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2017 ; 4
(3
): 312-319
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Idiopathic Anaphylaxis
#MMPMID28890861
Nwaru BI
; Dhami S
; Sheikh A
Curr Treat Options Allergy
2017[]; 4
(3
): 312-319
PMID28890861
show ga
Idiopathic anaphylaxis is a rare life-threatening disorder with symptoms similar
to other forms of anaphylaxis. There is lack of a robust evidence base
underpinning the treatment of anaphylaxis and even less so for idiopathic
anaphylaxis. Much of the evidence therefore comes from relatively small case
series and expert opinion. Idiopathic anaphylaxis is a diagnosis of exclusion,
requiring a thorough history and careful diagnostic work-up investigating
possible triggers and underlying predisposing factors. Key diagnostic tests
include skin-prick testing, tests for specific-IgE, component-resolved
diagnostics, and in some cases for allergen challenge tests. Other recognized
causes of anaphylaxis, such as foods, medications, insect stings, latex, and
exercise, should all be considered, as should differential diagnoses such as
asthma. While the cause of idiopathic anaphylaxis remains unknown, prompt
treatment with intramuscular epinephrine (adrenaline) administered into the
anterolateral aspect of the thigh is associated with good prognosis. There may
also be a role for H1-antihistamines and corticosteroids as second-line agents.
Patients need to be carefully monitored for signs of deterioration and/or a
possible protracted or biphasic reaction. Patients with frequent episodes of
anaphylaxis (e.g., six or more episodes/year) should be considered for preventive
therapy, which may include corticosteroids, H1- and H2-antihistamines, and, in
some cases, mast cell stabilizers such as ketotifen. Alternative
immune-suppressants (e.g., methotrexate) and anti-IgE may rarely also need to be
considered. In many cases, the frequency of anaphylaxis declines such that
regular use of corticosteroids can be discontinued after 9-12 months. Pediatric
patients should be treated with similar regimens as adults, but with appropriate
dose adjustments. Patients should carry their self-injectable epinephrine and
other emergency medications at all times in order to deal with emergency
situations.