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Table 3 Outcomes of discontinuation

From: Attenuated androgen discontinuation in patients with hereditary angioedema: a commented case series

Case Changes to HAE attack frequency and/or severity Side effects during or after discontinuation Replacement treatment for AAs Management of HAE attacks and side effects Time since discontinuation, months Current patient outcome
1 No attacks None Lanadelumab 300 mg every 14 days NA 10 No attacks (Angioedema Activity Score)
High QoL (AE-QoL Questionnaire score)
Headaches reduced and weight loss
2 Increased frequency to up to four attacks/week None rhC1-INH 12,600 U once/week After 3 months, rhC1-INH was switched to IV pdC1-INH 2,000 U once/week with on-demand icatibant
Breakthrough attacks continued and prophylactic lanadelumab 300 mg twice every 14 days was introduced, with on-demand icatibant 30 mg and/or pdC1-INH 2,000 U
26 No attacks
QoL improved (clinician reported)
3 Increased frequency, with severe abdominal attacks High weight IV pdC1-INH 1,000 U twice/week HAE attacks became milder and less frequent over a 2-month period 72 Zero to one attacks/year
Good QoL (clinician reported)
No reported hypertension, myalgia or headaches, and weight decreased
4 One attack in 7 years None Icatibant 30 mg on-demand 84 One attack in this time
QoL has been affected by a stroke and other health conditions
5 Increased frequency, with abdominal attacks Depression and anxiety, likely due to both cancer and HAE attacks Icatibant 30 mg on-demand Antidepressants, and IV prophylactic pdC1-INH 1,000 U/3 days introduced after 6 months
Patient then switched to lanadelumab 300 mg every 14 days because of a deep vein thrombosis
84 No attacks
Satisfied with prophylaxis but concerned with cancer progression
6 No attacks None Lanadelumab 300 mg every 14 days None required 7 No attacks
QoL improved (clinician reported)
7 Increased frequency and severity, with severe laryngeal attack None Icatibant 30 mg on-demand Patient supply of on-demand therapy exhausted prior to laryngeal attack. The patient experienced respiratory failure. Cricothyrotomy and pdC1-INH 2,000 U were required, and danazol was reintroduced at 200 mg QD. Danazol was then tapered to 100 mg QD, and then 50 mg QD NA Non-optimal level of attacks
Poor QoL (clinician reported)
8 One to two/month during double-blind phase of trial
None on open-label
lanadelumab 300 mg every 14 days
None Placebo/lanadelumab during double-blind phase of trial
Open-label lanadelumab 300 mg every 14 days
On-demand C1-INH for breakthrough attacks during double-blind phase of trial 48 Almost no attacks
Good QoL (clinician reported)
Patient benefited from frequent contact with research nurses during trial and support with self-cannulation during the double-blind period of the trial, when acute treatment was required
9 Increased frequency and severity None 1,000–1,500 U IV pdC1-INH twice/week Dose of pdC1-INH titrated to 500 U QOD. Danazol reinstated once patient had ceased breastfeeding NA One to three attacks/year
AE-QoL Questionnaire total score = 36.76
10 Increased frequency with mostly abdominal attacks Fatigue Icatibant 30 mg on-demand On-demand C1-INH, prophylactic IV or SC C1-INH, and lanadelumab also available, but patient reinstated danazol after 19 weeks NA No attacks
Patient has strong reservations about using injectables and a strong psychological dependence on danazol
  1. AE-QoL, Angioedema Quality of Life; IV, intravenous; NA: not applicable; QD: every day; QOD: every other day; SC, subcutaneous