Document Detail

Unmasking of acquired autoimmune C1-inhibitor deficiency by an angiotensin-converting enzyme inhibitor.
MedLine Citation:
PMID:  11345293     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: A majority of angioedema arise from unknown etiologies. Angioedema may also arise from medications or deficiency of C1-esterase inhibitor (C1-INH); either of these may lead to recurrent, sometimes life-threatening attacks of subcutaneous or submucosal edema if the angioedema involves the tongue, throat, or larynx. We describe a patient with unknown acquired C1-INH deficiency, who experienced only mild attacks of angioedema before treatment with an angiotensin-converting enzyme (ACE) inhibitor. This therapy led to life-threatening respiratory distress. OBJECTIVE: To investigate this patient's life-threatening angioedema. METHODS: Serum protein electrophoresis and immunofixation were performed. The titer of anti-C1-inhibitor autoantibody was determined by ELISA, and the specificity of the autoantibody demonstrated by using purified C1-INH to block binding in the ELISA. Finally, fractions from the immunoelectrophoresis gel were tested for C1-INH autoantibody by ELISA. RESULTS: Complement activation was documented by reduced C1-INH, C1q, and C4, and the patient was found to have an autoantibody of IgG2 isotype specific for C1-INH. After discontinuation of the ACE inhibitor, he continued to have decreased C1-INH and positive C1-INH autoantibodies. CONCLUSIONS: This case describes a patient who had a history of mild facial and extremity swelling with abdominal symptoms before ACE inhibitor treatment; this medication resulted in life-threatening respiratory distress. The use of the ACE inhibitor may have unmasked this patient's acquired autoimmune C1-INH deficiency.
G I Kleiner; P Giclas; G Stadtmauer; C Cunningham-Rundles
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Publication Detail:
Type:  Case Reports; Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  Annals of allergy, asthma & immunology : official publication of the American College of Allergy, Asthma, & Immunology     Volume:  86     ISSN:  1081-1206     ISO Abbreviation:  Ann. Allergy Asthma Immunol.     Publication Date:  2001 Apr 
Date Detail:
Created Date:  2001-05-09     Completed Date:  2001-05-21     Revised Date:  2007-11-15    
Medline Journal Info:
Nlm Unique ID:  9503580     Medline TA:  Ann Allergy Asthma Immunol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  461-4     Citation Subset:  IM    
Division of Clinical Immunology, Mount Sinai-New York University Medical Center, New York, USA.
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MeSH Terms
Angioedema / immunology*
Angiotensin-Converting Enzyme Inhibitors / adverse effects*
Antibodies, Monoclonal / immunology
Autoantibodies / immunology
Autoimmune Diseases / immunology*
Blood Protein Electrophoresis
Complement C1 Inactivator Proteins / deficiency*,  immunology*
Immunoglobulin G / analysis,  immunology
Grant Support
Reg. No./Substance:
0/Angiotensin-Converting Enzyme Inhibitors; 0/Antibodies, Monoclonal; 0/Autoantibodies; 0/Complement C1 Inactivator Proteins; 0/Immunoglobulin G

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