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'Nuts and Bolts' of Laboratory Evaluation of Angioedema

Authors :
László Cervenak
Nóra Veszeli
Erika Kajdácsi
Henriette Farkas
Lilian Varga
Source :
Clinical Reviews in Allergy & Immunology. 51:140-151
Publication Year :
2016
Publisher :
Springer Science and Business Media LLC, 2016.

Abstract

Angioedema, as a distinct disease entity, often becomes a clinical challenge for physicians, because it may cause a life-threatening condition, whereas prompt and accurate laboratory diagnostics may not be available. Although the bedside diagnosis needs to be established based on clinical symptoms and signs, family history, and the therapeutic response, later, laboratory tests are available. Currently, only for five out of the nine different types of angioedema can be diagnosed by laboratory testing, and these occur only in a minority of the patient population. Hereditary angioedema with C1-inhibitor (C1-INH) deficiency type I can be diagnosed by the low C1-INH function and concentration, whereas in type II, C1-INH function is low, but its concentration is normal or even elevated. C1q concentration is normal in both forms. Acquired angioedema with C1-INH deficiency type I is characterized by the low C1-INH function and concentration; however, C1q concentration is also low, and autoantibodies against C1-INH cannot be detected. Complement profile of acquired angioedema with C1-INH deficiency type II is similar to that of type I, but in this form, autoantibodies against C1-INH are present. Hereditary angioedema due to a mutation of the coagulation factor XII can be diagnosed exclusively by mutation analysis of FXII gene. Diagnostic metrics are not available for idiopathic histaminergic acquired angioedema, idiopathic non-histaminergic acquired angioedema, acquired angioedema related to angiotensin-converting enzyme inhibitor, and hereditary angioedema of unknown origin; these angioedemas can be diagnosed by medical and family history, clinical symptoms, and therapeutic response and by excluding the forms previously described. Several potential biomarkers of angioedema are used to date only in research. In the future, they could be utilized into the clinical practice to improve the differential diagnosis, therapy, as well as the prognosis of angioedema.

Details

ISSN :
15590267 and 10800549
Volume :
51
Database :
OpenAIRE
Journal :
Clinical Reviews in Allergy & Immunology
Accession number :
edsair.doi.dedup.....cc9724b064fdf1881b3e5de3d244967b
Full Text :
https://doi.org/10.1007/s12016-016-8539-6