Chronic urticaria (CU) has a widespread spectrum on causal or exacerbating factors, clinical manifestations, therapeutic response and quality of life affectation. Registries are useful tools in several real-life diagnosis and management approach. We aimed to evaluate the characteristics of CU patients living in Latin America through an original cross-sectional registry with data entered by regional allergologists. Results Three hundred patients were included, being 72% female, with median age of 36 years (1–85) and 20 months of CU median evolution time. The cause of CU was reported as unknown in 72% of them. Thirty-nine percent of suspected cases presented positive serology for Mycoplasma, positive autologous serum skin test (ASST) was reported in 47%, and occasional presence of thyroid or antinuclear autoantibodies and parasites. The impact of pruritus in their quality of life was moderate to severe in 60% of patients, with almost 3 out of four patients having partial or lack of urticaria control with anti-histamines. Conclusions Our registry provides retrospective data on the real-life assistance of a large number of patients from the region. Continuous search for associated conditions and better treatment possibilities are needed, in order to control the significant impact on quality of life and the length of disease.

Background Few studies have explored the association between obstructive sleep apnea (OSA) and chronic urticaria (CU). Our study aims to fill this gap by determining the frequency of the risk categories for OSA and how they might correlate with the specific CU patient reported outcome measures urticaria activity score (UAS7), urticaria control test (UCT) and CU quality of life questionnaire (CU-Q2oL). Methods We conducted a cross-sectional study involving a cohort of 171 Latin American CU patients. Descriptive statistics were used to determine frequency and proportions for demographic and clinical variables, while a chi-squared test for association between STOP-Bang OSA questionnaire categories and both UAS7 and UCT categories was performed to analyze how such variables interact. To further assess the strength of the correlation a Cramer's V coefficient was reported. Finally, a Kendall-Tau b correlation coefficient was performed to measure the correlation between the STOP-Bang score and other independent continuous variables. Results The average STOP-Bang score was 2.5, with 24% and 21% of patients falling into the intermediate and high-risk category for moderate-to-severe OSA, respectively. There was a strong statistically significant association (Cramer's V = 0.263; p = .000) between UAS-7 categories and STOP-Bang risk categories . A similar pattern of strong significant association (Cramer's V = .269; p = .002) was observed between UCT categories and STOP-Bang risk categories. A weak positive correlation between the STOP-Bang score and the CU-Q2oL average score (τ b = 0.188, p = .001) was identified. Overall, 72.5% patients reported limitations with respect to sleep in a varied degree according to the CU-Q2oL. Conclusions Our results suggest that a considerable proportion of patients with CU are at intermediate to high risk for OSA. Higher disease activity, poor CU control, and worse quality of life were all found to be associated with an increased risk. Additional studies are needed to determine the exact link between these conditions, and to determine whether screening and treatment for OSA might benefit patients with CU.

Background Patients with deficiencies of terminal components of complement are at hundreds to thousands fold increased risk of severe and fatal Neisseria spp. infections compared with the general population. Eculizumab is a newly approved monoclonal antibody C5 complement inhibitor. It is indicated for the treatment of atypical hemolytic uremic syndrome (atypical HUS), myasthenia gravis, and paroxysmal nocturnal hemoglobinuria. Because of the complement-depleting effect of Eculizumab dosing (Soliris®, Alexion Pharmaceuticals, Munich, Germany), patients are immunosuppressed for specific infectious pathogens (including Neisseria species) against which protection partially relies on normal complement activity. Because Eculizumab treatment is associated with a dramatically increased risk of Neisseria species. infections, recommendations for Neisseria meningitidis vaccination and antibiotic prophylaxis are contained in Eculizumab prescribing information. However, the most appropriate prevention of infections after Eculizumab has yet to be determined. Methods Case report and literature review. Results A previously healthy 7-year-old male was diagnosed with atypical HUS which included renal failure progressing to dialysis, persistent thrombocytopenia, hemolytic anemia, and hemoglobinuria. Stool cultures and a stool multiplex PCR panel did not detect Shiga-like producing E. coli nor E. coli O157/H7. Eculizumab dosing was therefore planned and Infectious Diseases consultation was obtained for appropriate preventions. The FDA Prescribing Information recommends Neisseria meningitidis vaccination before starting Eculizumab or, if immediate Eculizumab is necessary, to use antibiotic prophylaxis until 2 weeks after vaccination. The accepted protective titer after meningococcal vaccination is population based and uses the serum bactericidal assay (SBA). An antibody titer of >1:4 (human compliment) or 1:8 (rabbit complement) is considered protective. However, this “gold standard” assay incorporates the use of exogenous human or rabbit complement. The protective SBA titers in subjects with terminal complement component deficiencies may not be properly assessed using these same SBA titer protective thresholds. Furthermore, serious meningococcal infections have occurred after appropriate vaccination in patients receiving chronic Eculizumab treatments (ie for paroxysmal nocturnal hemoglobinuria). Finally, SBA protective levels after single Neisseria meningitidis vaccination have not been achieved in majorities of patients with renal failure receiving dialysis and or transplant immunosuppression. Conclusions The current Eculizumab prescribing information recommendations for vaccination and antimicrobial prophylaxis may be inadequate to prevent serious Neisseria infections. Repeated Neisseria meningitidis vaccination and extended antibiotic prophylaxis may afford better protection in patients chronically dosed with Eculizumab.

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