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 |