cholecystectomy. In addition, 11 cases of EGPA-associated cholecystitis have been reported and were described in details in this article. Gallbladder involvement is very uncommon in EGPA. All cases reviewed showed multiple organs involved as well as obviously elevated eosinophilic granulocyte proportion with inflammatory index, although antineutrophil cytoplasmic antibody may be negative. All patients in this cohort that showed gallbladder involvement were eventually confirmed with EGPA by histology examination after cholecystectomy. The pathological change could be infiltration of inflammatory mononuclear cells of small- and medium-sized vessels. Of the cases, 91.7 % responded well to steroid and immunosuppressant therapy. Gallbladder involvement is a very rare comorbid condition in EGPA. However, it is an important symptom or secondary condition to alert physicians the diagnosis of EGPA. Moreover, timely diagnosis and correct administration in the early stage of this disease could obviously improve the prognosis.     

Imatinib mesylate use in refractory eosinophilic granulomatosis with polyangiitis: a literature review and a case report

Clinical Rheumatology - Recent advances in pharmacology have greatly expanded the drug repertoire for treatment of anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis. Eosinophilic...     

原发性肺隐球菌病例报告及文献复习

目的:探讨肺隐球菌病的临床及病理特点,提高对肺隐球菌病的认识,以减少误诊。方法:回顾性分析1例原发性肺隐球菌病的诊断和治疗经过,分析总结治疗经验,结合文献复习肺隐球菌病的发病机制、临床表现、诊断与治疗要点。结果:患者男性,56岁,有糖尿病史,胸部影像学检查发现右肺内胸膜下多发性片状或团块状阴影,给予抗生素治疗无效,曾怀疑为肺癌行经皮肺活检提示肺隐球菌病,入我院进一步诊治。病理检查提示肉芽肿形成,可见隐球菌孢子,高碘酸希夫染色(PAS)和六胺银染色均阳性,确诊原发性肺隐球菌病。给予氟康唑400 mg/d,静脉滴注,6 w后病灶明显吸收,改为氟康唑口服出院。结论:肺隐球菌病缺乏特异性的临床和影像学表现,容易误诊、误治。临床医生需要提高对肺隐球菌病的认识,尽可能行病理检查结合特殊染色明确诊断。标本来源以创伤小的经皮肺穿刺为首选,治疗前需全面评估病情。