HAE Diagnosis/Treatment
This screening laboratory abnormality is almost always present between attacks in classic HAE due to C1 inhibitor deficiency.
What is a low C4 level?
Recurrent angioedema without urticaria, abdominal pain, and a family history should prompt evaluation for this condition.
What is hereditary angioedema?
This anti-IgE monoclonal antibody is first-line biologic therapy for antihistamine-refractory CSU.
What is omalizumab?
The primary mediator of swelling in hereditary and acquired C1 inhibitor deficiency.
What is bradykinin?
CSU is defined by urticaria occurring most days of the week for at least this duration.
What is 6 weeks?
Two laboratory tests that differentiate Type I from Type II HAE.
What are C1 inhibitor antigen level and C1 inhibitor functional assay?
Urticaria lasting >24 hours with residual hyperpigmentation and possible systemic symptoms suggests this diagnosis.
What is urticarial vasculitis?
The standard, guideline-supported dose of omalizumab for CSU.
What is 300 mg every 4 weeks?
This system generates bradykinin through activation of factor XII and plasma kallikrein.
What is the contact system?
Up-dosing of this medication class (up to 4× standard dose) is first-line therapy for CSU.
What are second-generation H1 antihistamines?
This complement component helps distinguish acquired C1 inhibitor deficiency from hereditary disease.
What is C1q?
Neutrophil-predominant urticaria should prompt evaluation for this autoinflammatory syndrome associated with IgM gammopathy.
What is Schnitzler syndrome?
This BTK inhibitor showed high efficacy for CSU in recent network meta-analysis but has less certain long-term safety.
What is remibrutinib?
In CSU, mast cell activation commonly results in release of this mediator responsible for pruritus.
What is histamine?
Angioedema plus wheals in CSU does not exclude this mechanism.
What is mast cell–mediated disease?
These medications are ineffective for acute swelling in bradykinin-mediated angioedema.
What are antihistamines, corticosteroids, and epinephrine?
This medication class is a common cause of nonhistaminergic angioedema, particularly in African American patients.
What are ACE inhibitors?
This IL-4 receptor antagonist improves itch and wheals in CSU but lacks angioedema-specific trial data.
What is dupilumab?
IgG autoantibodies directed against this receptor support an autoimmune subtype of CSU.
What is FcεRIα?
Sensitivity to this medication class is associated with prolonged and more severe CSU.
What are NSAIDS?
This class of medications directly targets the bradykinin pathway and is effective for acute HAE attacks.
What are C1 inhibitor replacement or bradykinin-pathway agents (e.g., icatibant)?
Normal C1 inhibitor levels, estrogen sensitivity, and mutations in FXII characterize this subtype of angioedema.
What is HAE with normal C1 inhibitor (HAE-nl-C1INH)?
This immunosuppressant may be highly effective for CSU but is associated with increased adverse events.
What is cyclosporine?
This biologic pathway explains why antihistamines fail in bradykinin-mediated angioedema.
What is non–mast cell–mediated vascular permeability?
This type if prophylaxis is recommended before dental procedures or surgery in patients with HAE
What is short-term prophylaxis with C1 inhibitor?