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Abstract
purpose: In this study, we investigated the clinical and biochemical features and the responses
to treatment of eight patients with auto-antibody-mediated Cl inhibitor (C1-INH) deficiency
and symptoms of angioedema.
patients and methods: In addition to the 8 patients with acquired angioedema (AAE), we also studied 36
subjects with hereditary angioedema (HAE), 15 of them treated with C1-INH plasma concentrate,
and 26 patients with different autoantibodies in their plasma (10 with systemic lupus
erythematosus, 6 with lupus-like anticoagulant, and 10 with chronic liver disease).
Functional C1-INH was measured with the reagent kit of Immuno (Vienna, Austria); C1-INH,
C4, and Clq antigen were determined by radial immunodiffusion; and autoantibodies
to C1-INH were detected by an enzyme-linked immunosorbent assay method.
results: Four patients with AAE had no other diseases, one had breast cancer, one liver hydatidosis,
one Waldenstrom's disease, and one a benign M component. Functional C1-INH levels
were below 30% of normal, and Clq plasma levels were low in seven patients but normal
in one. Autoantibodies to C1-INH were detectable in all eight AAE patients but in
none of the others. Prophylactic treatment with attenuated androgens was successful
in one of four patients, and with antifibrinolytic agents (tranexamic acid) in six
of seven patients. Laryngeal attacks in five patients were treated with C1-INH plasma
concentrate; two patients had marked clinical and biochemical responses. In three,
the symptoms resolved only with high doses, and the biochemical parameters did not
significantly increase.
conclusions: Our results suggest that patients with autoimmune AAE are clinically and biochemically
heterogeneous. They have different responses to treatment that seem to be related
to variable C1-INH consumption.
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References
- C1 inhibitor and hereditary angioneurotic edema.Annu Rev Immunol. 1988; 6: 595-628
- A biochemical abnormality in hereditary angioneurotic edema: absence of serum inhibitor of C1-esterase.Am J Med. 1963; 31: 36-44
- Inherited and acquired C1 inhibitor deficiency: Biological and clinical characteristics in 235 patients.Medicine (Baltimore). 1992; 71: 206-215
- Acquired Cl inhibitor deficiency in lymphosarcoma.Clin Immunol Immunopathol. 1972; 1: 39-52
- Cl inhibitor deficiency: molecular and immunologic basis of hereditary and acquired angioedema.Lab Invest. 1991; 65: 381-388
- Acquired Cl esterase inhibitor deficiency and angioedema: a review.Medicine (Baltimore). 1979; 58: 311-318
- An IgG autoantibody which inactivates Cl inhibitor.Nature. 1986; 313: 722-724
- Autoantibody mediated acquired deficiency of Cl inhibitor.N Engl J Med. 1987; 316: 1360-1366
- Acquired angioedema type II (AAE II): a new autoimmune disease.Clin Res. 1987; 33 ([abstract]): 641A
- Acquired angioedema and anti-Cl-inhibitor autoantibody.Arch Intern Med. 1989; 149: 1213-1214
- Acute consumption of Cl inhibitor in a patient with acquired Cl-inhibitor deficiency syndrome.J Allergy Clin Immunol. 1992; 88: 908-918
- Acquired angioedema with lymphoproliferative disorder: association of C1 inhibitor deficiency with cellular abnormality.Blood. 1976; 48: 567-580
- Acquired Cl inhibitor deficiency associated with antiidiotypic antibody to monoclonal immunoglobulins.N Engl J Med. 1985; 312: 534-540
- Autoantibody facilitated cleavage of C1 inhibitor in autoimmune angioedema.J Clin Invest. 1989; 83: 698-707
- Acquired Cl inhibitor (Cl INH) deficiency type II.J Clin Invest. 1989; 83: 1794-1799
- Acquired angioedema: observations on the mechanism of action of autoantibodies directed against C1 esterase inhibitor.J Allergy Clin Immunol. 1988; 81: 1199-1204
- Treatment of hereditary angioedema with danazol.in: Reversal of clinical and biochemical abnormalities. 3rd ed. N Engl J Med. 295. 1976: 1444-1448
- Long-term treatment of hereditary angioedema with attenuated androgens: a survey of 13 years experience.J Allergy Clin Immunol. 1991; 87: 768-773
- Treatment of acquired C1 inhibitor deficiency with danazol.Ann Intern Med. 1977; 87: 577-578
- Acquired angioedema associated with rectal carcinoma and its response to danazol therapy.J Allergy Clin Immunol. 1978; 62: 217-221
- Acquired angioedema and hypocomplementemia in a patient with myelofibrosis.Acta Med Scand. 1980; 207: 123-125
- Acquired deficiency of the inhibitor of the first component of complement: report of five additional cases and commentary on the syndrome.J Allergy Clin Immunol. 1985; 75: 640-646
- Angioedema with acquired deficiency of the Cl-inhibitor: a constellation of syndromes.Mayo Clin Proc. 1989; 64: 1269-1275
- Long-term prophylaxis with Cl-inhibitor (Cl INH) concentrate in patients with recurrent angioedema caused by hereditary and acquired Cl-inhibitor deficiency.J Allergy Clin Immunol. 1989; 83: 677-682
- A case of hereditary angioneurotic edema, successfully treated with e-aminocaproic acid.Clin Exp Immunol. 1968; 3: 733-745
- Immunochemical quantitation of antigens by immunodiffusion.Immunochemistry. 1965; 2: 235-240
- Acquired Cl inhibitor deficiency with angioedema symptoms in a patient infected with Echinococcus granulosus.Complement. 1985; 2: 133-139
- Heterogeneity of nephritic factor and its identification as an immunoglobulin.in: 3rd ed. Proc Natl Acad Sci USA. 74. 1977: 3980-3983
- An introduction to factor VIII inhibitors: the detection and quantitation.Am J Med. 1991; 91: 1-5
- Acquired inhibitors in malignant and nonmalignant disease states.Am J Med. 1991; 91: 9-13
- Acquired C1 inhibitor deficiency associated with a lupus like anticoagulant activity.Ann Allergy. 1988; 61: 348-350
- The metabolism of Clq in patients with acquired C1 inhibitor deficiency.J Allergy Clin Immunol. 1986; 77: 312-316
- Acquired C1 inhibitor deficiency in a case of lymphosarcoma of the spleen.in: Reversal of complement abnormalities after splenectomy. 3rd ed. Clin Exp Immunol. 36. 1979: 523-531
- Angioneurotic edema with acquired Cl-inhibitor deficiency and autoantibody to C1-inhibitor: response to plasmapheresis and cytotoxic therapy.J Lab Clin Med. 1992; 119: 397-406
- Hepatic function and fibrinolysis in patients with hereditary angioedema undergoing long-term treatment with tranexamic acid.Allergy. 1978; 32: 211-216
- Cl inhibitor concentrate in the therapy of hereditary angioedema.Allergy. 1983; 37: 81-84
- Behavior in vivo of normal and dysfunctional Cl inhibitor in normal subjects and in patients with hereditary angioneurotic edema.J Clin Invest. 1983; 71: 1041-1046
Article info
Publication history
Accepted:
December 23,
1992
Received:
July 1,
1992
Footnotes
☆This work has been supported by the Ministero Universita' Ricerca Scientifica Technologica National Project “Inflammation: Biological and Clinical Aspects,” Rome, Italy.
Identification
Copyright
© 1993 Published by Elsevier Inc.
