Mazzaro Cesare, Dal Maso Luigino, Urraro Teresa, Mauro Endri, Castelnovo Laura, Casarin Pietro, Monti Giuseppe, Gattei Valter, Zignego Anna Linda, Pozzato Gabriele
Clinical and Experimental Onco-Hematology Unit, CRO Aviano National Cancer Institute, Aviano, Italy.
Epidemiology and Biostatistics Unit, CRO-Aviano National Cancer Institute, Aviano, Italy.
Dig Liver Dis. 2016 Jul;48(7):780-4. doi: 10.1016/j.dld.2016.03.018. Epub 2016 Apr 2.
Cryoglobulinemic vasculitis (CV) related to Hepatitis-B Virus (HBV) is rare and its treatment is ill-defined.
To describe clinical and treatment characteristics of HBV-related CV patients. In addition, the efficacy of treatment with antiviral agent nucleotide (NUC), including Entecavir, Adefovir, and Lamivudine, was explored.
In four Italian centres, 17 HBV-positive CV patients (median age 56 years, range 45-70) were enrolled.
The extrahepatic manifestations were: purpura (100%), arthralgias (71%), peripheral neuropathy (29%), chronic hepatitis (47%), liver cirrhosis (29%), and glomerulonephritis (18%). Mixed cryoglobulinemias were type II (88%) and type III (12%). The median cryocrit was 3% (range 1-14), rheumatoid factor was 200U/L (range 20-5850), C4 was 12mg/dl (range 2-31), ALT 71U/L (range 36-114). All patients were HBsAg-positive and 80% anti-HbeAg-positive. At enrollment, they were treated with steroids (eight), Entecavir (five), Alpha-IFN (two), Adefovir and Lamivudine (one each). After NUC treatment, no disease progression was observed and, in all patients, HBV-DNA became undetectable. Moreover, a regression of purpura and a reduction of cryocrit were observed. Four patients died during therapy, two of kidney failure and two of liver cirrhosis.
NUC therapy appeared to be safe and effective in CV-related HBV.
与乙型肝炎病毒（HBV）相关的冷球蛋白血症性血管炎（CV）较为罕见，其治疗方法尚不明确。
描述HBV相关CV患者的临床和治疗特征。此外，探讨了核苷类抗病毒药物（NUC），包括恩替卡韦、阿德福韦和拉米夫定的治疗效果。
在四个意大利中心，纳入了17例HBV阳性的CV患者（中位年龄56岁，范围45 - 70岁）。
肝外表现为：紫癜（100%）、关节痛（71%）、周围神经病变（29%）、慢性肝炎（47%）、肝硬化（29%）和肾小球肾炎（18%）。混合性冷球蛋白血症为II型（88%）和III型（12%）。冷球蛋白血症中位数为3%（范围1 - 14），类风湿因子为200U/L（范围20 - 5850），C4为12mg/dl（范围2 - 31），谷丙转氨酶（ALT）为71U/L（范围36 - 114）。所有患者HBsAg均为阳性，80%抗HbeAg阳性。入组时，他们接受了类固醇治疗（8例）、恩替卡韦治疗（5例）、α干扰素治疗（2例）、阿德福韦和拉米夫定治疗（各1例）。接受NUC治疗后，未观察到疾病进展，所有患者的HBV - DNA均检测不到。此外，观察到紫癜消退和冷球蛋白血症降低。4例患者在治疗期间死亡，2例死于肾衰竭，2例死于肝硬化。
NUC治疗在与CV相关的HBV感染中似乎安全有效。
