[Acquired angioedema due to C1-inhibitor deficiency: CREAK recommendations for diagnosis and treatment]. / Angiœdèmes par déficit acquis en C1-inhibiteur : recommandations du CREAK pour le diagnostic et la prise en charge.

Acquired angioedema with C1-inhibitor deficiency is a rare and peculiar entity belonging to the spectrum of bradykinin angioedemas. It usually occurs in subjects over 60 years old, and is mostly associated with a B-cell lymphoid hemopathy or a monoclonal gammopathy. The diagnosis relies on at least one angioedema episode, lasting more than 24 h, and on the decrease of functional C1-inhibitor. Low C1q is observed in 90% of patients, and an anti C1-inhibitor antibody is found in 50% of patients. The treatment of severe attacks relies on icatibant or C1-inhibitor perfusions. Long term prophylaxis in patients with frequent attacks requires treatment of the associated hemopathy if so. In case of idiopathic angioedema, tranexamic acid and danazol may be used, provided that there is-no thrombophilia; as well as rituximab as second-line treatment. Inhibitors of kallikrein still need to be evaluated in this therapeutic indication.

Angioedema associated with thrombolysis for ischemic stroke: analysis of a case-control study.

BACKGROUND: Bradykinin-mediated angioedema (AE) is a complication associated with thrombolysis for acute ischemic stroke. Risk factors are unknown and management is discussed. OBJECTIVES: To clarify risk factors associated with bradykinin-mediated AE after thrombolysis for acute ischemic stroke. METHODS: In a case-control study conducted at a French reference centre for bradykinin angiœdema, patients with thrombolysis for acute ischemic stroke and a diagnosis of bradykinin-mediated angiœdema, were compared to controls treated with thrombolysis treatment without angiœdema. RESULTS: Fifty-three thrombolysis-related AE were matched to 106 control subjects. The sites of attacks following thrombolysis for ischemic stroke mainly included tongue (34/53, 64%) and lips (26/53, 49%). The upper airways were involved in 37 (70%) cases. Three patients required mechanical ventilation. Patients with bradykinin-mediated angiœdema were more frequently women [33 (62%) vs. 44 (42%); P = 0.01], had higher frequency of prior ischemic stroke [12 (23%) vs. 9 (8%); P = 0.01], hypertension [46 (87%) vs. 70 (66%); P = 0.005], were more frequently treated with angiotensin-converting enzyme inhibitor [37 (70%) vs. 28 (26%); P < 0.001] and were more frequently hospitalized in intensive care medicine [ICU; 11 (21%) vs. 5 (5%); P = 0.004]. In multivariate analysis, factors associated with thrombolysis-related AE were female sex [odds ratio (OR), 3.04; 95% confident interval (CI), 1.32-7.01; P = 0.009] and treatment with angiotensin-converting enzyme inhibitors [(OR), 6.08; 95% (CI), 2.17-17.07; P < 0.001]. CONCLUSIONS: This case-control study points out angiotensin-converting enzyme inhibitors and female sex as risk factors of bradykinin AE associated with thrombolysis for ischemic stroke.

[Management of angiotensin-converting enzyme inhibitor-related angioedema: recommendations from the French National Center for Angioedema]. / Prise en charge des angiœdèmes induits par les inhibiteurs de l'enzyme de conversion de l'angiotensine : recommandations du Centre de référence national des angiœdèmes.

Angiotensin-converting enzyme (ACE) inhibitor-related angioedema (AE) may be fatal in the absence of specific treatment. No consensus for this side effect currently exists. Also, the French national reference centre for angioedema (CREAK) decided to establish recommendations, developed by an expert group and proposed at a national meeting. A scientific committee conducted a comprehensive literature review and worked out with proposals. These proposals were submitted to a vote to the expert panel of CREAK at a national meeting. Proposals that had received the majority were retained. Diagnosis of ACE inhibitor-related AE is based on clinical events. Regarding the severity of the disease, this diagnosis has to be put forward in any patient currently treated with or who has been treated with ACE inhibitors in the previous 6 months. The diagnosis is important because AE does not respond to usual treatment of histamine-induced AE (antihistamines, corticosteroids, and epinephrine), but only to specific treatment of bradykinin-induced AE, as antagonists of bradykinin or concentrates of C1 inhibitor. The subsequent use of ACE is strictly contra-indicated. A report to pharmacovigilance centres of every case is essential. These recommendations should improve the standardization of the management of ACE inhibitor-related AE.

[Bradykinin-mediated angioedema secondary to angiotensin converting enzyme: initiate treatment from the prehospital phase]. / Angiœdème bradykinique secondaire aux inhibiteurs de l'enzyme de conversion : instaurer le traitement en préhospitalier.

We are reporting the case of a bradykinin-mediated angioedema, secondary to the angiotensin converting enzyme inhibitors, which delayed treatment could have unfavorably influence the vital prognostic of the patient. Initially, the patient had an isolated edema of the superior lip. Prehospital treatment included methylprednisolone, hydroxyzine and epinephrine. The patient was subsequently taken to the emergency department. His situation deteriorated. An edema of the cheeks and the tongue appeared. The transfer of the patient to an emergency department, specializing in kinin angioedema was organized, in order for the patient to receive specific treatments. After a subcutaneous injection of icatibant, the situation improved very rapidly, with a regression of the edema. This observation is consistent with the early use of the specific therapeutic in bradykinin-mediated angioedema. Any delay in administering the treatment can negatively impact the prognostic. The availability of such treatments should therefore be organized during the prehospital phase.