Angioedema
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In medicine and immunology, angioedema is type of hypersensitivity that is a "swelling involving the deep dermis, subcutaneous, or submucosal tissues, representing localized edema. Angioedema often occurs in the face, lips, tongue, and larynx."[1] Angioedema of sudden onset, as in anaphylaxis, can be a life-threatening respiratory emergency.
Causes
Among patients with recurrent angioedema without urticaria, 38% are idiopathic, 25% are due to deficiency of functional complement C1 inhibitor protein (C1 esterase inhibitor), 16% are related to an environmental exposure (medication, foodstuff, insect bite, other environmental allergen, or physical irritation), and 11% are due to angiotensin-converting enzyme inhibitor treatment.[2]
C1 esterase inhibitor deficiency
Angioedema due to deficiency of functional complement C1 inhibitor protein manifest by edema without urticaria, without pruritis[3][4][5] and may be reduced have reduced d-dimer levels, especially during attacks.[6]
Hereditary angioedema (Hereditary C1 esterase inhibitor deficiency)
Hereditary deficiency is characterized by normal levels of complement C1q and complement C1 inhibitor protein function.[5] Complement C1 inhibitor protein antigen is low in type I and normal in type II.
Acquired angioedema (Acquired C1 esterase inhibitor deficiency)
Acquired C1 esterase inhibitor deficiency is rare.[4][5]
- Type I disease is associated with lymphoproliferative disorders.[5]
- Type II disease is associated with autoantibodies[5] and monoclonal gammopathies[7].
Acquired deficiency is characterized by low levels of complement C1q.[5] Like hereditary angioedema, it has low complement C1 inhibitor protein function.[5] Complement C1 inhibitor protein antigen is low in type I and normal in type II. It also has decreased complement C4[8] unless a paraprotein is present[9].
Treatment includes oral tranexamic acid oral 1 gram 3 times a day. Tranexamic acid is an "inhibitor of plasminogen activation, and at much higher concentrations, a noncompetitive inhibitor of plasmin, i.e., actions similar to aminocaproic acid." It is similar to, but more potent than aminocaproic acid.[10] Concomittent use of warfarin may be needed to prevent embolism and thrombosis.
Insect bite
Angioedema may be due to insect bites.[11]
The risk of subsequent systemic anaphylaxis is 5% to 15% according to a review[11] that cites several case series.[12][13][14][15][16] This risk may be increased in patients with a positive skin test[13] or elevated IgE to bee venom.[17]
Immunotherapy may reduce the size and duration of subsequent episodes.[18]
References
- ↑ Anonymous, (2009) Angioedema (English). Medical Subject Headings. U.S. National Library of Medicine.
- ↑ Zingale LC, Beltrami L, Zanichelli A, et al (October 2006). Angioedema without urticaria: a large clinical survey. CMAJ 175 (9): 1065–70. DOI:10.1503/cmaj.060535. PMID 17060655. PMC 1609157.
- ↑ Agostoni A, Aygören-Pürsün E, Binkley KE, et al (September 2004). Hereditary and acquired angioedema: problems and progress: proceedings of the third C1 esterase inhibitor deficiency workshop and beyond. J. Allergy Clin. Immunol. 114 (3 Suppl): S51–131. DOI:10.1016/j.jaci.2004.06.047. PMID 15356535.
- ↑ 4.0 4.1 Cicardi M, Zingale LC, Pappalardo E, Folcioni A, Agostoni A (July 2003). Autoantibodies and lymphoproliferative diseases in acquired C1-inhibitor deficiencies. Medicine (Baltimore) 82 (4): 274–81. DOI:10.1097/01.md.0000085055.63483.09. PMID 12861105.
- ↑ 5.0 5.1 5.2 5.3 5.4 5.5 5.6 Markovic SN, Inwards DJ, Frigas EA, Phyliky RP (January 2000). Acquired C1 esterase inhibitor deficiency. Ann. Intern. Med. 132 (2): 144–50. PMID 10644276.
- ↑ Cugno M, Zanichelli A, Bellatorre AG, Griffini S, Cicardi M (February 2009). Plasma biomarkers of acute attacks in patients with angioedema due to C1-inhibitor deficiency. Allergy 64 (2): 254–7. DOI:10.1111/j.1398-9995.2008.01859.x. PMID 19076541.
- ↑ Frémeaux-Bacchi V, Guinnepain MT, Cacoub P, et al (August 2002). Prevalence of monoclonal gammopathy in patients presenting with acquired angioedema type 2. Am. J. Med. 113 (3): 194–9. PMID 12208377.
- ↑ Gompels MM, Lock RJ, Morgan JE, Osborne J, Brown A, Virgo PF (February 2002). A multicentre evaluation of the diagnostic efficiency of serological investigations for C1 inhibitor deficiency. J. Clin. Pathol. 55 (2): 145–7. PMID 11865013. PMC 1769585.
- ↑ McLean-Tooke A, Stroud C, Sampson A, Spickett G (May 2007). Falsely normal C4 in a case of acquired C1 esterase inhibitor deficiency. J. Clin. Pathol. 60 (5): 565–6. DOI:10.1136/jcp.2006.041350. PMID 17513516.
- ↑ Anonymous. cyklokapron (tranexamic acid) injection, solution. U.S. National Library of Medicine. Retrieved on 2009-02-19.
- ↑ 11.0 11.1 Severino M, Bonadonna P, Passalacqua G (August 2009). Large local reactions from stinging insects: from epidemiology to management. Curr Opin Allergy Clin Immunol 9 (4): 334–7. DOI:10.1097/ACI.0b013e32832d0668. PMID 19458526.
- ↑ Mauriello PM, Barde SH, Georgitis JW, Reisman RE (October 1984). "Natural history of large local reactions from stinging insects". J. Allergy Clin. Immunol. 74 (4 Pt 1): 494–8. PMID 6491095.
- ↑ 13.0 13.1 Golden DB, Marsh DG, Freidhoff LR, et al. (December 1997). Natural history of Hymenoptera venom sensitivity in adults. J. Allergy Clin. Immunol. 100 (6 Pt 1): 760–6. PMID 9438483.
- ↑ Schuberth KC, Lichtenstein LM, Kagey-Sobotka A, Szklo M, Kwiterovich KA, Valentine MD (April 1982). "An epidemiologic study of insect allergy in children. I. Characteristics of the disease". J. Pediatr. 100 (4): 546–51. PMID 7062201.
- ↑ Schuberth KC, Lichtenstein LM, Kagey-Sobotka A, Szklo M, Kwiterovich KA, Valentine MD (March 1983). "Epidemiologic study of insect allergy in children. II. Effect of accidental stings in allergic children". J. Pediatr. 102 (3): 361–5. PMID 6827407.
- ↑ Reisman RE (September 1992). "Natural history of insect sting allergy: relationship of severity of symptoms of initial sting anaphylaxis to re-sting reactions". J. Allergy Clin. Immunol. 90 (3 Pt 1): 335–9. PMID 1345753.
- ↑ Fernandez J, Soriano V, Mayorga L, Mayor M (February 2005). Natural history of Hymenoptera venom allergy in Eastern Spain. Clin. Exp. Allergy 35 (2): 179–85. DOI:10.1111/j.1365-2222.2005.02169.x. PMID 15725189.
- ↑ Golden DB, Kelly D, Hamilton RG, Craig TJ (June 2009). Venom immunotherapy reduces large local reactions to insect stings. J. Allergy Clin. Immunol. 123 (6): 1371–5. DOI:10.1016/j.jaci.2009.03.017. PMID 19443022.

