Hereditary angioedema (HAE) is an episodic swelling disease with autosomal Mendelian
dominant inheritance. One of the oldest descriptions of the disease is from the 1840s
by Graves, who clearly described patients with episodic angioedema [
[1]
]. In 1882, Quincke described a constellation of angioedema symptoms including peripheral
swelling and self-limited attacks of abdominal pain; the disease is known in Europe
as Quincke's disease [
[2]
]. His original description includes concern about compromise of the airway. Because
the attacks he described usually lasted a matter of hours rather than days, the length
of time we commonly associate with HAE today, we cannot be completely certain that
the attacks described 124 years ago were hereditary angioedema. A few years later
in 1888, Osler described the disease observed in each of five generations of a family
and identified the autosomal dominant inheritance that we now associate with the disease
[
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References
- Clinical lectures on the practice of medicine, 1843.in: Major M.H. Classic descriptions of disease. 3rd ed. Charles C. Thomas, Springfield, IL1955: 623-624
- Concerning the acute localized oedema of the skin. Monatsh. Prakt.Dermat. 1882; I: 129-131
- Hereditary angio-neurotic oedema.Am J Med Sci. 1888; 95: 362-367
- Hereditary angioedema: the clinical syndrome and its management.Ann Intern Med. 1976; 84: 589-593
- Angioedema due to angiotensin-converting enzyme inhibitors.Immunopharmacol. 1999; 44: 21-25
- Effect of sex hormones on the complement-related clinical disorder of hereditary angioedema.Arthritis Rheum. 1979; 22: 1295-1299
- Hereditary angioedemal with exclusive abdominal presentation.An Pediatr (Barc). 2004; 61: 346-347
- Hereditary angioneurotic edema.J Allergy. 1962; 33: 330-341
- A biochemical abnormality in hereditary angioneurotic edema.Am J Med. 1963; 35: 37-44
- Hereditary angioneurotic edems: two genetic varients.Science. 1965; 148: 957-958
- Action of complement in hereditary angioedema: the role of C'1 esterase.J Clin Invest. 1969; 43: 2204-2213
- Angioedema induced by a peptide derived from complement component C2.J Exp Med. 1988; 168: 1685-1698
- Role of the second component of complement (C2) and plasmin in kinin release in hereditary angioneurotic edema (H.A.N.E.) plasma.Trans Assoc Am Physicians. 1977; 90: 174-183
- Urine-histamine levels in patients with hereditary angioedema (HAE).J Allergy Clin Immunol. 1988; 82: 403-406
- Immunoregulatory diseases associated with hereditary angioedema: 1. Clinical manifestations of autoimmune disease.J Allergy Clin Immunol. 1986; 77: 749-757
- Urticaria, angioedema, and autoimmunity.Clin Lab Med. 1997; 17: 559-569
- Autoantibodies and lymphoproliferative diseases in acquired C1-inhibitor deficiencies.Medicine (Baltimore). 2003; 82: 274-281
- Hereditary angioedema. 2. Serologic and cellular abnormalities.J Allergy Clin Immunol. 1986; 77: 758-767
- Methyltestosterone therapy for hereditary episodic edema (hereditary angioneurotic edema).Ann Intern Med. 1960; 53: 739-745
- Treatment of hereditary angioedema with danazol.N Engl J Med. 1976; 295: 1444-1448
- Long-term therapy of hereditary angioedema (HAE). Preventive management with fluoxymesterone and oxymetholone in severely affected males and females.Johns Hopkins Med J. 1974; 135: 391-398
- The effect of synthetic androgens in hereditary angioneurotic edema: alteration of C1 inhibitor and C4 levels.Trans Assoc Am Phys. 1976; 89: 122-132
- Clinical and biochemical effects of stanozolol therapy for hereditary angioedema.J Allergy Clin Immunol. 1981; 68: 181-187
- Monocyte C1-inhibitor synthesis in patients with C1-inhibitor deficiency.Eur J Clin Invest. 1989; 19: 45-52
- Increased expression of C1 inhibitor mRNA in patients with hereditary angioedema treated with danazol.Immunol Lett. 2003; 86: 271-276
- Normal C1 inhibitor mRNA expression level in type 1 hereditary angioedema patients: newly found C1 inhibitor gene mutations.Allergy. 2006; 61: 260-264
- Long-term therapy of hereditary angioedema with danazol.Ann Intern Med. 1980; 93: 809-812
- The long-term safety of danazol in women with hereditary angioedema.Fertil Steril. 1990; 54: 64-72
- Side effects of long-term prophylaxis with attenuated androgens in hereditary angioedema: comparison of treated and untreated patients.J Allergy Clin Immunol. 1997; 99: 194-196
- Adverse effects of danazol prophylaxis on the lipid profiles of patients with hereditary angioedema.J Allergy Clin Immunol. 2005; 115: 864-869
- Oxandrolone treatment of childhood hereditary angioedema.Ann Allergy Asthma Immunol. 2004; 92: 377-378
- Epsilon amino-caproic acid (E-ACA) as a therapeutic agent based on 5 years' clinical experience.Acta Med Scand Suppl. 1966; 448: 1-46
- A case of hereditary angioneurotic edema successfully treated with epsilon-aminocaproic acid: studies on C'1 esterase inhibitor, C'1 activation, plasminogen level and histamine metabolism.Clin Exp Immunol. 1968; 3: 733-745
- Hereditary angio-oedema treated with epsilon aminocaproic acid.Br J Dermatol. 1969; 81: 763-765
- Epsilon aminocaproic acid therapy of hereditary angioneurotic edema. A double-blind study.N Engl J Med. 1972; 286: 808-812
- Tranexamic acid therapy in hereditary angioneurotic edema.N Engl J Med. 1972; 287: 452-454
- Self-administration of C1-inhibitor concentrate in patients with hereditary or acquired angioedema caused by C1-inhibitor deficiency.J Allergy Clin Immunol. 2006; 117: 904-908
- Hereditary angioedema: the use of fresh frozen plasma for prophylaxis in patients undergoing oral surgery.J Allergy Clin Immunol. 1975; 55: 386-393
- A review of 25 patients with hereditary angioedema requiring surgery (see comments).Anesthesiology. 1989; 71: 309-311
- Oral manifestations and dental management of patients with hereditary angioedema.J Oral Pathol Med. 1991; 20: 139-142
- Tranexamic acid: preoperative prophylactic therapy for patients with hereditary angioneurotic edema.J Allergy Clin Immunol. 1977; 60: 38-40
- The efficacy of short-term danazol prophylaxis in hereditary angioedema patients undergoing maxillofacial and dental procedures.J Oral Maxillofac Surg. 1999; 57: 404-408
- Replacement therapy in hereditary angioedema. Successful treatment of two patients with fresh frozen plasma.Lancet. 1969; 1: 326-330
- Replacement therapy in hereditary angioedema.N Engl J Med. 1980; 302: 542-546
- Treatment of acute attacks of hereditary angioedema with C1-inhibitor concentrate.Ann Allergy. 1980; 44: 299-301
- Hereditary angioedema: new findings concerning symptoms, affected organs, and course.Am J Med. 2006; 119: 267-274
- Treatment of hereditary angioedema with a vapor-heated C1 inhibitor concentrate.N Engl J Med. 1996; 334: 1630-1634
- A review of the reported defects in the human C1 exterase inhibitor gene producing hereditary angioedema including four new mutations.Clin Immunol. 2001; 98: 157-163
- C1 inhibitor deficiency: consensus document.Clin Exp Immunol. 2005; 139: 379-394
Farkas H. Hereditary and acquired angioedema: problems and progress: proceedings of the third C1 esterase inhibitor deficiency workshop and beyond. J Allergy Clin Immunol 2004;114(Suppl 3):S51–S131.
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