title: Central nervous system granulomastous phlebitis with limited extracranial involvement of the heart and lungs: An autopsy case.
abstract: Primary angiitis of the central nervous system is a rare condition, usually with an insidious onset. There is a wide variety of histological types (granulomatous, lymphocytic or necrotizing vasculitis) and types of vessel involved (arteries, veins or both). Most cases are idiopathic. We describe a first case of idiopathic granulomatous central nervous system phlebitis with additional limited involvement of the heart and lung, exclusively affecting small and medium sized veins in a 22-year-old woman, presenting as a sub acute headache. The reasons for this peculiar limitation of inflammation to the veins and the involvement of the heart and lungs are unknown.
authors.affiliation: Institute of Pathology, Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia.
authors.forename: Jernej
authors.initials: J
authors.lastname: Mlakar
authors.affiliation: Department of Infectious Diseases, University Medical Centre Ljubljana, Ljubljana, Slovenia.
authors.forename: Jerneja Videčnik
authors.initials: JV
authors.lastname: Zorman
authors.affiliation: Department of Infectious Diseases, University Medical Centre Ljubljana, Ljubljana, Slovenia.
authors.forename: Mojca
authors.initials: M
authors.lastname: Matičič
authors.affiliation: Department of Radiology, University Medical Centre Ljubljana, Ljubljana, Slovenia.
authors.forename: Matej
authors.initials: M
authors.lastname: Vrabec
authors.affiliation: Institute of Forensic Medicine, Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia.
authors.forename: Armin
authors.initials: A
authors.lastname: Alibegović
authors.affiliation: Institute of Pathology, Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia.
authors.forename: Mara
authors.identifier: http://orcid.org/0000-0002-0443-1217
authors.initials: M
authors.lastname: Popović
mesh_terms: D001344:Autopsy; D001929:Brain Edema / Q000209:etiology / Q000473:pathology; D002493:Central Nervous System Diseases / Q000473:pathology*; D002550:Cerebral Veins / Q000473:pathology; D017809:Fatal Outcome; D005260:Female; D006099:Granuloma / Q000150:complications / Q000473:pathology*; D006261:Headache / Q000209:etiology; D006801:Humans; D008168:Lung / Q000473:pathology*; D009206:Myocardium / Q000473:pathology*; D010689:Phlebitis / Q000209:etiology / Q000473:pathology*; D020293:Vasculitis, Central Nervous System / Q000150:complications / Q000473:pathology; D055815:Young Adult
keywords: central nervous system; granulomatous angiitis; heart; lung; phlebitis