Current treatment options for idiopathic angioedema.
BACKGROUND
Idiopathic angioedema is defined as localized swelling of the cutaneous and mucosal tissue that occurs in episodes without a clear etiology. It can be problematic to treat when the underlying pathophysiology is not well understood.


OBJECTIVE
To identify successful treatments of idiopathic angioedema reported in the literature.


METHODS
A literature search was performed using PubMed. Published case reports and articles discussing treatment of idiopathic angioedema were used in the formulation of this review. In addition, 2 case reports are provided.


RESULTS
Although there are no approved treatments for idiopathic angioedema, several medications used for the treatment of hereditary angioedema, such as bradykinin receptor antagonists (icatibant), kallikrein inhibitors (ecallantide), and C1 inhibitors, were successful in 10 patients. Anti-IgE monoclonal antibody (omalizumab) proved successful in 5 patients. The most widely used and successful medication was tranexamic acid (154 patients).


CONCLUSIONS
Despite an unknown etiology, this article highlights viable treatment options for idiopathic angioedema. More clinical trials and better markers identifying the cause of angioedema are needed.
Children's Mercy Hospital, Kansas City, Missouri. Electronic address: jashroba@cmh.edu.
Jodi
J
Shroba
Children's Mercy Hospital, Kansas City, Missouri.
Jill
J
Hanson
Children's Mercy Hospital, Kansas City, Missouri.
Jay
J
Portnoy
D000293:Adolescent; D000799:Angioedema / Q000188:drug therapy*; D000888:Antibodies, Anti-Idiotypic / Q000627:therapeutic use*; D001920:Bradykinin / Q000031:analogs & derivatives* / Q000627:therapeutic use; D005260:Female; D006801:Humans; D010455:Peptides / Q000627:therapeutic use*; D014148:Tranexamic Acid / Q000627:therapeutic use*
D000888:Antibodies, Anti-Idiotypic; D010455:Peptides; C026408:anti-IgE antibodies; C511194:ecallantide; D014148:Tranexamic Acid; C065679:icatibant; D001920:Bradykinin