This protocol addresses hereditary C1 inhibitor deficiency in patients experiencing recurrent angioedema attacks whose frequency and severity are substantially impacting quality of life.
The decision to initiate long-term prophylactic treatment is not based on rigid criteria — it reflects the individual patient's needs, taking into account attack burden, attack severity, comorbid conditions, treatment preferences, and access to emergent care.
Second-line long-term prophylaxis is indicated in this setting, with oral therapeutic options from established pharmacological classes available for consideration.
DOI: 10.1016/j.jaip.2020.08.046
The decision on when to use long-term prophylactic treatment cannot be made on rigid criteria but should reflect the needs of the individual patient.
Decisions regarding which patients should be considered for LTP should take into account the patient's QoL and treatment preferences in the context of attack frequency, attack severity, comorbid conditions, and access to emergent treatment.
Second-line therapies include the anabolic androgens (ie, Danazol) and antifibrinolytics (tranexamic acid or epsilon aminocaproic acid).
Second-line prophylactic medications should be reserved for when first-line medications are not available or when the patient will only accept oral therapy, with acknowledgment of potential side effects.
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