Idiopathic Non-histaminergic Angioedema: Successful Treatment with Omalizumab in Five Patients.

Idiopathic non-histaminergic acquired angioedema (InH-AAE) is a rare disease characterized by AE resistant to antihistamines and a chronic course. We report five new cases of InH-AAE (two women and three men) with a rapid and dramatic response to the anti-immunoglobulin-E antibody omalizumab. In our literature review, we found 13 other relevant cases with a good response to this treatment. Overall, in 6 out of 18 patients, the doses of omalizumab required to prevent recurrences of attacks were higher than the licensed dose for chronic urticaria. No significant adverse effects have been reported.

Angioedema Triggered by Medication Blocking the Renin/Angiotensin System: Retrospective Study Using the French National Pharmacovigilance Database.

INTRODUCTION: Bradykinin-mediated angioedema (AE) is a rare side effect of some medications, including angiotensin converting enzyme inhibitors (ACEi) and angiotensin receptor blockers (ARB). In France, side-effects to treatments are reported to the national pharmacovigilance database. METHODS: The national MedDRA database was searched using the term "angioedema". Patients were included if they met the clinical criteria corresponding to bradykinin-mediated AE, if their C1-inhibitor levels were normal, and if they were treated with an ACEi or an ARB. RESULTS: 7998 cases of AE were reported between 1994 and 2013. Among these, 112 met the criteria for bradykinin-mediated AE with normal C1-inhibitor levels. On the 112 drug-AE, patients were treated with an ARB in 21% of cases (24 patients), or an ACEi in 77% of cases (88 patients), in combination with another treatment in 17 cases (mTORi for 3 patients, iDPP-4 for 1 patient, hormonal treatment for 7 patients). ENT involvement was reported in 90% of cases (tongue: 48.2%, larynx: 23.2%). The median duration of treatment before the first attack was 720 days, and the mean duration of attacks was 36.6 h. Forty-one percent (19/46) of patients relapsed after discontinuing treatment. CONCLUSION: Angioedema triggered by medication blocking the renin/angiotensin system is rare but potentially severe, with a high risk of recurrence despite cessation of the causative drug.

Lack of association of some chemokine system polymorphisms with the risks of death and hepatocellular carcinoma occurrence in patients with alcoholic cirrhosis: a prospective study.

BACKGROUND: Polymorphisms in genes encoding for the chemokines stromal cell-derived factor-1 (SDF-1)/CXCL12, monocyte chemotactic protein-1 (MCP-1)/CCL2, or for the chemokine receptors, CC chemokine receptor 5 (CCR5) or CC chemokine receptor 2 (CCR2) have been associated with the progression of hepatitis C virus-related liver injury and with various cancer development. Their influence on the prognosis of alcoholic liver disease is unknown. PATIENTS AND METHODS: SDF-1 3'A, MCP-1(-2518), CCR5-Delta32 and CCR2-64I polymorphisms, SDF-1alpha, regulated upon activation normal T cells expressed and secreted (RANTES)/CCL5 and MCP-1 sera levels were determined in 222 alcoholic patients, included at the time of cirrhosis diagnosis and prospectively followed up. RESULTS: Carriers and noncarriers of each genetic marker had similar baseline characteristics estimating the severity of liver disease. Mean time of follow-up of the cohort was 62.9+/-43.2 months. One hundred and forty-seven out of 222 (66.3%) patients were alive at the end of the study. The occurrence of death (75/222; 33.7%) or hepatocellular carcinoma (67/222; 30.1%) during follow-up was similar among carriers and noncarriers of each polymorphism. No association between the carriage of mutated alleles and chemokine sera levels was found: CCR5-Delta32/RANTES, SDF-1 3'A/SDF-1alpha and CCR2-64I or MCP-1(-2518)/MCP-1. Baseline RANTES, SDF-1alpha and MCP-1 sera levels were associated neither with the risk of death nor with the risk of hepatocellular carcinoma. CONCLUSIONS: The present study suggests the lack of association of SDF-1 3'A, MCP-1(-2518), CCR5-Delta32 and CCR2-64I polymorphisms with death and hepatocellular carcinoma occurrence in cirrhotic alcoholic patients.