Acquired deficiency of the inhibitor of the first complement component: presentation, diagnosis, course, and conventional management.
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.
Zingale
Lorenza Chiara
LC
Department of Internal Medicine, San Giuseppe Hospital-AFaR (Ospedale San Giuseppe), University of Milan, Via San Vittore 12, 20123 Milano, Italy.
Castelli
Roberto
R
Zanichelli
Andrea
A
Cicardi
Marco
M
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
D001323:Autoantibodies; D050718:Complement C1 Inhibitor Protein