Angioedema: Difference between revisions
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In [[medicine]] and [[immunology]], '''angioedema''' is "swelling involving the deep dermis, subcutaneous, or submucosal tissues, representing localized [[edema]]. Angioedema often occurs in the face, lips, tongue, and larynx."<ref>{{MeSH}}</ref> Angioedema may be due to deficiency of [[complement C1 inhibitor protein]] which may be hereditary or acquired. | In [[medicine]] and [[immunology]], '''angioedema''' is "swelling involving the deep dermis, subcutaneous, or submucosal tissues, representing localized [[edema]]. Angioedema often occurs in the face, lips, tongue, and larynx."<ref>{{MeSH}}</ref> Angioedema may be due to deficiency of [[complement C1 inhibitor protein]] (C1 esterase inhibitor) which may be hereditary or acquired. | ||
==Classification of C1 esterase inhibitor deficiency== | ==Classification of C1 esterase inhibitor deficiency== | ||
Revision as of 08:05, 19 February 2009
In medicine and immunology, angioedema is "swelling involving the deep dermis, subcutaneous, or submucosal tissues, representing localized edema. Angioedema often occurs in the face, lips, tongue, and larynx."[1] Angioedema may be due to deficiency of complement C1 inhibitor protein (C1 esterase inhibitor) which may be hereditary or acquired.
Classification of C1 esterase inhibitor deficiency
Angioedema due to deficiency of complement C1 inhibitor protein manifest by edema without urticaria[2][3] and may be reduced have reduced d-dimer levels, especially during attacks.[4]
Hereditary angioedema (Hereditary C1 esterase inhibitor deficiency)
Hereditary deficiency is characterized by normal levels of complement C1q and complement C1 inhibitor protein function.[3] 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.[2][3]
- Type I disease is associated with lymphoproliferative disorders.[3]
- Type II disease is associated with autoantibodies[3] and monoclonal gammopathies[5].
Acquired deficiency is characterized by low levels of complement C1q.[3] Like hereditary angioedema, it has low complement C1 inhibitor protein function.[3] Complement C1 inhibitor protein antigen is low in type I and normal in type II. It also has decreased complement C4[6] unless a paraprotein is present[7].
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.[8] Concomittent use of warfarin may be needed to prevent embolism and thrombosis.
References
- ↑ Anonymous (2024), Angioedema (English). Medical Subject Headings. U.S. National Library of Medicine.
- ↑ 2.0 2.1 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. Research Blogging.
- ↑ 3.0 3.1 3.2 3.3 3.4 3.5 3.6 Markovic SN, Inwards DJ, Frigas EA, Phyliky RP (January 2000). "Acquired C1 esterase inhibitor deficiency". Ann. Intern. Med. 132 (2): 144–50. PMID 10644276. [e]
- ↑ 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. Research Blogging.
- ↑ 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. [e]
- ↑ 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. [e]
- ↑ 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. Research Blogging.
- ↑ Anonymous. cyklokapron (tranexamic acid) injection, solution. U.S. National Library of Medicine. Retrieved on 2009-02-19.