title: Immune reconstitution inflammatory syndrome of the brain: case illustrations of a challenging entity.
abstract: Immune reconstitution inflammatory syndrome represents a spectrum of clinicopathologic entities encountered in human immunodeficiency virus-infected patients who have received highly active anti-retroviral therapy. The diagnosis is often challenging, treatment options are limited, and the prognosis is variable. To increase awareness and define the clinicopathologic features, we present our experience with 6 probable cases involving the brain, including 1 autopsy. Clinicopathologic review was supplemented by immunohistochemical analysis. There were 5 men and 1 woman, ranging in age from 34 to 47 (mean, 41; SD, 5.39) years. All patients experienced neurologic deterioration (focal deficits in 5/6) after highly active anti-retroviral therapy. All specimens showed a predominance of CD8+ lymphocytic inflammation. Concurrent CNS infections included human immunodeficiency virus encephalitis, progressive multifocal leukoencephalopathy, cryptococcal meningitis, and syphilis. One patient died, 1 was lost to follow-up, 2 improved, and 2 showed no substantial clinical changes. Subtle and overlapping features may preclude a definitive diagnosis. To capture all suspected cases, it is important to consider the possibility of this entity. In this study, the degree of CD8+ inflammation was more pronounced in the single fatal example, and mast cells were not identified in the infiltrates. Although nonspecific, imaging findings may offer clues to early diagnosis.
authors.affiliation: Department of Neuropathology and Ophthalmic Pathology, Armed Forces Institute of Pathology, Washington, District of Columbia 20306-6000, USA. elisabeth.rushing@gmail.com
authors.forename: Elisabeth J
authors.initials: EJ
authors.lastname: Rushing
authors.forename: Angelike
authors.initials: A
authors.lastname: Liappis
authors.forename: James D
authors.initials: JD
authors.lastname: Smirniotopoulos
authors.forename: Alice B
authors.initials: AB
authors.lastname: Smith
authors.forename: James M
authors.initials: JM
authors.lastname: Henry
authors.forename: Yan-Gao
authors.initials: YG
authors.lastname: Man
authors.forename: Ann M
authors.initials: AM
authors.lastname: Nelson
mesh_terms: D000328:Adult; D015703:Antigens, CD / Q000378:metabolism; D023241:Antiretroviral Therapy, Highly Active / Q000379:methods*; D001921:Brain / Q000000981:diagnostic imaging / Q000473:pathology* / Q000821:virology; D002493:Central Nervous System Diseases / Q000209:etiology / Q000473:pathology* / Q000628:therapy* / Q000821:virology; D005260:Female; D006801:Humans; D007249:Inflammation / Q000209:etiology / Q000473:pathology* / Q000628:therapy*; D008279:Magnetic Resonance Imaging; D008297:Male; D008407:Mast Cells / Q000276:immunology / Q000473:pathology; D008875:Middle Aged; D011859:Radiography; D012189:Retrospective Studies
chemical_list: D015703:Antigens, CD