Should all patients with acute undiagnosed angioedema have mast cell tryptase levels assessed?

Date First Published:

July 12, 2010

Last Updated:

July 13, 2010

Report by:

Katherine Palmer, Medical Student (Manchester Royal Infirmary)

Search checked by:

Katherine Palmer, Manchester Royal Infirmary

Three-Part Question:

In [patients with acute undiagnosed angioedema] are [mast cell tryptase levels] better than [clinical impression] at [determining diagnosis]?

Clinical Scenario:

A 21 year old ,ale with no family history of angioedema and no clear allergen exposure has an acute episode of urticaria, genital oedema and stridor which requires IV hydrocortisone and antihistamines in the Emergency Department. Would mast cell tryptase levels help to make a diagnosis?

Search Strategy:

MEDLINE 1950 to 2010 June Week 5 and Embase 1980 to 2010 Week 27 using the OVID interface on the world wide web on 12/7/2010
CINAHL using the EBSCOhost on the world wide web on 12/7/2010

Search Details:

MEDLINE and Embase - (Hereditary Angioedema Type III/ OR Angioedema/ OR "Hereditary Angioedema Types I and II"/ OR angioedema.mp.) AND (exp Tryptases/ OR tryptase.mp.)
LIMIT to English Language and Humans
CINAHL - (Angioedema) AND (Tryptase)

Outcome:

MEDLINE - 16 papers, 3 relevant
Embase - 66 papers, 3 relevant
CINAHL - 1 paper, 0 relevant
MEDLINE and Embase found the same 3 papers that were relevant

Relevant Paper(s):

Study Title	Patient Group	Study type (level of evidence)	Outcomes	Key results	Study Weaknesses
Mast cell activation in acquired chronic urticaria-angioedema Bruno, G., Andreozzi, P., Magrini, L., Graf, U., Santangeio, G., Zaino, S. 2001 Italy	8 patients with chronic acquired urticaria-angioedema vs 7 healthy controls	Case-control study	Serum tryptase levels	(mean ± SD.: 9.6 ± 4.3 μg/l) vs (3.0 ± 1.2μg/l)	Very small study size Chronic angioedema studied, not acute
Serial measurements of Serum Tryptase in Angioetensin Converting Enzyme Inhibitor-Assosciated Angioedema Regner, K.R., Riegert-Johnson, D.L., Volcheck, G.W. 2003 USA	1 patient with ACE-i induced angioedema	Case report	Mast cell tryptase levels	Acute 9.78ng/mL (norm <11.5ng/mL) vs 4 days later ~2-4ng/mL	No actual value given for the base line 4 days later Case report only
Hypotensive shock and angio-oedema from angiotensin II receptor blocker: a class effect in spite of tripled tryptase values Nielsen, E.W. 2005 Norway	1 patient with Angiotensin II blocker angioedema	Case report	Mast cell tryptase levels	20 mins (11.7μgL -1); 8hrs (15.1 μgL -1); 12hrs (13.1 μgL -1); 2mths (5.4 μgL -1); Normal (<13.5 μgL -1)	Case report

Author Commentary:

Despite a very few case of documented tryptase levels in angioedema there seems to be situations in which patients have elevated levels of mast cell tryptase when suffering an acute episode of angioedema. There are also cases in which the level is not elevated clinically but is significantly elevated compared to the patients baseline. Thus the tryptase level during an acute episode may be beneficial in discovering the diagnosis.

Bottom Line:

More trials need to be conducted to see in which types of angioedema tryptase levels are elevated. Until then taking the mast cell tryptase level during an acute attack of angioedema may be useful in determining a diagnosis at a later date compared to a patients base line tryptase level. The evidence suggests that taking tryptase levels during an acute attack at presentation, at approximately 8 hours and 12 hours after presentation and a baseline after resolution could help with a diagnosis.

References:

Bruno, G., Andreozzi, P., Magrini, L., Graf, U., Santangeio, G., Zaino, S.. Mast cell activation in acquired chronic urticaria-angioedema
Regner, K.R., Riegert-Johnson, D.L., Volcheck, G.W.. Serial measurements of Serum Tryptase in Angioetensin Converting Enzyme Inhibitor-Assosciated Angioedema
Nielsen, E.W.. Hypotensive shock and angio-oedema from angiotensin II receptor blocker: a class effect in spite of tripled tryptase values