Is there new literature surrounding icatibant (Firazyr) regarding safety and efficacy for severe acute cases of ACEI-induced angioedema?

Comment by InpharmD Researcher

A comprehensive literature search identified limited recently published literature supporting the safety and efficacy of icatibant (Firazyr®) for severe acute cases of ACEI-induced angioedema. One retrospective study published in 2021 included 7 patients with acute ACEI-induced angioedema and observed favorable results following administration of icatibant. Another case report published in 2022 describes successful relief of severe ACEI-induced angioedema following icatibant administration. In general, the inclusion of patients with mild ACEI-induced angioedema in available studies makes results difficult to generalize to patients in the emergency department with more acute severe cases. Available data from the phase 3 trial for icatibant involving most patients with moderate cases and a meta-analysis have observed no significant differences between icatibant and placebo for angioedema-related outcomes.

Background

A 2019 meta-analysis of three identified randomized controlled trials (RCTs; N= 179) was conducted to evaluate the effectiveness and tolerability of icatibant in the treatment of angiotensin-converting enzyme inhibitor (ACEI)-induced angioedema (AE). Included RCTs were determined to have low risk of bias, with similar patient baseline characteristics among all trials. While one trial administered icatibant 30 mg subcutaneously twice, with six hours between the two doses, as compared to only once for the other two RCTs, data from the three RCTs were combined to determine the elapsed time to complete resolution of edema. For the primary efficacy outcome of the time taken to achieve complete resolution of symptoms, defined as absence of symptoms of edema, patients treated with icatibant reached complete symptom resolution earlier than those in control group (mean difference -7.77 hours, 95% confidence interval [CI] -25.18 to 9.63 hours), although this difference was not found to be statistically significant (p= 0.38). For the secondary outcomes, the number of patients exhibiting complete resolution of symptoms after <4 hours was marginally increased in the icatibant group than the control group (relative risk [RR] 1.20; 95% CI 0.48 to 3.04; p= 0.70). Again, no significant difference was noted in the time to onset of symptom relief between the two groups. For the safety outcomes, there was no significant difference in adverse event incidence (RR 0.95, 95% CI 0.43 to 2.10) or drug‐related adverse event incidence (RR 1.29, 95% CI 0.58 to 2.87) between the two groups, respectively. In summary, the results of this meta-analysis indicate that the reduction in time to complete symptom resolution seen from icatibant administration in patients with ACEI-induced AE was not clinically significant. Additionally, further research is required to accurately evaluate the efficacy of icatibant therapy for ACEI‐induced AE before it can be recommended for use in clinical practice. Results of this meta-analysis are not necessarily specific to patients with acute severe ACEI-induced AE. [1]

A systematic review published in 2017 describes available evidence behind various therapies for ACEI-induced angioedema. It is noted that the inclusion of mild angioedema in the large randomized controlled trial evaluating icatibant makes results difficult to generalize to patients in the emergency department with more acute severe cases. Use of icatibant in these patients may be more cost-effective as icatibant may help prevent progression to intubation or tracheostomy and help shorten the course. Overall, data supporting use of icatibant, specifically in acute severe cases, are limited. [2]

A 2021 retrospective study investigated the indications and efficacy of off-label use of icatibant for the management of acute non-hereditary AE. A review of pharmacy records revealed 8 males and 10 females receiving icatibant (median age of 62 years), with 14 cases prescribed for drug-induced AE. Angiotensin-converting enzyme inhibitors were most commonly implicated (n= 7), while other culprit drugs included angiotensin receptor blockers (ARB; n= 3), tissue plasminogen activator (tPA; n= 3) and dipeptidyl peptidase-4 (DPP4) inhibitors (n= 1, in combination with ARB). Most patients (78% of cases) received initial icatibant administration in the emergency department. Severity of AE score was categorized as Type 1, lip and anterior tongue involvement; Type 2, floor of mouth, palatal or oropharyngeal edema; Type 3, laryngeal or hypopharyngeal edema. Based on these measurements, one patient with ACEI-induced AE had Type 1, one had Type 1/2, three had Type 2, and two had Type 3. The reported median resolution time was 9 h in patients presenting with ACEI-associated AE, which was shorter than the 18 h for all causes. Overall, individual cases of both ACEI- and non-ACEI-associated AE appeared to have favorable responses to icatibant. [3]

A 2018 systematic review evaluated the efficacy of icatibant, ecallantide, and tranexamic acid (TA) for ACEI-induced and idiopathic AE (non-hereditary AE). This systematic review included 61 studies, with 38 describing treatment in the acute setting, although the majority of evidence was case reports. There were no direct comparisons between icatibant, ecallantide, or TA. For ACEI-induced AE, two randomized controlled studies of ecallantide do not suggest a significant benefit based on response rate versus placebo (10%-16% response rate). Icatibant may have similar efficacy to C1NH and fresh frozen plasma with an average response time of less than 2 hours, but the majority of included studies were not controlled and of lower quality. Data for TA in ACEI-induced AE was not available. [4]

As opposed to tranexamic acid, there are more studies for icatibant with polarizing results. One randomized comparison study between TA and conventional therapy consisting of steroids and antihistamines for ACEI-induced AE found significant benefits for icatibant therapy in reducing time to edema resolution and complete resolution. However, two placebo-controlled studies reported time to resolution of symptoms no better than placebo. Generalizability of these results, specifically to patients with severe ACEI-induced AE, may be limited. [5], [6]

References:

[1] Jeon J, Lee YJ, Lee SY. Effect of icatibant on angiotensin-converting enzyme inhibitor-induced angioedema: A meta-analysis of randomized controlled trials. J Clin Pharm Ther. 2019;44(5):685-692. doi:10.1111/jcpt.12997
[2] Riha HM, Summers BB, Rivera JV, Van Berkel MA. Novel Therapies for Angiotensin-Converting Enzyme Inhibitor-Induced Angioedema: A Systematic Review of Current Evidence. J Emerg Med. 2017;53(5):662-679. doi:10.1016/j.jemermed.2017.05.037
[3] Le TA, Smith W, Hissaria P. Real-world off-label use of icatibant for acute management of non-hereditary angioedema. Intern Med J. 2021;51(3):419-423. doi:10.1111/imj.15241
[4] van den Elzen M, Go MFCL, Knulst AC, Blankestijn MA, van Os-Medendorp H, Otten HG. Efficacy of Treatment of Non-hereditary Angioedema. Clin Rev Allergy Immunol. 2018;54(3):412-431. doi:10.1007/s12016-016-8585-0
[5] Sinert R, Levy P, Bernstein JA, et al. Randomized Trial of Icatibant for Angiotensin-Converting Enzyme Inhibitor-Induced Upper Airway Angioedema. J Allergy Clin Immunol Pract. 2017;5(5):1402-1409.e3. doi:10.1016/j.jaip.2017.03.003
[6] Straka BT, Ramirez CE, Byrd JB, et al. Effect of bradykinin receptor antagonism on ACE inhibitor-associated angioedema. J Allergy Clin Immunol. 2017;140(1):242-248.e2. doi:10.1016/j.jaci.2016.09.051

Literature Review

A search of the published medical literature revealed 3 studies investigating the researchable question:

Is there new literature surrounding icatibant (Firazyr) regarding safety and efficacy for severe acute cases of ACEI-induced angioedema?

Please see Tables 1-3 for your response.


 

Randomized Trial of Icatibant for Angiotensin-Converting Enzyme Inhibitor-Induced Upper Airway Angioedema

Design

Multicenter, phase 3, randomized, double-blind clinical trial

N= 121

Objective

To evaluate the efficacy of icatibant in subjects with angiotensin-converting enzyme inhibitors (ACE-I)-induced angioedema

Study Groups

Icatibant (n= 61)

Placebo (n= 60)

Inclusion Criteria

Age ≥ 18 years; currently being treated with an ACE-I; presented with ACE-I–induced angioedema of the head and/or neck; moderately severe ACE-I–induced angioedema < 12 hours' duration

Exclusion Criteria

Diagnosis of angioedema of other etiology (hereditary angioedema, acquired angioedema, or allergic angioedema); family history of recurrent angioedema; history of angioedema attacks before starting ACE-I treatment; anaphylaxis, trauma, abscess or infection or associated disease; local inflammation; local tumor; postoperative or postradiogenic edema; salivary gland disorders; non-ACE-I drug-induced angioedema; acute urticaria; vascular condition that was a contraindication to study participation in the investigator's judgment; requiring immediate airway intervention; evident clinical response to antihistamines, corticosteroids, or epinephrine

Methods

Patients were randomized (1:1) to receive icatibant 30 mg or placebo administered as a single subcutaneous injection. Severity of the ACE-I–induced angioedema attack was determined by the patient's worst severity rating at baseline among 4 clinical domains (difficulty breathing, difficulty swallowing, voice changes, and tongue swelling) assessed by the enrolling physician. Antihistamines, corticosteroids, and epinephrine were allowed at any time before or after study drug administration

Duration

Enrollment: December 2013 to August 2015

Follow-up: 8 hours after drug administration

Outcome Measures

Primary: time to meeting discharge criteria (time from study drug administration to earliest time that difficulty breathing and difficulty swallowing absent [rating of 0 out of 4], and voice change and tongue swelling mild or absent [0 or 1])

Secondary: time to onset of symptom relief (TOSR; time from study drug administration to earliest time at which symptoms of at least moderate severity [pretreatment rating ≥ 2] improved by a minimum of 1 severity grade, and mild or absent [pretreatment rating of 0 or 1] symptoms again assessed as mild or absent); occurrence of airway intervention; admission to hospital

Baseline Characteristics

 

Icatibant (n= 61)

Placebo (n= 60)

 

Age, years

60.9 ± 12.1 61.8 ± 13.4  

Male

34 (55.7%) 25 (41.7%)  

Black or African American

41 (67.2%) 43 (71.7%)  

Body mass index, kg/m2

33.5 ± 8.9 30.5 ± 7.4  

Attack severity

Moderate

Severe or very severe

 

45 (73.8%)

16 (26.2%)

 

42 (70%)

18 (30%)

 

ACE-I taken

Lisinopril

Ramipril

Lisinopril and hydrochlorothiazide

Enalapril

Perindopril

Other

 

40 (65.6%)

6 (9.8%)

5 (8.2%)

4 (6.6%)

3 (4.9%)

3 (4.9%)

 

44 (73.3%)

2 (3.3%)

3 (5%)

1 (1.7%)

2 (3.3%)

6 (10%)

 

ACE-I treatment started within 90 days of the attack

16 (26.7%)

15 (24.6%)

 

Conventional medications administered

Antihistamines

Corticosteroids

Epinephrine

 

55 (90.2%)

52 (85.2%)

49 (80.3%)

 

55 (91.7%)

53 (88.3%)

51 (85%)

 

Median time from attack onset to conventional medication administration, hours (IQR)

3.4 (1.8 to 5.8)

3.6 (2.5 to 5.1)

 

Median time from conventional medication to study drug administration, hours (IQR)

3.7 (2 to 5.4)

3.1 (1.6 to 4.4)

 

Median time from attack onset to study drug administration, hours (IQR)

7.9 (5.5 to 9.7)

7.8 (5.6 to 9.4)

 

IQR, interquartile range

Results

Endpoint

Icatibant (n= 61)

Placebo (n= 60)

p-value

Median time to meet discharge criteria, hours (IQR)

4 (2 to 6) 4 (1 to 6) 0.63

Median TOSR, hours (IQR)

2 (0.6 to 3.1) 1.6 (0.5 to 3.9) 0.57

Admitted to hospital, n

34 32 Not disclosed

Admitted to intensive care unit, n

14 16 Not disclosed

Endotracheal intubation, n

1 0 Not disclosed

Adverse Events

Common Adverse Events: any treatment-related event (18.3% vs. 13.8%), injection site reaction (65% vs. 31%), erythema (51.7% vs. 22.4%), swelling (28.3% vs. 22.4%), cutaneous pain (16.7% vs. 12.1%), burning sensation (25% vs. 12.1%), itching (21.7% vs. 10.3%), warm sensation (26.7% vs. 13.8%)

Serious Adverse Events: any serious event (3.3% vs. 1.7%)

Percentage that Discontinued due to Adverse Events: No fatal events occurred.

Study Author Conclusions

Icatibant was no more efficacious than placebo in at least moderately severe ACE-I-induced angioedema of the upper airway.

InpharmD Researcher Critique

There was noted to be a significantly higher proportion of patients weighing 75 kg or more in the icatibant group compared to the placebo group, and weight has been observed to influence angioedema outcomes in some reports. Overall, the results of this study may be somewhat generalizable to patients with severe ACE-I-induced angioedema due to the inclusion of such patients. 



References:

Sinert R, Levy P, Bernstein JA, et al. Randomized Trial of Icatibant for Angiotensin-Converting Enzyme Inhibitor-Induced Upper Airway Angioedema. J Allergy Clin Immunol Pract. 2017;5(5):1402-1409.e3. doi:10.1016/j.jaip.2017.03.003

A Randomized Trial of Icatibant in ACE-Inhibitor–Induced Angioedema

Design

Double-blind, double-dummy, phase 2, randomized controlled trial

N= 27

Objective

To report the results of a phase 2 study of icatibant, as compared with standard combination therapy consisting of a glucocorticoid and an antihistamine, in patients with angiotensin-converting enzyme (ACE-I)–induced angioedema of the upper aerodigestive tract

Study Groups

Icabitant (n= 13)

Standard therapy (n= 14)

Inclusion Criteria

Age 18 to 95 years, receiving ACE inhibitors and presented to the emergency department with ACE-I–induced angioedema affecting the upper aerodigestive tract

Exclusion Criteria

Angioedema due to other causes, history of angioedema before the initiation of ACE inhibitor therapy, acute urticaria, unstable angina, acute myocardial ischemia, acute heart failure with a New York Heart Association class of III or IV, pregnancy/lactation

Methods

Patients were randomized (1:1) within 10 hours of symptom onset to receive icatibant 30 mg subcutaneously in the abdominal wall or standard therapy consisting of prednisolone intravenous (IV) 500 mg plus clemastine 2 mg.

Patients received an IV and subcutaneous normal saline (0.9%) placebo to mask therapy. The intensity of six symptoms (pain, shortness of breath, dysphagia, change in voice, sensation of a foreign body, and feeling of pressure) was assessed before treatment at multiple intervals up to 48 hours using a visual analog scale (VAS) ranging from 1 to 10. Then a composite score was determined using the average of the six symptoms. Angioedema was also assessed at the lips/cheeks, tongue, oropharynx, and hypopharynx/larynx. 

Rescue therapy via icatibant 30 mg and prednisolone 500 mg were allowed if the symptoms did not reduce six hours after treatment initiation. 

Duration

48 hours

Follow-up: 14 days after hospital admission

Outcome Measures

Primary: time to complete resolution of edema after administration of study treatment

Secondary: complete resolution of edema at 4 hours of treatment, time to onset of symptom relief, patients who did not respond to treatment as defined by requiring rescue therapy

Baseline Characteristics

 

Icatibant (n= 13)

Standard therapy (n= 14)

 

Age, years

62.4 ± 9.7 69.4 ± 16.6  

Female

4 (31%) 6 (43%)  

ACE inhibitor

Benazepril

Captopril

Captopril-hydrochlorothiazide

Enalapril

Lisinopril

Ramipril

 

1 (8%)

1 (8%)

1 (8%)

5 (38%)

0

5 (38%)

 

0

1 (7%)

0

2 (14%)

3 (21%)

8 (57%)

 

Previous episode of ACE-I–induced angioedema

5 (38%) 5 (36%)  

Scores for baseline severity of symptoms

Composite investigator-assessed symptom score

Composite investigator-assessed angioedema score

Composite patient-assessed VAS score

 

1.1 ± 0.2

1.1 ± 0.2

2.9 ± 0.6

 

1.2 ± 0.2

1.1 ± 0.2

3.5 ± 0.6

 

Angioedema location

Lips

Cheeks

Tongue

Pharynx and soft palate

Larynx

Floor of mouth

Face

 

3 (23%)

3 (23%)

8 (62%)

4 (31%)

6 (46%)

6 (46%)

3 (23%)

 

4 (29%)

2 (14%)

10 (71%)

5 (36%)

7 (50%)

9 (64%)

3 (21%)

 

Results

Endpoint

Icatibant (n= 13)

Standard therapy (n= 14)

p-value

Median time to complete resolution of edema, hours (interquartile range [IQR])

8.0 (3.0 to 16.0)

27.1 (20.3 to 48.0)

0.002

Patients with complete resolution of edema at 4 hours after treatment

5 (38%)

0

0.02

Median time to onset of symptom relief, hours (95% confidence interval [CI])

According to composite investigator-assessed symptom score

According to composite patient-assessed VAS score

According to composite investigator-assessed angioedema score

 

2.0 (1.0 to 8.1)

2.0 (2.0 to 6.3)

2.0 (2.0 to 12.0)

 

11.7 (8.0 to 18.0)

7.9 (1.2 to 11.8)

12.0 (11.3 to not estimable)

 

0.03

0.36

0.003

Requiring rescue therapy

0

3

 

Any adverse events

Drug-related adverse events

Serious adverse events

Injection-site reaction

Redness

Swelling

Pain

Itching

Sensation of warmth

1 (7%)

1 (7%)

0

--

12 (80%)

8 (53%)

7 (47%)

4 (27%)

4 (27%)

4 (27%)

1 (7%)

1 (7%)

--

4 (27%)

3 (20%)

2 (13%)

1 (7%)

0

 

Study Author Conclusions

Among patients with ACE-inhibitor–induced angioedema, the time to complete resolution of edema was significantly shorter with icatibant than with combination therapy with a glucocorticoid and an antihistamine.

InpharmD Researcher Critique

The study was conducted in Germany which may not reflect the U.S. healthcare landscape. Approximately 36% of patients in both groups had a previous history of ACEi-angioedema, representing a portion of the population that may not be treatment-naive. Lastly, the small patient population makes it difficult to determine the magnitude of effects as seen by the wide range of confidence intervals and interquartile range.
References:

Baş M, Greve J, Stelter K, et al. A randomized trial of icatibant in ACE-inhibitor-induced angioedema. N Engl J Med. 2015;372(5):418-425. doi:10.1056/NEJMoa1312524

 

CAN’T INTUBATE, CAN’T OXYGENATE: A RARE CASE OF A DIFFICULT AIRWAY DUE TO NONHEREDITARY ANGIOEDEMA

Design

Case report

Case presentation

A 68-year-old man with a high body mass index (BMI) and a history of hypertension, type II diabetes, and chronic kidney disease presented to the emergency department with dyspnea and edema. He had a history of similar episodes in the past, which had been successfully treated with corticosteroids, antihistamines, and epinephrine. He had four different antihypertensive medications in his therapy, including an angiotensin-converting enzyme inhibitor (ACEI), ramipril 5 mg, for 8 years. During the physical examination, the patient was hypertensive, tachycardic, tachypneic, and edematous. Laboratory tests showed elevated liver enzymes, glucose, and creatinine. The patient was admitted with a presumptive diagnosis of angioedema and started on high-dose systemic corticosteroid therapy. However, his condition worsened, and a tracheotomy was performed to secure an airway. Subsequently, the patient was administered icatibant, which led to rapid regression of the edema and stabilization of his vital signs. The patient was discharged after five days with a recommendation to discontinue the ACEI and other drugs from the Renin-Angiotensin-Aldosterone System (RAAS). Hereditary angioedema types I and II were excluded with normal C1q inhibitor and complement levels.

Study Author Conclusions

In conclusion, emergency physicians should be adequately educated on when to suspect nonhereditary angioedema and how to manage the rapid onset of airway obstruction it causes in the most severe cases. Emergency departments should also be equipped with the medications used in its management.

 

References:

Delalić Đ, Borčić V, Prkačin I. CAN'T INTUBATE, CAN'T OXYGENATE: A RARE CASE OF A DIFFICULT AIRWAY DUE TO NONHEREDITARY ANGIOEDEMA. Acta Clin Croat. 2022;61(Suppl 1):99-103. doi:10.20471/acc.2022.61.s1.17