Background: Current guidelines on chronic spontaneous urticaria (CSU) suggest a treatment based on a 3-step approach that aims at total symptom control, starting with H1-antihistamines. However, a significant number of patients present an antihistamine-resistant urticaria that must be treated with an alternative third-line therapy such as omalizumab. Methods: Patients with a history of CSU who did not respond to treatment with high doses of modern antihistamines were treated with 150 or 300 mg of omalizumab every 4 weeks. The response to treatment was recorded as complete (CR), partial (PR) or no response. A dose adjustment was proposed according to response. Results: We treated 47 CSU patients with omalizumab (40 females), of whom 39.5% had evidence of autoimmunity. The average number of treatments was 11.4 (range 2-87). All patients had been refractory to high-dose modern antihistamines. A CR was seen in 84.6% of patients who started with 300 mg and in 60% of those who started with 150 mg. Only 1 patient had no response to both the 150- and 300-mg doses. In 6 of the PR patients with 150 mg, a higher dose of 300 mg was proposed and 4 had a CR. Four patients discontinued the treatment. No severe adverse events were reported in the patients who finished the study. Discussion: Although good results were seen in both groups, CR rates were higher in those under a high-dose initial treatment. Our data strongly suggest that the therapy should be individualized.
BACKGROUND: Chronic urticaria affects patients by interfering with their daily activities, damaging their self-esteem and negatively affecting their interpersonal relationships. Healthcare professionals may underestimate the impact of the condition on patients' quality of life. OBJECTIVES: To evaluate quality of life using one generic and one disease-specific health-related quality of life instrument; to compare quality of life between the different clinical types of chronic urticaria and to evaluate whether angioedema further impairs quality of life. METHODS: Sixty-two patients with chronic urticaria, who had had signs or symptoms of the disease within the preceding week, were divided into groups according to whether they had ordinary chronic urticaria, physical chronic urticaria or mixed chronic urticaria. RESULTS: There was a predominance of women in this study (72.6%). The mean age of patients was 39.8 years and angioedema was associated with chronic urticaria in 75.8% of cases. Associated angioedema was more commonly found in patients with ordinary chronic urticaria (p=0.011) and in women (p=0.024). With respect to the different clinical types, 32.3% of the patients had ordinary chronic urticaria, 27.4% had physical chronic urticaria and 40.3% had mixed chronic urticaria. Mean overall score for the disease-specific questionnaire was 10.4. In this questionnaire, the most affected domains were "symptoms and feelings" and "daily activities", while the most affected domains in the SF-36 were "physical role" and "vitality". Quality of life was found to be impaired in women, in patients of up to 30 years of age, in those attending a first consultation, in those with higher education levels, in patients who had had the disease for up to one year and in those with angioedema. CONCLUSIONS: Chronic urticaria affects quality of life, as measured using a disease-specific questionnaire and a generic questionnaire. There was no statistically significant difference between the clinical types. The presence of angioedema impaired patients' quality of life even further. There was a statistically significant correlation between the scores obtained using the disease-specific questionnaire and those obtained using the generic questionnaire. Keywords: Angioedema; Quality of life; Questionnaires; Sickness impact profile; Urticaria Resumo: FUNDAMENTOS: A urticária crônica compromete o doente por interferir nas atividades diárias, prejudicar a autoestima e as relações interpessoais. Os profissionais de saúde subestimam seu impacto na qualidade de vida dos doentes. OBJETIVOS: Avaliar a qualidade de vida com questionário específico e genérico. Compará-la entre os tipos clínicos de urticária crônica e avaliar se o angioedema piora a qualidade de vida. MÉTODOS: Participaram 62 doentes com urticária crônica, com sinais e sintomas da doença até 7 dias da consulta, que foram divididos em urticária crônica comum, urticária crônica física e urticária crônica mista. RESULTADOS: Observou-se predominância de mulheres (72,6%), idade m...
In this article, three cases of hereditary angioedema (HAE) type III (estrogen-dependent or with normal C1 inhibitor) are reported. The HAE was initially described in women of the same family in association with high-leveled estrogenic conditions such as the use of oral contraceptives and pregnancy. There is no change in the C1 inhibitor as happens in other types of hereditary angioedema, and mutations are observed in the encoding gene of the XII factor of coagulation in several patients. The current diagnosis is mainly clinical and treatment consists in the suspension of the triggering factors and control of acute symptoms. A brief review of physiopathology, clinical features, genetic alterations and treatment are also presented.
Background Urticarias are frequent diseases, with 15% to 20% of the population presenting at least one acute episode in their lifetime. Urticaria are classified in acute ( ≤ 6 weeks) or chronic (> 6 weeks). They may be induced or spontaneous. Objectives To verify the diagnostic and therapeutic recommendations in chronic spontaneous urticaria (CSU), according to the experience of Brazilian experts, regarding the available guidelines (international and US). Methods A questionnaire was sent to Brazilian experts, with questions concerning diagnostic and therapeutic recommendations for CSU in adults. Results Sixteen Brazilian experts answered the questionnaire related to diagnosis and therapy of CSU in adults and data were analyzed. Final text was written, considering the available guidelines (International and US), adapted to the medical practices in Brazil. Diagnostic work up in CSU is rarely necessary. Biopsy of skin lesion and histopathology may be indicated to rule out other diseases, such as, urticarial vasculitis. Other laboratory tests, such as complete blood count, CRP, ESR and thyroid screening. Treatment of CSU includes second-generation anti-histamines (sgAH) at licensed doses, sgAH two, three to fourfold doses (non-licensed) and omalizumab. Other drugs, such as, cyclosporine, immunomodulatory drugs and immunosuppressants may be indicated (non-licensed and with limited scientific evidence). Conclusions Most of the Brazilian experts in this study partially agreed with the diagnostic and therapeutic recommendations of the International and US guidelines. They agreed with the use of sgAH at licensed doses. Increase in the dose to fourfold of sgAH may be suggested with restrictions, due to its non-licensed dose. Sedating anti-histamines, as suggested by the US guideline, are indicated by some of the Brazilian experts, due to its availability. Adaptations are mandatory in the treatment of CSU, due to scarce or lack of other therapeutic resources in the public health system in Brazil, such as omalizumab or cyclosporine.
Resumo: Urticária aquagênica é forma rara de urticária física caracterizada por aparecimento de urticas após o contato com água, independente da temperatura. Há poucos casos descritos de urticária aquagênica e, destes, somente cinco da forma familiar. Apresentamos o primeiro relato de urticária aquagênica familiar no Brasil, acometendo mãe e filha. Ambas apresentavam urticas, principalmente após banho de chuveiro, independentemente da temperatura da água. A mãe referia ter o quadro há quatro anos, e a filha, desde o nascimento. Para diagnóstico, foram realizados testes de provocação com água, com aparecimento de lesões em ambas, e testes com dermografômetro, com cubo de gelo envolvido em plástico e de provocação para urticária colinérgica, sem o aparecimento de lesões, excluindo assim outras formas de urticária física. Palavras-chave: Água; Antagonistas dos receptores H1 de histamina; Prurido; Urticária Abstract: Aquagenic urticaria is a rare form of physical urticaria, characterized by pruritic wheals that appear following contact with water, independently of its temperature. There are few reports of cases of aquagenic urticaria, and only five include the familial form. We present the first case of familial aquagenic urticaria in Brazil (mother and daughter). Both patients presented wheals following contact with water, especially when showering, regardless of its temperature. The mother reported onset of urticaria four years before and the daughter presented wheals since birth. For diagnostic purposes, they were submitted to a challenge test with water, and both subjects presented wheals, as well as to tests using ice cubes in plastic bag with dermographometer and challenge tests for cholinergic urticaria, with no appearance of lesions, excluding other forms of physical urticaria.
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