Angioedema
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}}
Acquired deficiency is characterized by normal levels of complement C1q.
Acquired angioedema (Acquired C1 esterase inhibitor deficiency)
Acquired C1 esterase inhibitor deficiency is rare.[3][4]
- 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 or normal levels of complement C1q.
Treatment includes tranexamic acid which 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.[6]
References
- ↑ Anonymous (2024), Angioedema (English). Medical Subject Headings. U.S. National Library of Medicine.
- ↑ 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.
- ↑ 3.0 3.1 3.2 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]
- ↑ 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.
- ↑ 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]
- ↑ Anonymous. cyklokapron (tranexamic acid) injection, solution. U.S. National Library of Medicine. Retrieved on 2009-02-19.