title: Acquired deficiency of the inhibitor of the first complement component: presentation, diagnosis, course, and conventional management.
abstract: Acquired deficiency of the inhibitor of the first complement component (C1-INH) is a rare, potentially life-threatening disease whose cause, course, and management are not completely defined. This article analyzes the etiopathogenetic mechanism, the clinical presentation, and the relationship between acquired C1-INH deficiency and lymphoproliferative disorders. Moreover, the authors give an overview of the outcome of the disease and the different therapies proposed to cure it.
authors.affiliation: Department of Internal Medicine, San Giuseppe Hospital-AFaR (Ospedale San Giuseppe), University of Milan, Via San Vittore 12, 20123 Milano, Italy.
authors.forename: Lorenza Chiara
authors.initials: LC
authors.lastname: Zingale
authors.forename: Roberto
authors.initials: R
authors.lastname: Castelli
authors.forename: Andrea
authors.initials: A
authors.lastname: Zanichelli
authors.forename: Marco
authors.initials: M
authors.lastname: Cicardi
mesh_terms: D000799:Angioedema / Q000175:diagnosis* / Q000209:etiology* / Q000503:physiopathology*; D001323:Autoantibodies / Q000276:immunology; D001327:Autoimmune Diseases / Q000276:immunology; D050718:Complement C1 Inhibitor Protein / Q000378:metabolism*; D006801:Humans
chemical_list: D001323:Autoantibodies; D050718:Complement C1 Inhibitor Protein