Download Disease Hereditary Angioedema HAE with normal C1

Disease
Hereditary Angioedema
HAE with normal
C1-INH Function
Acquired Angioedema
Angiotensin Converting
Enzyme Inhibitor-Induced
Angioedema
Idiopathic Angioedema
Plasma-derived
Nanofiltered C1-INH
concentrate (C1INH)
(Berinert/Cinryze)
Successful treatment of acute episodes of
angioedema with partly purified C1 inhibitor –used
successfully in 5 patients
Gadek et al (31)
Hereditary angioedema
caused by missense
mutations in the factor XII
gene: clinical features,
trigger factors, and therapy.
C1-INH infusions effective
in 6 women. Progestins
and tranexamic acid were
also used. Bork et al. (63)
Acquired angioedema. Review of
literature recommending efficacy
of C1-INH, Icatibant or Ecallantide
in AAE. Cicardi et al. (98)
Use of C1-Inhibitor for ACE-I AE Decreases
Mechanical Ventilation Time. Case report of
efficacy and resolution of airway edema.
Urnoski, et al. (85)
Prophylactic treatment with
plasma-derived C1-inhibitor in
idiopathic non-histaminergic
angioedema. Report of three
cases of idiopathic nonhistaminergic receiving C1-INH
infusions in literature and one
additional case of a 11 year old
child receiving C1-INH infusion.
Gravante et al (21)
A randomized, controlled trial to study the efficacy
and safety of C1 inhibitor concentrate in treating
hereditary angioedema. 22 patients treated with
C1-INH improved twice as fast compared to 13 in
placebo group. Kunschak et al. (32).
Angioedema Related to AngiotensinConverting Enzyme inhibitor. Both Icatibant
and C1-INH infusions decreased time to
resolution. Javaud, et. Al. (86)
Effect of C1-Esterase-inhibitor in angiotensinconverting enzyme inhibitor-induced
angioedema. Less intubation and more rapid
recovery in the C1-INH treated group (10
patients). Greve, et Al. (87)
Treatment of hereditary angioedema with a vaporheated C1 inhibitor concentrate. 11 patients
treated with more rapid resolution of angioedema.
Waytes et al. (33)
Efficacy of human C1 esterase inhibitor
concentrate compared with placebo in acute
hereditary angioedema attacks. More rapid relief of
symptoms with C1-INH than with placebo in 125
patients. No seroconversion to HIV or hepatitis
occurred with infusions.
Craig et al. (34)
ACE-I induced angioedema treated with
complement C1-inhibitor concentrate. Case
report of efficacy.
Rasmussen et al. (88)
Nanofiltered C1 Inhibitor Concentrate for
Treatment of Hereditary Angioedema. 68 patients
treated with C1-INH had resolution in 2 hours and
placebo >4 hours. Zuraw et al. (35)
Safety and efficacy of prophylactic nanofiltered C1inhibitor in hereditary angioedema. 146 subjects
studied and 93% reduction in attacks with C1-INH.
Zuraw et al. (36)
Recombinant
Human C1-INH
(rC1INH)
(Conestat alpha or
Ruconest)
11 adult patients (with HAE or acquired AE)
studied with rapid resolution but with quicker
relapse of symptoms in some patients.
Manson et al (9)
Recombinant replacement therapy for hereditary
angioedema due to C1 inhibitor deficiency. Review
of 10 clinical studies demonstrating drug efficacy.
Moldovan et al. (39)
Recombinant replacement
therapy for hereditary
angioedema due to C1
inhibitor deficiency. Review
of 10 clinical studies
demonstrating drug efficacy.
Moldovan et al. (39)
11 adult patients (with HAE or
acquired AE) studied with rapid
resolution but with quicker relapse
of symptoms in some patients.
Manson et al (9)
NONE
NONE
Treatment of an acute
attack of type III hereditary
angioedema with
ecallantide. Case report
demonstrating efficacy.
Cronin et al. (68)
Ecallantide for treatment of acute
attacks of acquired C1 esterase
inhibitor deficiency. 12 episodes in
3 patients effectively treated. Patel
et al. (10)
Effectiveness of ecallantide in treating ACE-I
induced angioedema in the emergency
department. Triple-blind (patient, physician,
and statistician), randomized, placebocontrolled, phase 2 study. 10% more patients
on Ecallantide had early discharge from
emergency room compared to placebo.
Bernstein et al. (89)
Idiopathic nonhistaminergic
angioedema successfully treated
with ecallantide, icatibant, and C1
esterase inhibitor replacement.
Case report. Stahl et al. (22)
Summary of 2 randomized trials comparing
recombinant and purified C1-INH versus placebo
showed active drug greatly decreased time to
resolution of angioedema. Kawalec et al (41)
Kallikrein inhibitor
Ecallantide
(Kalbitor)
Management of acute attacks of hereditary
angioedema: Review of two trials revealed safety
and efficacy of ecallantide. Duffey et al. (91)A
clinical trial of ecallantide, a novel kallikrein
inhibitor. 49 participated in a dose escalation study.
All doses tolerated, and symptoms greatly
improved with ecallantide over placebo.
Schneider et al. (25)
EDEMA4 double blinded plaebo controlled study of
96 HAE patients. Symptom severity score
improved with ecallantide. Levy et al. (43)
Ecallantide for the Treatment of Acute Attacks in
Hereditary Angioedema. (EDEMA3 trial). 71
patients treated and treatment group experienced
greatly improved symptoms and outcomes
compared to placebo. Cicardi, et. al. (44)
Ecallantide for the Acute Treatment of
Angiotensin-Converting Enzyme InhibitorInduced Angioedema: A Multicenter,
Randomized, Controlled Trial. Multicenter,
phase 2, double-blind study with subjects
randomized to receive a single subcutaneous
dose of ecallantide (10, 30, or 60 mg) or
placebo plus physician-directed conventional
therapy. Not much improvement with
Ecallantide over placebo. Lewis et al. (90)
Successful treatment of idiopathic
angioedema with ecallantide. Case
report. Berry et al. (23)
Resolution of an acute attack of
idiopathic angioedema with
ecallantide. Case report. Dy et al.
(24)
Icatibant
Bradykinin B2
receptor antagonist
Icatibant (Firazyr)
Icatibant, a New Bradykinin-Receptor Antagonist,
in Hereditary Angioedema. FAST-1 (56 patientsversus placebo) and FAST-2 (74 patients-versus
tranexamic acid). Relief of angioedema symptoms
faster with Icatibant. Cicardi et al. (25)
Prophylactic use of icatibant
before tracheal intubation of
a patient with hereditary
angioedema type III.
Iturri et al. (62)
Icatibant for Multiple Hereditary Angioedema
Attacks across the Controlled and Open-Label
Extension Phases of FAST-3. Well-tolerated and
effective in 88 episodes of laryngeal or cutaneous
angioedema. Lumry et al. (26)
Bradykinin receptor 2
antagonist for hereditary
angioedema type III attacks.
Case report showing
efficacy.
Bouillet et al. (63)
Randomized placebo-controlled trial of the
bradykinin B(2) receptor antagonist icatibant for the
treatment of acute attacks of hereditary
angioedema: the FAST-3 trial. Rapid and near
complete resolution seen with Icatibant in 43
patients. Lumry et al. (27)
Icatibant for HAE type III.
Canadian Agency for Drugs
and Technologies in Health.
Summary document of
published efficacy studies.
(64)
Rapid resolution of erythema
marginatum after icatibant in
acquired angioedema.
Bygum et al. (11)
Treatment of acquired
angioedema with icatibant: Case
report of efficacy
Zanichelli et al. (12)
Successful treatment of an acute
attack of acquired angioedema
with the bradykinin-B2-receptor
antagonist icatibant. Case report
of efficacy. Weller et al. (13)
A randomized trial of Icatibant…Multicenter,
double-blind, double-dummy, randomized
phase 2 study.
The time to complete resolution of edema
was significantly shorter with icatibant than
with combination therapy with a
glucocorticoid and an antihistamine
Bas, et. al. (91)
Therapeutic efficacy of Icatibant in
Angioedema Induced by ACEI: A Case
Series. 8 patients with ACEI-induced
angioedema treated with single dose of
Icatibant. First symptom improvement after
Icatibant injection occurred at a mean time of
50.6 minutes and complete relief of
symptoms at 4.4 hours. Bas, et. al. (92)
Treatment of ACE-I related angioedema with
icatibant: a case series. 13 patients treated.
Symptom relief was reported at 30 min. A
complete resolution of symptoms was
observed at 5 hours.
Bova, et. Al. (93)
Idiopathic nonhistaminergic
angioedema successfully treated
with ecallantide, icatibant, and C1
esterase inhibitor replacement.
Case report.
Stahl et al. (22)
Successful treatment of a facial
attack of angioedema with icatibant
in a patient with idiopathic
angioedema. Case report.
Montinaro et al. (26)
Nonhistaminergic idiopathic
angioedema: clinical response to
icatibant. Case report. Colas et al.
(27)
Treatment of idiopathic
nonhistaminergic angioedema with
bradykinin B2 receptor antagonist
icatibant. Case report.
Del et (28)
Supplemental Table 1: Evidence supporting the use of specific therapies in non-histaminergic (bradykinin-mediated) forms of angioedema.