title: Chronic granulomatous disease: why an inflammatory disease?
abstract: Chronic granulomatous disease is a primary immunodeficiency caused by mutations in the genes encoding subunits of the phagocytic NADPH oxidase system. Patients can present with severe, recurrent infections and noninfectious conditions. Among the latter, inflammatory manifestations are predominant, especially granulomas and colitis. In this article, we systematically review the possible mechanisms of hyperinflammation in this rare primary immunodeficiency condition and their correlations with clinical aspects.
authors.affiliation: Departamento de Pediatria, Faculdade de Medicina de Ribeirão Preto, Universidade de São Paulo, Ribeirão Preto, SP, Brasil.
authors.forename: P
authors.initials: P
authors.lastname: Roxo-Junior
authors.affiliation: Departamento de Biologia e Farmácia, Faculdade de Medicina de Santa Cruz, Universidade de Santa Cruz do Sul, Santa Cruz do Sul, RS, Brasil.
authors.forename: H M L
authors.initials: HM
authors.lastname: Simão
mesh_terms: D006105:Granulomatous Disease, Chronic / Q000235:genetics / Q000276:immunology / Q000382:microbiology; D006801:Humans; D018836:Inflammation Mediators / Q000502:physiology; D019255:NADPH Oxidases / Q000172:deficiency / Q000235:genetics*; D009504:Neutrophils / Q000276:immunology* / Q000382:microbiology; D017382:Reactive Oxygen Species / Q000276:immunology / Q000378:metabolism
chemical_list: D018836:Inflammation Mediators; D017382:Reactive Oxygen Species; D019255:NADPH Oxidases