5. Conclusion: a proposal for STP algorithm

Based on the clinical experience of the Department of Allergology of the University Hospital La Paz (Madrid) and the University General Hospital Nuestra Señora del Prado (Talavera de la Reina), these algorithms have been updated and modified.

Most studies do not take into account the disease severity in order to plan STP and to study the STP efficacy.

Jurado-Palomo et al. [15] calculated retrospectively the C1-INH-HAE severity by using the Diagnostic, Therapeutic, and Management Algorithm for Hereditary Angioedema, from Agostoni et al. [70] (Table 5). They studied the efficacy of STP with AAs and/or pdhC1INH in patients with C1-INH-HAE and found that all the patients who suffered mild pharynx laryngeal AE curiously occurred in the group of patients with milder stages of C1-INH-HAE. Curiously, these patients had not received long- or short-term AA prophylaxis nor pre-procedural pdhC1INH [15].


Table 5. Criteria for the evaluation of disease severity [70] (these parameters are determined over the period of 1 year. The sum of the scores defines the severity of the disease for that year).

We advise to classify procedures according to the risk of producing AE as minor, intermediate and major risks.

pdhC1INH reduced the AE risk more after invasive procedures than AAs and AAs more than tranexamic acid [73]. It is important to emphasize that the AE risk after surgical/medical procedures is not completely avoided with STP [71, 73] and is independent of C1-INH-HAE severity [15] and thus, at least one therapeutic dose of a specific treatment for acute AE attacks should be available during and after the procedure [8, 74]. If the procedure involves the ENT area, the patient should be informed about the possibility to develop a laryngeal oedema, not only in the 12 hours following the procedure but also later [72], and one should establish an action plan for the patient.

Based on the clinical experience of the Department of Allergology of the University Hospital La Paz (Madrid) and the University General Hospital Nuestra Señora del Prado (Talavera de la

Most studies do not take into account the disease severity in order to plan STP and to study the

Jurado-Palomo et al. [15] calculated retrospectively the C1-INH-HAE severity by using the Diagnostic, Therapeutic, and Management Algorithm for Hereditary Angioedema, from Agostoni et al. [70] (Table 5). They studied the efficacy of STP with AAs and/or pdhC1INH in patients with C1-INH-HAE and found that all the patients who suffered mild pharynx laryngeal AE curiously occurred in the group of patients with milder stages of C1-INH-HAE. Curiously, these patients had not received long- or short-term AA prophylaxis nor pre-proce-

Mild attacks (discomfort noticed, but no disruption of normal daily activities) 0.5 for each 24 hours Moderate attacks (discomfort sufficient to reduce or affect normal daily activities) 1 for each 24 hours Severe attacks (inability to work or perform daily activity) 2 for each 24 hours

Table 5. Criteria for the evaluation of disease severity [70] (these parameters are determined over the period of 1 year.

The sum of the scores defines the severity of the disease for that year).

Attack severity Score


5. Conclusion: a proposal for STP algorithm

196 A Comprehensive Review of Urticaria and Angioedema

Reina), these algorithms have been updated and modified.

STP efficacy.

dural pdhC1INH [15].

Need for treatment:

If we classified dental procedures according to the surgical risk, the injection of local anaesthetic would be of minor AE risk, but it has been identified to be able to precipitate an attack of AE [43]. In our series, even the placement of orthodontic appliances (lower risk according to our classification) showed triggering of mild palate oedema in the months after the placement [15]. Thus, it is important that patients have specific drugs, for the treatment of acute attacks, available.

We would recommend using danazol, stanozolol and tranexamic acid in minor risk manipulations and not using tranexamic acid for those procedures of intermediate or major AE risk and attenuated androgens if pdhC1INH is available.

Given this and in our experience, we would propose the following algorithms for the prophylaxis of DOMFOPs (Figure 5). To elaborate on this algorithm, three special mentions can be done:

Figure 5. Proposed algorithm for short-term prophylaxis in C1-INH-HAE according to the authors' experience at University Hospital La Paz in Madrid and at University General Hospital Nuestra Señora del Prado in Talavera de la Reina.


Close coordination between different specialists is advisable to decide the attitude to follow pre-procedurally. Treatment for acute attacks should be available in the operating room, in the allergology department and even at patient's home.
