title: Orbital Pseudotumor as a Rare Extrahepatic Manifestation of Hepatitis C Infection.
abstract: 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.
authors.affiliation: Division of Gastroenterology and Hepatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland.
authors.forename: Benjamin
authors.initials: B
authors.lastname: Misselwitz
authors.affiliation: Division of Gastroenterology and Hepatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland.
authors.forename: Jana
authors.initials: J
authors.lastname: Epprecht
authors.affiliation: Division of Gastroenterology and Hepatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland.
authors.forename: Joachim
authors.initials: J
authors.lastname: Mertens
authors.affiliation: Division of Gastroenterology and Hepatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland.
authors.forename: Luc
authors.initials: L
authors.lastname: Biedermann
authors.affiliation: Division of Gastroenterology and Hepatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland.
authors.forename: Michael
authors.initials: M
authors.lastname: Scharl
authors.affiliation: Division of Pathology, University Hospital Zurich, University of Zurich, Zurich, Switzerland.
authors.forename: Eugenia
authors.initials: E
authors.lastname: Haralambieva
authors.affiliation: Division of Neurology, University Hospital Zurich, University of Zurich, Zurich, Switzerland.
authors.forename: Andreas
authors.initials: A
authors.lastname: Lutterotti
authors.affiliation: Division of Neurology, University Hospital Zurich, University of Zurich, Zurich, Switzerland; Division of Ophthalmology, University Hospital Zurich, University of Zurich, Zurich, Switzerland.
authors.forename: Konrad P
authors.initials: KP
authors.lastname: Weber
authors.affiliation: Division of Gastroenterology and Hepatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland.
authors.forename: Beat
authors.initials: B
authors.lastname: Müllhaupt
authors.affiliation: Division of Ophthalmology, University Hospital Zurich, University of Zurich, Zurich, Switzerland.
authors.forename: Karla
authors.initials: K
authors.lastname: Chaloupka
keywords: Extrahepatic manifestation; Hepatitis C; Idiopathic orbital inflammation; Lymphocytic dacryoadenitis; Orbital pseudotumor