Angioedema: Difference between revisions

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imported>Robert Badgett
imported>Robert Badgett
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* Type II disease is associated with autoantibodies<ref name="pmid10644276"/> and monoclonal gammopathies<ref name="pmid12208377">{{cite journal |author=Frémeaux-Bacchi V, Guinnepain MT, Cacoub P, ''et al'' |title=Prevalence of monoclonal gammopathy in patients presenting with acquired angioedema type 2 |journal=Am. J. Med. |volume=113 |issue=3 |pages=194–9 |year=2002 |month=August |pmid=12208377 |doi= |url=http://linkinghub.elsevier.com/retrieve/pii/S0002934302011245 |issn=}}</ref>.
* Type II disease is associated with autoantibodies<ref name="pmid10644276"/> and monoclonal gammopathies<ref name="pmid12208377">{{cite journal |author=Frémeaux-Bacchi V, Guinnepain MT, Cacoub P, ''et al'' |title=Prevalence of monoclonal gammopathy in patients presenting with acquired angioedema type 2 |journal=Am. J. Med. |volume=113 |issue=3 |pages=194–9 |year=2002 |month=August |pmid=12208377 |doi= |url=http://linkinghub.elsevier.com/retrieve/pii/S0002934302011245 |issn=}}</ref>.


Acquired deficiency is characterized by low levels of [[complement C1q]].<ref name="pmid10644276">{{cite journal |author=Markovic SN, Inwards DJ, Frigas EA, Phyliky RP |title=Acquired C1 esterase inhibitor deficiency |journal=Ann. Intern. Med. |volume=132 |issue=2 |pages=144–50 |year=2000 |month=January |pmid=10644276 |doi= |url=http://www.annals.org/cgi/pmidlookup?view=long&pmid=10644276 |issn=}}</ref> Like hereditary angioedema, it has low [[complement C1 inhibitor protein]] function.<ref name="pmid10644276">{{cite journal |author=Markovic SN, Inwards DJ, Frigas EA, Phyliky RP |title=Acquired C1 esterase inhibitor deficiency |journal=Ann. Intern. Med. |volume=132 |issue=2 |pages=144–50 |year=2000 |month=January |pmid=10644276 |doi= |url=http://www.annals.org/cgi/pmidlookup?view=long&pmid=10644276 |issn=}}</ref> [[complement C1 inhibitor protein]] antigen is low in type I and normal in type II. It also has decreased complement C4 unless a paraprotein is present<ref name="pmid17513516">{{cite journal |author=McLean-Tooke A, Stroud C, Sampson A, Spickett G |title=Falsely normal C4 in a case of acquired C1 esterase inhibitor deficiency |journal=J. Clin. Pathol. |volume=60 |issue=5 |pages=565–6 |year=2007 |month=May |pmid=17513516 |doi=10.1136/jcp.2006.041350 |url=http://jcp.bmj.com/cgi/pmidlookup?view=long&pmid=17513516 |issn=}}</ref>.
Acquired deficiency is characterized by low levels of [[complement C1q]].<ref name="pmid10644276">{{cite journal |author=Markovic SN, Inwards DJ, Frigas EA, Phyliky RP |title=Acquired C1 esterase inhibitor deficiency |journal=Ann. Intern. Med. |volume=132 |issue=2 |pages=144–50 |year=2000 |month=January |pmid=10644276 |doi= |url=http://www.annals.org/cgi/pmidlookup?view=long&pmid=10644276 |issn=}}</ref> Like hereditary angioedema, it has low [[complement C1 inhibitor protein]] function.<ref name="pmid10644276">{{cite journal |author=Markovic SN, Inwards DJ, Frigas EA, Phyliky RP |title=Acquired C1 esterase inhibitor deficiency |journal=Ann. Intern. Med. |volume=132 |issue=2 |pages=144–50 |year=2000 |month=January |pmid=10644276 |doi= |url=http://www.annals.org/cgi/pmidlookup?view=long&pmid=10644276 |issn=}}</ref> [[Complement C1 inhibitor protein]] antigen is low in type I and normal in type II. It also has decreased [[complement C4]]<ref name="pmid11865013">{{cite journal |author=Gompels MM, Lock RJ, Morgan JE, Osborne J, Brown A, Virgo PF |title=A multicentre evaluation of the diagnostic efficiency of serological investigations for C1 inhibitor deficiency |journal=J. Clin. Pathol. |volume=55 |issue=2 |pages=145–7 |year=2002 |month=February |pmid=11865013 |pmc=1769585 |doi= |url=http://jcp.bmj.com/cgi/pmidlookup?view=long&pmid=11865013 |issn=}}</ref> unless a paraprotein is present<ref name="pmid17513516">{{cite journal |author=McLean-Tooke A, Stroud C, Sampson A, Spickett G |title=Falsely normal C4 in a case of acquired C1 esterase inhibitor deficiency |journal=J. Clin. Pathol. |volume=60 |issue=5 |pages=565–6 |year=2007 |month=May |pmid=17513516 |doi=10.1136/jcp.2006.041350 |url=http://jcp.bmj.com/cgi/pmidlookup?view=long&pmid=17513516 |issn=}}</ref>.


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]].<ref name="urlDailyMed: tranexamic acid">{{cite web |url=http://dailymed.nlm.nih.gov/dailymed/drugInfo.cfm?id=7849 |title=cyklokapron (tranexamic acid)  injection, solution  |author=Anonymous |authorlink= |coauthors= |date= |format= |work= |publisher=U.S. National Library of Medicine |pages= |language= |archiveurl= |archivedate= |quote= |accessdate=2009-02-19}}</ref> Concomittent use of [[warfarin]] may be needed to prevent [[embolism and thrombosis]].
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]].<ref name="urlDailyMed: tranexamic acid">{{cite web |url=http://dailymed.nlm.nih.gov/dailymed/drugInfo.cfm?id=7849 |title=cyklokapron (tranexamic acid)  injection, solution  |author=Anonymous |authorlink= |coauthors= |date= |format= |work= |publisher=U.S. National Library of Medicine |pages= |language= |archiveurl= |archivedate= |quote= |accessdate=2009-02-19}}</ref> Concomittent use of [[warfarin]] may be needed to prevent [[embolism and thrombosis]].

Revision as of 09:03, 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 which may be hereditary or acquired.

Classification of C1 esterase inhibitor deficiency

Angioedema due to deficiency of complement C1 inhibitor protein may manifest reduced d-dimer levels, especially during attacks.[2]

Hereditary angioedema (Hereditary C1 esterase inhibitor deficiency)

{main|Hereditary angioedema}}

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.[4][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

  1. ↑ Anonymous (2024), Angioedema (English). Medical Subject Headings. U.S. National Library of Medicine.
  2. ↑ 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.
  3. ↑ 3.0 3.1 3.2 3.3 3.4 3.5 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]
  4. ↑ 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.
  5. ↑ 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]
  6. ↑ 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]
  7. ↑ 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.
  8. ↑ Anonymous. cyklokapron (tranexamic acid) injection, solution. U.S. National Library of Medicine. Retrieved on 2009-02-19.
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