Cryoglobulinemia

冷球蛋白血症
  • 文章类型: Case Reports
    感染性心内膜炎是一种罕见但危及生命的疾病,偶尔与不同的免疫学表现有关,包括混合型冷球蛋白血症.这可能导致冷球蛋白血症性血管炎,有可能导致广泛的器官损伤。尽管有些病例强调了感染性心内膜炎和冷球蛋白性血管炎之间的关系,没有针对这种组合进行全面的流行病学评估或最佳治疗策略.我们介绍了一例甲氧西林敏感的金黄色葡萄球菌感染性心内膜炎与冷球蛋白性血管炎相关的病例,并进行了文献综述,以比较类似病例的治疗和结局。我们的患者表现为经典的Meltzer三联征和轻度肾脏受累。冷冻免疫固定证实III型冷球蛋白血症,血清细胞因子显示IL-6水平升高。鉴别诊断包括感染性心内膜炎和慢性活动性丙型肝炎病毒感染。抗生素治疗后症状迅速缓解,确定感染性心内膜炎是冷球蛋白性血管炎的可能原因。我们的案例和文献综述强调,早期识别冷球蛋白性血管炎的病因对于选择适当的治疗方法和预防复发或发病率至关重要。
    Infective endocarditis is a rare but life-threatening condition, occasionally linked to diverse immunologic manifestations, including mixed cryoglobulinemia. This can lead to cryoglobulinemic vasculitis, which has the potential for widespread organ damage. Although some cases have highlighted the relationship between infective endocarditis and cryoglobulinemic vasculitis, no comprehensive epidemiological evaluation or optimal treatment strategies have been advanced for such a combination. We present a case of methicillin-sensitive Staphylococcus aureus infective endocarditis associated with cryoglobulinemic vasculitis and conduct a literature review to compare management and outcomes in similar cases. Our patient presented with classical Meltzer\'s triad and mild renal involvement. Cryoimmunofixation confirmed type III cryoglobulinemia, and serum cytokines showed elevated IL-6 levels. The differential diagnosis included infective endocarditis and chronic active hepatitis C virus infection. Rapid symptom resolution after antibiotic treatment identified infective endocarditis as the likely cause of cryoglobulinemic vasculitis. Our case and review of the literature highlight that early identification of the cause of cryoglobulinemic vasculitis is crucial for selecting appropriate treatment and preventing recurrence or morbidity.
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  • 文章类型: Journal Article
    混合性冷球蛋白血症血管炎(MCV)约90%的病例由慢性丙型肝炎病毒(HCVposMCV)引起,很少由乙型肝炎病毒(HBV)感染引起。或者显然是非传染性的。HCVposMCV仅在约5%的慢性丙型肝炎(CHC)患者中发展,但是到目前为止,尚未发现女性以外的危险因素。我们进行了一项回顾性病例对照研究,调查过去是否活动性HBV感染,由乙型肝炎表面抗原(HBsAg)血清清除和抗核心抗体(HBcAb)阳性定义,可能是发展HCVposMCV的危险因素。HBsAg血清清除的患病率在123例HCVposMCV患者中为48%,在257例CHC患者中为29%(p=0.0003)。多元逻辑回归包括作为变量的性别,出生年份,年龄在HBV检测,肝硬化,和肝细胞癌,证实了HBsAg血清清除与HCVposMCV的关联[调整比值比(OR)2.82,95%置信区间(95%CI)1.73-4.59,p<0.0001]。按性别分层,然而,显示HBsAg血清清除与男性患者的HCVposMCV相关[OR4.63,95%CI2.27-9.48,p<0.0001],而不是女性患者[OR1.85,95%95%CI0.94-3.66,p=0.076]。HBsAg血清清除,更有可能隐匿性HBV感染,是男性CHC患者HCVposMCV的独立危险因素。
    Mixed cryoglobulinemia vasculitis (MCV) is caused in ~90% of cases by chronic hepatitis C virus (HCVposMCV) and more rarely by hepatitis B virus (HBV) infection, or apparently noninfectious. HCVposMCV develops in only ~5% of patients with chronic hepatitis C (CHC), but risk factors other than female gender have not been identified so far. We conducted a retrospective case control study investigating whether past active HBV infection, defined by hepatitis B surface antigen (HBsAg) seroclearance and anti-core antibody (HBcAb) positivity, could be a risk factor for developing HCVposMCV. The prevalence of HBsAg seroclearance was 48% within 123 HCVposMCV patients and 29% within 257 CHC patients (p=0.0003). Multiple logistic regression including as variables gender, birth year, age at HBV testing, cirrhosis, and hepatocellular carcinoma, confirmed an association of HBsAg seroclearance with HCVposMCV [adjusted odds ratio (OR) 2.82, 95% confidence interval (95% CI) 1.73-4.59, p<0.0001]. Stratification by gender, however, showed that HBsAg seroclearance was associated with HCVposMCV in male [OR 4.63, 95% CI 2.27-9.48, p<0.0001] and not in female patients [OR 1.85, 95% 95% CI 0.94-3.66, p=0.076]. HBsAg seroclearance, and more likely occult HBV infection, is an independent risk factor for HCVposMCV in male CHC patients.
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  • 文章类型: Journal Article
    Waldenström巨球蛋白血症(WM)是淋巴瘤的罕见变种,通过IgM副蛋白的存在鉴定为B细胞恶性肿瘤,克隆的浸润,骨髓中的小淋巴浆细胞B细胞,和MYD88L265P突变,在超过90%的病例中观察到。恶性细胞直接侵入淋巴结和脾脏等组织,伴随着与IgM相关的免疫反应,还会导致各种健康并发症,比如血细胞减少症,高粘度,周围神经病变,淀粉样变性,和Bing-Neel综合征.化学免疫疗法历来被认为是WM的首选治疗方法。其中利妥昔单抗和核苷类似物的组合,烷化药物,或蛋白酶体抑制剂在抑制肿瘤生长方面表现出显著的功效。最近的研究提供了证据,布鲁顿酪氨酸激酶抑制剂(BTKI),独立使用或与其他药物联合使用,已被证明在治疗WM中是有效和安全的。这种疾病被认为是不可治愈的,平均预期寿命为10至12岁。
    Waldenström macroglobulinemia (WM) is an infrequent variant of lymphoma, classified as a B-cell malignancy identified by the presence of IgM paraprotein, infiltration of clonal, small lymphoplasmacytic B cells in the bone marrow, and the MYD88 L265P mutation, which is observed in over 90% of cases. The direct invasion of the malignant cells into tissues like lymph nodes and spleen, along with the immune response related to IgM, can also lead to various health complications, such as cytopenias, hyperviscosity, peripheral neuropathy, amyloidosis, and Bing-Neel syndrome. Chemoimmunotherapy has historically been considered the preferred treatment for WM, wherein the combination of rituximab and nucleoside analogs, alkylating drugs, or proteasome inhibitors has exhibited notable efficacy in inhibiting tumor growth. Recent studies have provided evidence that Bruton Tyrosine Kinase inhibitors (BTKI), either used independently or in conjunction with other drugs, have been shown to be effective and safe in the treatment of WM. The disease is considered to be non-curable, with a median life expectancy of 10 to 12 years.
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  • 文章类型: Journal Article
    背景:完成抗病毒治疗后的持续冷球蛋白血症是慢性丙型肝炎患者临床管理的重要考虑因素。我们旨在调查在开始抗病毒治疗时无冷球蛋白血症的慢性丙型肝炎患者血清冷球蛋白血症的发生。
    方法:总共,776例无冷球蛋白血症的患者在完成抗HCV治疗后评估血清冷球蛋白血症。在治疗开始和完成时评估血清冷球蛋白血症沉淀,并分析与慢性丙型肝炎相关的临床实验室因素。
    结果:118名患者在治疗完成后检查血清低温沉淀,8例(4.6%)血清冷球蛋白血症阳性。冷球蛋白血症检测阳性的患者包括较高比例的肝硬化患者(4/50%,p=0.033)和其他器官癌症患者(5/62.5%,p=0.006)比治疗后无冷球蛋白血症迹象的患者。在多变量分析中,肝硬化(比值比[OR]-17.86,95%置信区间[95%CI]-1.79-177.35,p=0.014)和其他器官癌(OR-25.1795%CI-2.59-244.23,p=0.005)在抗病毒治疗后3个月与冷球蛋白血症阳性独立且显著相关.
    结论:抗病毒DAA治疗结束后三个月,8名患者冷球蛋白血症检测呈阳性,代表6.7%的患病率。抗病毒治疗后,肝硬化和其他器官癌与冷球蛋白血症阳性独立且显着相关。进一步研究DAA抗病毒治疗后冷球蛋白血症阳性的原因是必要的。
    BACKGROUND: Persistent cryoglobulinemia after the completion of antiviral treatment is an important consideration of clinical management in chronic hepatitis C patients. We aimed to investigate the occurrence of serum cryoglobulinemia in chronic hepatitis C patients without cryoglobulinemia at the initiation of antiviral treatment.
    METHODS: In total, 776 patients without cryoglobulinemia were assessed for serum cryoglobulinemia after the completion of anti-HCV treatment. Serum cryoglobulinemia precipitation was assessed upon both the initiation and the completion of the treatment and analyzed for the clinical laboratory factors associated with chronic hepatitis C.
    RESULTS: One hundred eighteen (118) patients were checked for serum cryo-precipitation after the completion of the treatment, and eight patients (4.6%) were positive for serum cryoglobulinemia. The patients who tested positive for cryoglobulinemia included a higher proportion of liver cirrhosis patients (4/50%, p = 0.033) and other organ cancer patients (5/62.5%, p = 0.006) than patients who showed no signs of cryoglobulinemia after treatment. In a multivariate analysis, liver cirrhosis (odds ratio [OR]-17.86, 95% confidence interval [95% CI]-1.79-177.35, p = 0.014) and other organ cancer (OR-25.17 95% CI-2.59-244.23, p = 0.005) were independently and significantly associated with positive cryoglobulinemia 3 months after antiviral treatment.
    CONCLUSIONS: Three months after the antiviral DAA therapy had concluded, eight patients tested positive for cryoglobulinemia, representing a 6.7% prevalence. Liver cirrhosis and other organ cancer were independently and significantly associated with positive cryoglobulinemia after antiviral treatment. Further investigation into the causes of positive cryoglobulinemia after DAA antiviral therapy is warranted.
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  • 文章类型: Case Reports
    我们报告了一名年轻患者的观察结果,该患者表现为严重的1型冷球蛋白血症性血管炎,显示出具有临床意义的单克隆丙种球蛋白病。美法仑-沙利度胺泼尼松治疗可改善症状。早期诊断可以防止严重的组织损伤。
    单克隆丙种球蛋白病包括多种临床形式。只有癌变的形式,多发性骨髓瘤(MM),根据特定的诊断标准进行治疗。一个新的临床实体,临床意义的单克隆丙种球蛋白病(MGCS),由于需要特殊治疗,因此值得特别注意。它涉及具有不符合MM标准的潜在严重器官受累迹象的患者。我们介绍了一名34岁的马达加斯加妇女,患有与非癌性单克隆丙种球蛋白病相关的严重I型冷球蛋白血症性血管炎,沙利度胺治疗后显示良好的结果。症状包括脚趾坏死,左小腿严重溃疡持续3个月,和长袜般的美感。调查显示单克隆丙种球蛋白病为30.1g/L,蛋白尿在1克/24小时,6%的髓质浆细胞,和循环的Igκ型冷球蛋白。CRAB标准(贫血,高钙血症,肾功能不全,和骨质溶解)不存在。用沙利度胺治疗,结合皮质类固醇和局部护理4个月,导致溃疡愈合,审美障碍的消失,丙种球蛋白持续正常化。我们的案例强调了MGCS特定治疗的重要性。
    UNASSIGNED: We report an observation of a young patient presenting with severe type 1 cryoglobulinemic vasculitis revealing a monoclonal gammopathy of clinical significant. Treatment with Melphalan-Thalidomide Prednisone improved the symptoms. Early diagnosis would prevent serious tissue damage.
    UNASSIGNED: Monoclonal gammopathy encompass diverse clinical forms. Only the cancerous form, multiple myeloma (MM), is treated based on specific diagnostic criteria. A new clinical entity, monoclonal gammopathy of clinical significance (MGCS), warrants special attention due to its need for specific treatment. It involves patients with signs of potentially severe organ involvement that do not meet MM criteria. We present the case of a 34-year-old Malagasy woman with severe type I cryoglobulinemic vasculitis associated with noncancerous monoclonal gammopathy, showing a favorable outcome after treatment with Thalidomide. Symptoms included toe necrosis, a severe ulcer on the left calf evolving for 3 months, and stocking-like dysesthesias. Investigations revealed monoclonal gammopathy at 30.1 g/L, proteinuria at 1 g/24 h, medullary plasma cell at 6%, and circulating cryoglobulin of Ig kappa type. CRAB criteria (anemia, hypercalcemia, renal insufficiency, and osteolysis) were absent. Treatment with Thalidomide, combined with corticosteroids and local care for 4 months, resulted in ulcer healing, disappearance of dysesthesias, and persistent normalization of gammaglobulin. Our case underscores the importance of specific treatment for MGCS.
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  • 文章类型: Journal Article
    HCV及其后遗症的治疗主要基于干扰素(IFN)。然而,由于其免疫刺激作用,这与显著的不良事件相关.自从他们的介绍,直接作用的抗病毒药物(DAA),已成为治疗HCV及其并发症(包括混合型冷球蛋白性血管炎(MCV))的标准护理。尽管实现持续的病毒应答(SVR),有许多报道描述了不受欢迎的并发症,如肝细胞和血液系统恶性肿瘤以及复发。由多种因素引起的长时间炎症,会导致DNA损伤并影响BAFF和4月,作为B细胞增殖的标志物。我们比较,头对头,HCV-MCV治疗的三种抗病毒方案关于治疗反应和复发,基于聚乙二醇干扰素α和游离方案的BAFF和APRIL水平(索非布韦+利巴韦林;SOF-RIBA,Sofosbuvir+Daclatasvir;SOF-DACLA)。关于临床反应HCV-MCV和SVR;在3种不同的治疗方案中没有发现显著差异,这也是使用IFN的独立形式。我们发现基于IFN和游离方案的DNA损伤之间没有显着差异,DNA修复的标记,或BAFF和4月的水平。然而,个体化药物间比较显示出许多差异.那些用基于IFN的方案治疗的人显示出降低的DNA损伤水平,而另外两个无IFN组的DNA损伤增加,是SOF-DACLA组最差的。在SOF-DACLA组中,3种方案的随访期间BAFF水平升高,效果最好(24周时降低)。在SOF-RIBA,CG在随访期间明显复发。我们使用基于IFN的方案治疗的患者均未出现明显的临床实验室复发。那些接受无IFNDAA的人显示出统计学上显着的体质表现复发。我们的发现表明,基于IFN的方案可有效治疗HCV-MCV,类似于无IFN方案。他们表现出低水平的DNA损伤和修复。我们相信我们的发现可以为淋巴增生的过程提供解释,恶性肿瘤的发生,并通过揭示这种可能的机制而复发。
    The treatment of HCV and its sequelae are used to be predominantly based on Interferon (IFN). However, this was associated with significant adverse events as a result of its immunostimulant capabilities. Since their introduction, the directly acting antiviral drugs (DAAs), have become the standard of care to treat of HCV and its complications including mixed cryoglobulinemic vasculitis (MCV). In spite of achieving sustained viral response (SVR), there appeared many reports describing unwelcome complications such as hepatocellular and hematological malignancies as well as relapses. Prolonged inflammation induced by a multitude of factors, can lead to DNA damage and affects BAFF and APRIL, which serve as markers of B-cell proliferation. We compared, head-to-head, three antiviral protocols for HCV-MCV treatment As regards the treatment response and relapse, levels of BAFF and APRIL among pegylated interferon α-based and free regimens (Sofosbuvir + Ribavirin; SOF-RIBA, Sofosbuvir + Daclatasvir; SOF-DACLA). Regarding clinical response HCV-MCV and SVR; no significant differences could be identified among the 3 different treatment protocols, and this was also independent form using IFN. We found no significant differences between IFN-based and free regimens DNA damage, markers of DNA repair, or levels of BAFF and APRIL. However, individualized drug-to-drug comparisons showed many differences. Those who were treated with IFN-based protocol showed decreased levels of DNA damage, while the other two IFN-free groups showed increased DNA damage, being the worst in SOF-DACLA group. There were increased levels of BAFF through follow-up periods in the 3 protocols being the best in SOF-DACLA group (decreased at 24 weeks). In SOF-RIBA, CGs relapsed significantly during the follow-up period. None of our patients who were treated with IFN-based protocol had significant clinico-laboratory relapse. Those who received IFN-free DAAs showed a statistically significant relapse of constitutional manifestations. Our findings suggest that IFN-based protocols are effective in treating HCV-MCV similar to IFN-free protocols. They showed lower levels of DNA damage and repair. We believe that our findings may offer an explanation for the process of lymphoproliferation, occurrence of malignancies, and relapses by shedding light on such possible mechanisms.
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  • 文章类型: Journal Article
    肾脏通常是全身性血管炎(SV)的目标,以许多不同的形式受到影响,并代表潜在的多器官疾病的可能前哨。肾活检仍是鉴定的金标准,这些疾病的表征和分类,由于光学显微镜(LM)的联合应用,解决了复杂的鉴别诊断,免疫荧光(IF)和电子显微镜(EM)。由于肾血管炎分类系统的复杂性逐渐增加(例如pauci免疫vs免疫复合物相关形式),临床病理方法是强制性的,并且需要足够的肾病理学技术和解释性专业知识,以确保为我们的患者提供最佳的护理标准.在这个复杂的背景下,本综述旨在总结当前肾血管炎的知识和挑战,揭示了数字病理学在这种环境中的潜在作用,从创建轴辐式网络到人工智能(AI)工具的未来应用,以帮助诊断和评分/分类过程。
    Kidneys are often targets of systemic vasculitis (SVs), being affected in many different forms and representing a possible sentinel of an underlying multi-organ condition. Renal biopsy still remains the gold standard for the identification, characterization and classification of these diseases, solving complex differential diagnosis thanks to the combined application of light microscopy (LM), immunofluorescence (IF) and electron microscopy (EM). Due to the progressively increasing complexity of renal vasculitis classification systems (e.g. pauci-immune vs immune complex related forms), a clinico-pathological approach is mandatory and adequate technical and interpretative expertise in nephropathology is required to ensure the best standard of care for our patients. In this complex background, the present review aims at summarising the current knowledge and challenges in the world of renal vasculitis, unveiling the potential role of the introduction of digital pathology in this setting, from the creation of hub-spoke networks to the future application of artificial intelligence (AI) tools to aid in the diagnostic and scoring/classification process.
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  • 文章类型: Case Reports
    本研究提出了2例II型混合型冷球蛋白血症。一个案例是必不可少的,而另一种可能与乙型肝炎病毒(HBV)感染有关。两名患者的单克隆IgMκ检测呈阳性,但MyD88突变阴性.他们对利妥昔单抗联合糖皮质激素方案表现出耐药性,但对BTK抑制剂反应积极。这些病例突出了BTK抑制剂在治疗无MyD88突变的难治性II型冷球蛋白血症中的显着有效性。首例患者在开始伊布替尼后一个月内实现肾病综合征的快速完全缓解,随着冷球蛋白水平和异常克隆细胞的显着降低。第二名患者在开始奥列拉布替尼后三天内皮疹迅速消失,伤口愈合加速,伴随着C反应蛋白的减少。然而,12个月随访期间冷球蛋白水平没有降低.这些发现表明BTK抑制剂通过不同的机制在II型冷球蛋白血症中的不同作用机制。
    This study presents two cases of type II mixed cryoglobulinemia. One case is essential, while the other is presumably associated with hepatitis B virus (HBV) infection. Both patients tested positive for monoclonal IgMκ, but negative for MyD88 mutation. They showed resistance to rituximab combined with a glucosteroid regimen, but responded positively to BTK inhibitors. These cases highlight the remarkable effectiveness of BTK inhibitors in treating refractory type II cryoglobulinemia without MyD88 mutation. The first patient achieved rapid complete remission of nephrotic syndrome within one month of starting ibrutinib, along with a significant reduction in cryoglobulin levels and abnormal clonal cells. The second patient had a rapid disappearance of rash within three days and accelerated wound healing within one week of initiating orelabrutinib, accompanied by a reduction in C-reactive protein. However, there was no reduction in cryoglobulin levels during the 12-month follow-up. These findings suggest varied mechanisms of action of BTK inhibitors in type II cryoglobulinemia through different mechanisms.
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  • 文章类型: Journal Article
    背景:冷球蛋白血症伴肺部受累是罕见的,及其特点,放射学发现,结果仍然知之甚少。
    方法:本回顾性研究纳入北京协和医院491例冷球蛋白血症患者的10例肺部受累患者。我们分析了特点,肺部受累患者的放射学特征和管理,并与非肺部受累的冷球蛋白血症进行比较。
    结果:10例肺部受累患者(2例男性;中位年龄,53年)包括3例I型冷球蛋白血症患者和7例混合性冷球蛋白血症患者。10例患者均为IgM同种型冷球蛋白血症。所有I型患者均继发于B细胞非霍奇金淋巴瘤。四个混合患者是必不可少的,其余患者继发于感染(n=2)和系统性红斑狼疮(n=1),分别。六名患者有其他受影响的器官,包括皮肤(60%),肾脏(50%),周围神经(30%),接头(20%),心脏(20%)。肺部症状包括呼吸困难(50%),干咳(30%),胸闷(30%),咯血(10%)。胸部计算机断层扫描(CT)显示弥漫性毛玻璃混浊(80%),结节(40%),胸腔积液(30%),和网状(20%)。两名患者经历了危及生命的弥漫性肺泡出血。五名患者接受了基于皮质类固醇的方案,4人接受了以利妥昔单抗为基础的治疗方案.所有以利妥昔单抗为基础的患者均达到临床缓解。估计的两年总生存率(OS)为40%。与冷球蛋白血症的非肺部受累患者相比,肺部受累患者的OS和无进展生存期明显更差(P<0.0001)。
    结论:对于冷球蛋白血症和胸部CT显示的浸润而没有其他解释的患者,应高度怀疑肺部受累的诊断。肺部受累患者预后不良。基于利妥昔单抗的治疗可以改善结果。
    BACKGROUND: Cryoglobulinemia with pulmonary involvement is rare, and its characteristics, radiological findings, and outcomes are still poorly understood.
    METHODS: Ten patients with pulmonary involvement of 491 cryoglobulinemia patients at Peking Union Medical College Hospital were enrolled in this retrospective study. We analyzed the characteristics, radiological features and management of pulmonary involvement patients, and compared with those of non-pulmonary involvement with cryoglobulinemia.
    RESULTS: The 10 patients with pulmonary involvement (2 males; median age, 53 years) included three patients with type I cryoglobulinemia and seven patients with mixed cryoglobulinemia. All of 10 patients were IgM isotype cryoglobulinemia. All type I patients were secondary to B-cell non-Hodgkin lymphoma. Four mixed patients were essential, and the remaining patients were secondary to infections (n = 2) and systemic lupus erythematosus (n = 1), respectively. Six patients had additional affected organs, including skin (60%), kidney (50%), peripheral nerves (30%), joints (20%), and heart (20%). The pulmonary symptoms included dyspnea (50%), dry cough (30%), chest tightness (30%), and hemoptysis (10%). Chest computed tomography (CT) showed diffuse ground-glass opacity (80%), nodules (40%), pleural effusions (30%), and reticulation (20%). Two patients experienced life-threatening diffuse alveolar hemorrhage. Five patients received corticosteroid-based regimens, and four received rituximab-based regimens. All patients on rituximab-based regimens achieved clinical remission. The estimated two-year overall survival (OS) was 40%. Patients with pulmonary involvement had significantly worse OS and progression-free survival than non-pulmonary involvement patients of cryoglobulinemia (P < 0.0001).
    CONCLUSIONS: A diagnosis of pulmonary involvement should be highly suspected for patients with cryoglobulinemia and chest CT-indicated infiltrates without other explanations. Patients with pulmonary involvement had a poor prognosis. Rituximab-based treatment may improve the outcome.
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  • 文章类型: Journal Article
    慢性丙型肝炎病毒(HCV)感染的特点是各种肝外表现,周围神经病变(PN)是最常见的,特别是当混合性冷球蛋白血症(MCG)存在。在缺乏MCG的情况下,HCV相关PN的患病率和危险因素在很大程度上是未知的。我们进行了一个前瞻性的,单中心研究,在没有MCG的情况下检查HCV相关神经病的患病率和可逆性。在HCV治疗开始之前和持续病毒学缓解(SVR)后1年,通过皮肤活检和神经电图(ENG)评估表皮中的神经纤维密度。包括40名HCV感染者(9名HIV共感染),没有其他神经元伤害因素;由于存在糖尿病,其他4名HCV单感染和3名HIV共感染个体被排除在外。B12不足,或神经毒性药物。还招募了12个没有神经元损伤条件的连续对照;由于符合排除标准,另外8个被排除在外。4例患者有多发性神经病的ENG征象(2例HCV单发和2例HIV合并感染),而另外7例(5例HCV单感染和2例HIV共感染)有单一神经病变的迹象,导致单感染和共感染的PN患病率为22.5%和44%,分别(p值0.179)。两名患有HCV单一感染和多发性神经病的患者和一名患有尺神经损伤的患者在SVR后1年表现出ENG改善。关于表皮内神经密度,HCV感染,不管艾滋病毒共同感染,与SVR后1年改善的较低表皮内神经元密度相关(HCV的p值0.0002和HCV/HIV共感染患者的p值0.0326)。PN在HCV感染中很常见;成功根除HCV导致PN改善。
    Chronic hepatitis C virus (HCV) infection is characterized by a variety of extra-hepatic manifestations; peripheral neuropathy (PN) is one of the most common, especially when mixed cryoglobulinemia (MCG) is present. The prevalence and risk factors of HCV-related PN in the absence of MCG are largely unknown. We conducted a prospective, single-center study, examining the prevalence and reversibility of HCV-associated neuropathy in the absence of MCG. Nerve fiber density in the epidermis was evaluated through skin biopsy and electroneurography (ENG) before HCV-treatment initiation and 1 year post sustained virological remission (SVR). Forty HCV-infected individuals (nine HIV co-infected) with no other neuron-harming factors were included; four other HCV mono- and three HIV co-infected individuals were excluded due to presence of diabetes, B12 insufficiency, or neurotoxic drugs. Twelve consecutive controls with no neuron-harming conditions were also recruited; eight more were excluded due to meeting exclusion criteria. Four patients had ENG signs of polyneuropathy (two with HCV mono- and two with HIV co-infection), while seven more (five with HCV mono- and two with HIV co-infection) had signs of mono-neuropathy, leading to PN prevalences of 22.5% and 44% for mono- and co-infection, respectively (p value 0.179). The two patients with HCV mono-infection and polyneuropathy and the one with ulnar nerve damage showed ENG improvement 1 year post SVR. Regarding intraepidermal nerve density, HCV infection, irrespective of HIV co-infection, was correlated with a lower intraepidermal neuron density that improved 1 year post SVR (p value 0.0002 for HCV and 0.0326 for HCV/HIV co-infected patients). PN is common in HCV infection; successful eradication of HCV leads to PN improvement.
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