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Background Patients affected by angioedema due to hereditary and acquired C1-inhibitor (C1-INH) deficiency (HAE and AAE, respectively) report trouble accessing dental care, due to the risk of a life-threatening oropharyngeal and laryngeal attack triggered by dental procedures. The aim of this study was to assess the identification of hurdles in receiving dental care, and the effectiveness of short-term prophylaxis (STP) in preventing angioedema attacks. In addition, the study evaluated the impact of dental care in angioedema disease. All patients affected by angioedema due to C1-INH deficiency who were treated in the dentistry outpatient department of ASST Fatebenefratelli Sacco hospital (Milan, Italy) between 2009 and 2017 were considered for the analysis. Data were collected from patients' records. Results Twenty-nine patients were analyzed (27 with HAE and 2 with AAE). Of these, 63.0% reported that they had previously experienced hurdles in accessing dental care. Among patients with pathological oral status, at the first visit, 59.26% patients had moderate-tosevere oral disease. Seventy-five dental procedures were performed in 20 patients. Sixty procedures were preceded by STP (58 with plasma-derived C1-INH and 2 with danazol) in patients with/without long-term prophylaxis (LTP). Post-procedural attacks occurred in two patients. One HAE patient undergoing a tooth extraction without STP/LTP experienced a laryngeal attack. The other post-procedural attack occurred in an AAE patient with anti-C1-INH antibodies with STP with pdC1-INH. The angioedema disease did not worsen in any patient after dental care, but improved in four of them.

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RATIONALE: Low functional C1 inhibitor (C1-INH) levels are associated with a high risk of an angioedema attack in patients with hereditary angioedema with C1-INH deficiency (C1-INH-HAE). We measured functional C1-INH levels to inform treatment decisions for long-term prophylaxis with plasma-derived C1-INH in patients with C1-INH-HAE, including dose escalation of intravenous C1-INH and switching to subcutaneous C1-INH. METHODS: Three female patients (aged 50, 45, and 15 years) are described in this case series. Functional C1-INH was measured using the commercially available ELISA method (Quidel, San Diego, CA). Measurements were taken at the nadir immediately before the next scheduled dose. RESULTS: Despite receiving intravenous C1-INH (Cinryze Ò) 1000 U twice weekly for long-term prophylaxis, patients continued to have breakthrough attacks. Functional C1-INH levels were 33%, 40%, and 29%, respectively. Intravenous doses were administered in the clinic because patients had venous access problems. Doses were escalated to 2500 U in two patients and 1500 U in one patient. After dose escalation to 2500 U, functional C1-INH levels were 76% and 72%, respectively. Patients switched to subcutaneous C1-INH (Haegarda Ò) for multiple reasons, including the ability to administer treatment at home. After receiving subcutaneous C1-INH for 2 weeks, functional C1-INH levels were 72%, 91%, and 73%, respectively. Patients have received subcutaneous C1-INH for 10 months with no physician-confirmed attacks. CONCLUSIONS: Measurement of functional C1-INH levels was useful in identifying when patients required higher doses of C1-INH long-term prophylaxis. Patients had normal and sustained functional C1-INH levels and better control of symptoms after switching to subcutaneous C1-INH 60 IU/kg twice weekly.