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CC July 2017 Angioedema

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echoecho's version from 2018-04-25 14:40

Section

Question Answer
Define angioedema?Refers to localized swelling caused by vasodilation and increase vascular permeability
What is the most feared complication of angioedema?Life threatening air compromise with involvement of the pharynx or larynx
List the other areas that angioedema can also be present in?Edema of 1) face 2) lips 3) limbs 4) genitals 5) GI tract
The endothelial changes that give rise to angioedema are similar to those causing ________ but occur at a deeper level of the dermis and subcutaneous tissue?Urticaria
True or false? Angioedema may occur in conjunction w/ urticaria or may present in the absence of these more superficial skin changes?True
Multiple biochemical pathways have been implicated in the pathogenesis of angiogenesis, list 2 forms that have been identified?1) hereditary angioedema 2) acquired angioedema
Angioedema may be ____-mediated as in food and drug sensitivity. It may be ______-mediated in C1-inhibitor deficiency. It may be ________ mediated as in vasculitis. It may be non-immunologic or ______?1) IgE 2) bradykinin 3)complement 4) idiopathic
A careful hx and physical exam, guided by knowledge of various causes of angioedema can help determine what?The cause and select appropriate therapy for acute management and prevention of reoccurrences
In patients who have concomitant urticaria, what treatment is reasonable?Empiric treatment with antihistamines
Define idiopathic histamine-responsive acquired angioedema?Angioedema responding to antihistamines even in the absence of associated urticaria or allergic symptoms
Other forms of angioedema appear to result from dysregulation of the ______-______ system, with excessive bradykiknin activity as the final common pathway?Kallikrien-kinin system
In the last decade, specific treatments have been developed to target what system?Kallikrien-kinin system
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HEREDITARY ANGIOEDEMA:
Question Answer
Presents usually at what age?20
The most common form of hereditary angioedema is an autosomal dominant disease resulting in decreased levels of what protein?Inhibitor of complement protein 1 (C1-INH)
The above results in what?Spontaneous activation of C1 and increased vascular permeability
In another form of hereditary angioedema, there are normal C1-INH levels but decreased _____ functional activity?C1-INH
List the management for hereditary angioedema?1) treatment of acute attacks 2) short-term prophylaxis prior to procedures that may trigger attacks(dental procedures or surgery) 3) long term prophylaxis for patients with frequent attacks
List the 3 distinct classes of medication that the FDA approved for the management of acute attacks?1) plasma-derived or recombinant C1-INH resulting in down-regulation of bradykinin production 2) Ecallantide (Kalbitor) reduces bradykinin production via direct inhibition of kallikrein 3) Icatibant (Firazyr) is a bradykinin-receptor antagonist
Although several guidelines discuss prophylactic therapies in those with hereditary angiedema, there is significant variation in recommendation due to _____ evidence? List the commonly recommended prophylaxis or treatment?1) limited 2) C1-INH replacement therapy and Danazol (an attenuated androgen that increases endogenous C1-INH production)
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ANGIODEMA DUE TO MEDICATIONS:
Question Answer
The most common cause of acquired angioedema is what medication?ACEI (angiotensin converting enzyme inhibitor)
Angiotensin converting enzyme is the primary enzyme involved in catabolism of ______?bradykinin
A retrospective cohort study of early 2 million uses of ACE inhibitors estimated an incidence rate of _____ cases per 1000 person-years?4.38
A separate meta-analysis of randomized trials of ACEI for various cardiovascular indications estimated a risk ratio of _____ for angioedema compared w/ control interventions in patients > 65 years?2.79
While ACEI-associated angioedema usually occurs early in the treatment course, there have been reports of cases occurring after _____ of ACE-inhibitor therapy?years
What is treatment for this?1) supportive 2) one small randomized trial comparing Icatibant with a combination of a steroid and antihistamine found that time to resolution of symptoms was significantly reduced with Icatibant but this is not an FDA approved indication
What other drug (less commonly) may cause angioedema?NSAIDs
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ANGIOEDEMIA DUE TO ACQUIRED C1-INH DEFICIENCY:
Question Answer
Presents usually at what age?after 40
This is often due to any underlying ___________ disorder?myeloproliferative
The C1-INH deficiency may be the result of either _________ directed against the protein or increased _______ in neoplastic lymphatic tissue?autoantibodies; metabolism
What is the treatment?1) focused on treatment of underlying neoplastic or autoimmune process 2) some case reports supporting off-label use of Ecallantide and Icatibant
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SUMMARY:
Question Answer
*** SUMMARY = Angioedema is caused by increased _______ permeability and involves the ____ portion of the dermis and subcutaneous tissue?1) vascular 2) deep
*** SUMMARY = Involvement of the ______ airway may be life-threatening, andinvolvement of the ________ may result in unnecessary surgical intervention?1) upper 2) bowel
*** SUMMARY = Angioedema may be ____-mediated as in food or drug sensitivity. It may be _______-educated as in C1-inhibitor deficiency. It may be _______-mediated as in vasculitis. It may be nonimmunologic or ________?IgE; bradykinin; complement, idiopathic
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