混合性冷球蛋白血症性肾小球肾炎

混合性冷球蛋白血症(ＭＣ)是一种较少见的系统性疾病。1966年,Ｍｅｌｔｚｅｒ[1]首先报道了一种综合征,其主要的临床表现为紫癜、乏力、关节痛和肾小球肾炎,偶有胃肠道、心肺和神经系统等血管炎的系统症状。这些患者常伴有肝脏病变,部分可以多年无症状,但其中许多患者肝脏病变呈进行性发展,常死于肝脏疾患。由于当时未找到明确病因,因而称之为原发性ＭＣ,直到1990年,Ｐａｓｃｕａｌ等[2]才发现原发性ＭＣ与丙型肝炎病毒(ＨＣＶ)感染密切相关。此后,在大部分原发性ＭＣ患者中均发现有抗ＨＣＶ抗体,并认为ＨＣＶ感染在原发性ＭＣ的发生中起关…     

丙型肝炎病毒相关性冷球蛋白血症性肾炎

混合性冷球蛋白血症,特别是含有单克隆IgM类风湿因子的Ⅱ型冷球蛋白血症,与HCV感染的关系尤为密切,而且常导致肾脏损害[1～4 ] 。现将有关问题综述如下。1　发病机理在HCV抗原持续刺激下由B淋巴细胞(CD 5或CD 5B1)单个克隆产生的冷球蛋白,在一定的条件下滞留于小口径血管内引     

利妥昔单抗治疗冷球蛋白血症相关膜增生性肾小球肾炎的疗效

目的:观察利妥昔单抗(RTX)治疗冷球蛋白血症相关膜增生肾小球肾炎(MPGN)患者的疗效及不良反应.方法:回顾性分析8例经肾活检病理诊断为冷球蛋白血症相关MPGN患者的资料,接受RTX治疗并随访≥12月;RTX按单次375 mg/m2静脉输注.结果:4例为乙型肝炎病毒(HBV)感染相关,4例原发病因不明确.所有患者均联...     

Ⅱ型冷球蛋白血症肾脏损害1例

冷球蛋白血症肾损害临床上极易漏诊误诊。加深对冷球蛋白血症肾损害的临床病理特点的认识,特别是提高做冷球蛋白检查的意识,是减少漏诊误诊的关键。本文讨论1例Ⅱ型冷球蛋白血症肾脏损害,临床表现为急性肾炎综合征,血肌酐升高并高血压,冷球蛋白明显升高伴类风湿因子阳性,补体降低,无丙肝感染。入院后通过临床-病理-实验室检查相结合,特别是冷球蛋白分析,诊断为Ⅱ型冷球蛋白血症(单克隆IgM+多克隆IgG)肾脏损害。希望通过复习本病例,提高临床医师对该病的认识及诊断水平。     

原发性混合性冷球蛋白血症肾损害

原发性混合性冷球蛋白血症肾损害陈惠萍朱茂艳黎磊石关键词冷球蛋白血症肾损害诊断中图法分类号Ｒ５９３南京军区南京医院解放军肾脏病研究所（南京，２１０００２）１病例报告患者男性，５１岁，因持续性蛋白尿５年，肾功能迅速减退１年入院。缘于１９９１年５月，患者发...     

单克隆丙种球蛋白病相关性冷球蛋白血症肾损害诊治

Ⅰ型及Ⅱ型冷球蛋白血症(CG) 发病均与单克隆免疫球蛋白(MIg) 相关。Ⅰ型冷球蛋白由单一的MIg 组成, Ⅰ型CG 的基础疾病为B 细胞淋巴增殖性疾病,多数为意义未明的丙种球蛋白病(MGUS),其次为多发性骨髓瘤及 华氏巨球蛋白血症等恶性病;Ⅱ型冷球蛋白由具有类风湿因子活性的单克隆IgM 与多克隆IgG 组成,Ⅱ型CG 的基 础疾病绝大多数为感染性疾病( 丙型肝炎病毒感染最常见),少数为干燥综合征等自身免疫性疾病。它们继发的肾 炎(GN) 主要为膜增生性肾小球肾炎。文章对这两型CG 及其GN 的发病机制、临床及病理表现和治疗原则做一讨论。     

冷球蛋白血症的肾脏损害

冷球蛋白是一种含有类风湿因子(RF)和单克隆或多克隆的免疫球蛋白,由B淋巴细胞增殖所致,在低温时沉淀的免疫复合物.冷球蛋白血症是由于冷球蛋白沉积于血管内皮,导致皮肤、肾脏、周围神经等病变,产生皮肤血管炎、肾小球肾炎和神经系统症状等主要累及中小动脉的系统性血管炎.冷球蛋白血症导致的肾脏损害常见的是膜增生性肾小球肾炎(MPGN)~([1]).     

多发性骨髓瘤继发Ⅰ型冷球蛋白血症患者的临床特征与转归

目的:探讨7例多发性骨髓瘤(multiple myeloma, MM)继发Ⅰ型冷球蛋白血症(cryoglobulinemia, CG)患者的临床特征、治疗及转归。方法:回顾性分析2015年1月—2023年3月北京协和医院确诊的7例MM继发Ⅰ型CG患者的临床资料、治疗方案和生存结局。结果:7例患者中,男5例,中位诊断年龄为56(40～71)岁。4例继发于冒烟型骨髓瘤。CG受累器官包括皮肤(n=5)、周围神经(n=4)、关节(n=4)和肾脏(n=1)。中位冷球蛋白水平为13 046.5(693.8～33 988.0) mg/L,所有患者均为IgG单克隆型冷球蛋白,与M蛋白类型一致。7例患者均接受了抗浆细胞治疗,其中1例因CG相关急性肾衰竭同时进行了血浆置换。6例有随访资料的患者均获得了MM血液学缓解,4例有CG相关症状者均达到临床缓解。中位随访37(10～45)个月后,1例失访,1例因治疗相关感染死亡。结论:MM是Ⅰ型CG相对少见病因,对于有皮肤、周围神经、关节等受累表现的MM患者,应考虑到继发性CG的可能。     

丙肝病毒相关性肾小球肾炎的治疗

丙肝病毒（HCV）感染可导致慢性肝病，也可导致严重的肝外损害，包括混合性冷球蛋白血症（MC）、淋巴增生性疾病和肾脏疾病。HCV相关性肾炎的传统治疗为阻断肾素血管紧张素系统（RAS）、抗病毒；表现为大量蛋白尿和（或）进行性肾功能衰竭的患者需要免疫抑制治疗。选择性抗B细胞单克隆抗体——抗CD20单克隆抗体美罗华（rituximab）疗效等于或优于传统免疫抑制剂环磷酰胺，安全性好，已开始应用于临床，血浆置换与激素冲击也用于MC相关性膜增殖性肾小球肾炎（MPGN）急性期治疗。本文就HCV相关性肾小球肾炎，尤其是MC相关性MPGN的治疗作一综述。     

Combination therapy of prednisolone and mizoribine improves cryoglobulinemic vasculitis with purpura and skin ulcers

Conventional treatment is not standardized for hepatitis C virus-negative cryoglobulinemia, but corticosteroids, immunosuppressive agents, and plasma exchange typically improved the symptoms. Mizoribine is an immunosuppressive agent that was developed in Japan and has been found to inhibit the proliferation of lymphocytes, especially B cells. We have encountered an elder patient who had hepatitis C virus-negative, type II cryoglobulinemic vasculitis with leg purpura and skin ulcers. Her symptoms improved and cryoglobulin disappeared by the combination therapy of prednisolone and mizoribine. We speculate the action mechanism of this therapy is due to immunosuppressive effects including up-regulation of the efficacy of prednisolone by mizoribine.     

Prevalence of cryoglobulinemia and cryoglobulinemic vasculitis in chronically HCV-infected Brazilian patients

Introduction and ObjectivesCryoglobulinemia is one of the most frequent extrahepatic manifestations of chronic hepatitis C virus (HCV) infection and it may evolve to cryoglobulinemic vasculitis (CryoVas) which is a systemic vasculitis that affects small-sized vessels. The objective of this study was to evaluate the prevalence of cryoglobulinemia and CryoVas in HCV patients in São Paulo, Brazil.Materials and methodsA cross-sectional study was conducted and included sixty-eight viremic HCV patients, without HIV or hepatitis B coinfection. A thorough clinical and laboratory evaluation was performed including the detection of serum cryoglobulins and measurement of serum complement components. The classification criteria for CryoVas were applied.ResultsThe study population comprised mainly women (61.8%) with long term HCV infection (median 11.0 years). Advanced hepatic fibrosis was detected in 20.6% (14/68) of cases. Cryoglobulins were detected in 48.5% (33/68) of HCV-patients with type III cryoglobulinemia being the most frequent. CryoVas was present in 10.3% (7/68) and the main manifestations were peripheral neuropathy (85.7%), palpable purpura (42.8%), arthralgias (42.8%) and renal involvement (42.8%). Life-threatening manifestations were rare. Low hemolytic C2, C4 and total hemolytic complement (CH100) levels were common findings in the cryoglobulinemia group. Low C4 levels were independently associated with the development of CryoVas.ConclusionA high prevalence of cryoglobulinemia and CryoVas was found in Brazilian HCV-patients. CryoVas patients mostly presented non-life-threatening manifestations, especially peripheral neuropathy. Complement abnormalities were common in patients with cryoglobulinemia and low serum C4 levels were associated with CryoVas.     

Systemic lupus erythematosus arising during interferon-alpha therapy for cryoglobulinemic vasculitis associated with hepatitis C

We present the case of a 53-year-old woman who developed systemic lupus erythematosus (SLE) after being treated with interferon-alpha (IFN-) for cryoglobulinemic vasculitis associated with hepatitis C virus (HCV) infection. Her cryoglobulinemic vasculitis resolved rapidly with IFN- treatment. However, after 10 months of IFN- therapy, she developed a photosensitive malar rash, oral ulcers, arthralgias, lymphopenia, and anti-SSA autoantibodies. She was diagnosed with SLE induced by IFN- therapy. IFN- was discontinued, she was treated with a short course of prednisone and hydroxychloroquine, and she improved rapidly. This is the first report of IFN--induced SLE complicating treatment of cryoglobulinemic vasculitis associated with HCV infection. The development of SLE during therapy with IFN- could be due to direct immunomodulation by IFN-, and review of experimental data and prior case reports suggests a pathogenic role for IFN- in SLE.No financial support or conflict of interest to declare     

Clinical outcome of HCV-associated cryoglobulinemic glomerulonephritis following treatment with direct acting antiviral agents: a case-based review

Clinical Rheumatology - Newer treatment protocols involving direct-acting antiviral agents (DAAs) have been associated with high rates of sustained virologic response (SVR) and clinical remission...     

矛盾性栓塞八例临床分析

目的提高对矛盾性栓塞的认识，减少误诊和漏诊。方法采用病例分析和文献复习的方法。结果本组8例肺栓塞合并矛盾性栓塞患者，男性6例、女性2例；平均年龄为47．6岁。3例发生脑血管栓塞，2例为肾动脉栓塞，左心房血栓、下肢动脉和主动脉栓塞各1例；3例发现卵圆孔开放合并右向左分流。确诊矛盾性栓塞是发现嵌顿于动静脉交通处的栓子，其余皆为临床诊断。本组矛盾性栓塞诊断，7例为临床诊断，1例为确诊。结论矛盾性栓塞并非少见，应加强诊断意识。对于静脉血栓栓塞症合并体循环动脉栓塞或不明原因的体循环动脉栓塞应考虑到矛盾性栓塞可能。     

Successful treatment of hepatitis E virus‐associated cryoglobulinemic membranoproliferative glomerulonephritis with ribavirin

Hepatitis E virus genotype‐3 (HEV3) infection can cause chronic hepatitis in immunosuppressed patients and induce extra‐hepatic manifestations, such as neurological symptoms, kidney injuries, and immune‐mediated thrombocytopenia. Very few cases of HEV‐induced kidney manifestations have been reported. Herein, we report, for the first time, a case of de novo membranoproliferative glomerulonephritis that occurred in a kidney transplant patient who developed a chronic HEV3 infection, which was successfully treated with ribavirin.     

8例嗜酸细胞性胃肠炎的临床分析

目的 分析嗜酸细胞性胃肠炎(EG)的临床特点、诊断要点和治疗预后,并探讨其误诊原因.方法 回顾性分析8例EG患者的病史、临床表现、实验室检查、内镜表现和治疗随诊情况.结果 8例EG患者多表现为腹痛、恶心、呕吐、腹泻、低热或腹胀、便秘等非特异性胃肠道症状.外周血和骨髓中嗜酸细胞计数明显增高.腹水为渗出液,可见嗜酸细胞.EG的内镜检查表现没有特异性,多为黏膜片状糜烂、浅溃疡、散在充血斑或出血点,活检可证实大量嗜酸细胞浸润.激素治疗可在1周内迅速缓解症状,并使嗜酸细胞恢复正常.结论 EG临床和内镜表现无特异性,外周血嗜酸细胞、腹水嗜酸细胞增多,尤其是胃肠黏膜组织中嗜酸细胞增多是诊断的关键.     

肺球孢子菌病8例临床分析

目的 研究非疫区感染的肺球孢子病的特点.方法 回顾性分析福建医科大学附属第一医院自2004年10月至2008年6月确诊的肺球孢子菌病8例.结果 8例经病理确诊的肺球孢子菌病患者中,男5例,女3例;平均年龄(42.87±4.93)岁,7例患者无基础疾病,HIV均为阴性.主要有以下临床表现:干咳3例,咳痰5例,胸痛3例,气喘1例,发热3例,头晕、头痛1例.症状轻,体征不明显.实验室检查:血常规白细胞出现异常者3例,痰培养均未见到球孢子菌生长.影像学表现:边缘模糊的结节状密度增高影6例,片状模糊影2例,多出现单侧肺叶,以下肺多见,2例结节见毛刺,4例病变内空洞.病变CT值在30～60 Hu,增强后均无明显强化.病理学表现:慢性肉芽肿性炎症伴多核巨细胞反应,内见球状真菌孢子.PAS染色阳性者6例,6-氨银染色阳性者5例.治疗及转归:1例失访,4例予氟康唑抗感染,2例氟康唑合用两性霉素B雾化吸人治疗,1例伊曲康唑联合两性霉素B雾化吸入、治疗1个月后,病变均有吸收.结论 非疫区可能发生肺球孢子菌病,临床表现轻微,影像学表现无特异性,极易误诊.早期通过肺穿刺活检可助确诊.该病预后较好.     

血栓性血小板减少性紫癜8例临床分析

目的：探讨血栓性血小板减少性紫癜（TTP）的临床特点和疗效。方法：1996年以前,5／8例应用泼尼松,肝素,双嘧达莫等治疗,仅1例缓解,4例死亡,1997年后,3／8例采用血浆置换（PE）加泼尼松,肝素,双嘧达莫等治疗,3例缓解,结果：随访1－3年,2例健康,1例复发,死于肾功能衰竭。结论：TTP为多系统损伤的严重疾病,PE是TTP的首选治疗。