Acquired C1-inhibitor deficiency in a patient with systemic lupus erythematosus: a case report and review of the literature.
The C1-inhibitor (C1-INH) is an important member of the serpin family which inhibits the first component of the human complement system and controls contact activation of the coagulation and kinin system. An acquired form of C1-INH deficiency was recognized and classified as type I, which is characterized by accelerated catabolism of C1-INH, whereas type II is defined by the presence of an autoantibody directed against the C1 inhibitor molecule. This study reports the case of a 32-year-old woman with systemic lupus erythematosus (SLE) who experienced recurrent angioedema because of an acquired C1-INH deficiency. The relevant literature is reviewed.
Nettis
E
E
Department of Medicine, Immunology and Infectious Diseases, Service of Allergy and Clinical Immunology, University of Bari Medical School, Bari, Italy. e.nettis@allergy.uniba.it
Colanardi
M C
MC
Loria
M P
MP
Vacca
A
A
D000328:Adult; D000799:Angioedema / Q000150:complications* / Q000188:drug therapy / Q000276:immunology; D050718:Complement C1 Inhibitor Protein / Q000032:analysis*; D015941:Complement Hemolytic Activity Assay; D003613:Danazol / Q000627:therapeutic use; D005260:Female; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000188:drug therapy / Q000276:immunology*; D010328:Patch Tests
D050718:Complement C1 Inhibitor Protein; D003613:Danazol