Misselwitz Benjamin, Epprecht Jana, Mertens Joachim, Biedermann Luc, Scharl Michael, Haralambieva Eugenia, Lutterotti Andreas, Weber Konrad P, Müllhaupt Beat, Chaloupka Karla
Division of Gastroenterology and Hepatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland.
Division of Pathology, University Hospital Zurich, University of Zurich, Zurich, Switzerland.
Case Rep Gastroenterol. 2016 May 19;10(1):108-14. doi: 10.1159/000444011. eCollection 2016 Jan-Apr.
Hepatitis C is frequently accompanied by immune-related extrahepatic manifestations affecting the skin, kidneys, central and peripheral nervous system and exocrine glands. We present the case of a 40-year-old man with left-sided ptosis, exophthalmos and headache. MRI demonstrated left-sided orbital pseudotumor with lacrimal and retro-orbital contrast enhancement extending to the cavernous sinus and the vestibulocochlear nerve. Immunological tests of serum and cerebrospinal fluid identified hepatitis C virus (HCV) as a potential causative agent but did not indicate any additional infectious, malignant or immunological disorder. Hepatological evaluation revealed no signs of advanced liver disease. After initial spontaneous improvement, the patient subsequently developed vestibulocochlear failure with gait disorder, tinnitus and transient left-parietal sensory loss. Lacrimal biopsy demonstrated lymphocytic infiltrate, prompting steroid treatment. After initial improvement, steroids could not be tapered below 40 mg daily for several months due to recurrent symptoms. Twelve months after the initial presentation, the patient's chronic HCV infection was successfully treated with sofosbuvir, simeprevir and ribavirin and he remains now free of symptoms without steroids. In patients with chronic hepatitis C, lymphocytic infiltrate of the salivary and lacrimal glands is a frequent phenomenon. However, the extent of the lymphocytic infiltrate beyond the lacrimal gland to the tip of the orbit, cavernous sinus and vestibulocochlear nerve as in our patient is highly unusual. For all symptomatic extrahepatic manifestations of hepatitis C infection, treatment of HCV as the underlying immune stimulus is recommended, and it helped to control the symptoms in our patient. In addition, long-term follow-up for recurrent lymphocyte infiltrate and development of lymphoma is warranted.
丙型肝炎常伴有免疫相关的肝外表现,累及皮肤、肾脏、中枢和周围神经系统以及外分泌腺。我们报告一例40岁男性,有左侧上睑下垂、眼球突出和头痛症状。磁共振成像(MRI)显示左侧眼眶假瘤,泪腺和眶后有对比增强,延伸至海绵窦和前庭蜗神经。血清和脑脊液的免疫学检查确定丙型肝炎病毒(HCV)为潜在病原体,但未显示任何其他感染性、恶性或免疫性疾病。肝脏学评估未发现晚期肝病迹象。在最初自发改善后,患者随后出现前庭蜗功能衰竭,伴有步态障碍、耳鸣和短暂的左侧顶叶感觉丧失。泪腺活检显示淋巴细胞浸润,促使使用类固醇治疗。在最初改善后,由于症状反复,类固醇在数月内无法减至每日40毫克以下。首次出现症状12个月后,患者的慢性HCV感染用索磷布韦、西米普明和利巴韦林成功治疗,目前在未使用类固醇的情况下无症状。在慢性丙型肝炎患者中,唾液腺和泪腺的淋巴细胞浸润是常见现象。然而,如我们的患者那样,淋巴细胞浸润范围超出泪腺至眶尖、海绵窦和前庭蜗神经的情况非常罕见。对于丙型肝炎感染的所有有症状肝外表现,建议将HCV作为潜在免疫刺激因素进行治疗,这有助于控制我们患者的症状。此外,有必要对复发性淋巴细胞浸润和淋巴瘤的发生进行长期随访。