10. Urticaria & Angioedema Flashcards
see paper cards for first half of questions (slides 1-55)
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Which of the following has the pattern of low C4, normal C1INH antigen level, low C1INH function, and normal C1q?
A. Type I HAE B. Type II HAE C. HAE with normal C1INH D. Acquired C1INH deficiency E. ACE-I associated AE F. non-histaminergic idiopathic AE
B. Type II HAE- mutations in SERPING1 gene causing functional C1 inhibitor deficiency
C1 INH belongs to family of serine protease
inhibitors (serpins) and inhibits all but which of the following?
A. C1s and C1r (classical pathway)
B. Mannin binding lectin associated serine proteases
C. Platelet activating factor
D. FXIa (intrinsic pathway of coagulation)
E. FXIIa /f and kallikrein (contact system)
F. Weakly inhibits fibrinolytic pathway (Thrombin, plasmin, tissue type plasminogen activator)
C. Platelet activating factor
C1 INH belongs to family of serine protease
inhibitors (serpins) and inhibits what pathways?
A. C1s and C1r (classical pathway)
B. Mannin binding lectin associated serine proteases
C. FXIa (intrinsic pathway of coagulation)
D. FXIIa /f and kallikrein (contact system)
E. Weakly inhibits fibrinolytic pathway (Thrombin, plasmin, tissue type plasminogen activator)
What is the key mediator involved in Hereditary Angioedema Pathophysiology and how does it work?
Bradykinin.
Bradykinin increases vascular
permeability through B2 bradykinin
receptor on endothelial cells
Which of the following is NOT one of the most common symptoms of an acute HAE attack?
A. extremity edema
B. vomiting
C. abdominal pain
D. laryngeal edema
B. vomiting
A. extremity edema 96%
C. abdominal pain 93%
D. laryngeal edema 50%
A rash described as round, with a pale-pink center, surrounded by a slightly raised red outline spreading on the trunk and limbs can be a prodromal sign associated with what process?
Erythema marginatum
> 50% HAE patients have had
prodromal symptoms including EM, myalgias, fatigue
A patient’s genetics reveals defective expression of 1 allele of C1 INH gene. What disease type would that be associated with?
A. Type I HAE B. Type II HAE C. HAE with normal C1INH D. Acquired C1INH deficiency E. ACE-I associated AE F. non-histaminergic idiopathic AE
A. Type I HAE (~ 85% patients with C1 INH deficiency)
Type II HAE ~ 15 % patients with C1 INH deficiency due to Dysfunctional mutant protein
ACE-I associated AE 2/2 impaired bradykinin degradation
Which of the following is NOT true about Acquired angioedema?
A. later onset than HAE
B. Facial angioedema is a more common presentation than laryngeal edema
C. almost all have C1q low
D. most commonly associated with MGUS and splenic marginal zone lymphoma
E. androgens are often effective for prophylaxis
E. Tranexamic acid often effective for prophylaxis (thromboembolic events a concern) but androgens with similar efficacy
A. 4th decade of life- Median onset age 62
B. Facial angioedema > abdominal pain > extremity > laryngeal
C. C1q low in 91%
Type I: paraneoplastic: consumption of C1-INH by neoplastic lymphatic tissue, low C1-INH level due to C1 activation
Type II: autoimmune: autoantibody to C1-INH, impairs enzyme function, normal C1-INH level
Hereditary Angioedema with
normal C1INH is associated with mutations in all but which of the following in an autosomal dominant pattern?
A. SERPING1
B. FXII
C. angiopoietin
D. plasminogen
A. SERPING1 (Type I and II HAE)
- Normal C1 INH levels and function
- Predominantly female
- Facial > extremity swelling
A 25 year old female presents with tongue and facial swelling. This happens every year or so, and does not happen to anyone else in her family. She has no symptoms preceding the onset of swelling, and no other swelling or pain in other areas. What is the most likely pattern of labs associated with this diagnosis?
A. low C4, normal C1INH antigen level, low C1INH function, and normal C1q
B. low C4, low C1INH antigen level, low C1INH function, and normal C1q
C. low C4, low/normal C1INH antigen level, low C1INH function, and low/normal C1q
D. normal C4, normal C1INH antigen level, normal C1INH function, and normal C1q
D. Hereditary Angioedema with
normal C1INH
A. Type II HAE
B. Type I HAE
C. Acquired AE
Which of the following is not a preferred therapy for acute treatment for C1 INH Deficiencies? A. FFP B. C1 INH concentrate C. kallikrein inhibitor D. bradykinin B2 receptor antagonist E. recombinant C1 INH
A. FFP (May worsen acute attack in small percentage)
What is the preferred short term
prophylaxis for C1INH Deficiencies with anticipated procedures?
- C1 INH concentrate 20
u/kg <1 hr before
procedure - Androgens 5 10 days
prior and 2 days after - FFP (3 rd line)
List the long term prophylaxis options for patients with for C1INH Deficiencies?
- C1 INH concentrate
- Landelumab (anti kallikrein mab
- Attenuated Androgens (danazol , stanozolol, oxandrolone)
- Antifibrinolytics (aminocaproic acid (Amicar tranexamic acid ( Lysteda)
What routine evaluation should be performed in patients receiving attenuated androgens for HAE prophylaxis?
Annual ultrasounds and liver function tests every 6-12 mos (usually dose related AE of hepatotoxicity (rarely liver carcinoma))
Which of the following is NOT a contraindication to receiving attenuated androgens for HAE prophylaxis?
A. Pregnancy B. headaches C. Lactation D. childhood E. prostate CA
B. headaches
Which HAE specific therapy is associated with a pseudoallergic reaction due to MRGPRX2 stimulation?
Bradykinin-B2 receptor
antagonist - Icatibant- Firazyr
Injection site reactions (97%)
Which HAE specific therapy is associated with a black box warning for anaphylaxis?
Kallikrein inhibitor
Ecallantide (Kalbitor)
Which HAE specific therapy is contraindicated in a patient with a known or
suspected rabbit allergy?
Recombinant C1-INH
(Ruconest ®)
- Made in transgenic rabbits
see chart for mechanisms of acute and long term treatments
(see additional slide in notes p 34)
see page 43 for kinin activation pathway
Which acute treatment for HAE is effective in 100% of patients?
Bradykinin-B2 receptor
antagonist - Icatibant- Firazyr
- C1INH 20u/kg effective in 91%
- Tranexamic acid least effective
What are the 3 subtypes of urticarial vasculitis?
Normocomplementemic (NUV)
Hypocomplementemic UV (HUV)
Hypocomplementemic UV syndrome (HUVS)
Which type of urticarial vasculitis is most commonly associated with SLE?
SLE: 54% HUV (3% NUV)
see additional slide in notes p 34
Which type of urticarial vasculitis is more severe and most commonly associated with COPD in 50%?
HUVS (McDuffie syndrome)
Patients with UV and low complement not fulfilling criteria considered to have HUV
(see additional slide in notes p 34)
