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Pênfigo vulgar e esquemas terapêuticos
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Introdução: O pênfigo vulgar é uma doença bolhosa autoimune mucocutânea, que acomete sobretudo adultos acima dos 40 anos, sem predileção por sexo. A abordagem terapêutica mais comum passa pela utilização de corticosteroides e/ou a realização de ciclos de rituximab, frequentemente em combinação com imunossupressores.
Objetivos: Caracterizar epidemiologicamente o pênfigo vulgar e avaliar a abordagem terapêutica.
Materiais e Métodos: Realizou-se um estudo retrospetivo no Hospital de Santa Maria, em Lisboa, tendo sido incluídos 99 pacientes com diagnóstico de pênfigo vulgar entre 2013 e 2022. Foram recolhidos, a partir das fichas clínicas, dados como o sexo, a idade, a distribuição das lesões e a terapêutica instituída. Estes foram, posteriormente, analisados e tratados estatisticamente.
Resultados e Discussão: A distribuição por sexos foi homogénea e a idade média no diagnóstico foi de 59,66 anos. As manifestações exclusivamente cutâneas foram as mais comuns (56,6%) e o tronco surge como localização cutânea mais acometida. 40,4% dos pacientes tinham manifestações orais. O tempo médio de evolução da sintomatologia antes do diagnóstico foi de 4,65 meses. O grupo farmacológico mais prescrito foi o dos corticosteroides e a prednisolona foi utilizada em 84,4% dos pacientes que cumpriram corticoterapia. Os imunossupressores foram o segundo grupo de fármacos mais utilizado, sendo a azatioprina o princípio ativo de eleição. O rituximab foi uma abordagem terapêutica em 21 pacientes. Ademais, também os anti-histamínicos foram prescritos em 31 pacientes. A combinação de fármacos mais utilizada foi a de um corticosteroide sistémico, um tópico e um imunossupressor, em 14 pacientes. O tempo médio de doença foi de 304 dias e o tempo sem doença ativa, para os indivíduos nos quais ocorreu recidiva (35,4%) foi, em média, de 415 dias.
Conclusões: No presente estudo, a terapia com corticosteroides foi a abordagem mais prevalente. Porém, atualmente, o tratamento de 1ª linha está cada vez mais direcionado para o uso de rituximab, empregue em cerca de ¼ da amostra em estudo.
Introduction: Pemphigus vulgaris is a mucocutaneous autoimmune blistering condition that predominantly affects adults over the age of 40, with no gender predilection. The most common treatment involves the use of systemic corticosteroids and/or cycles of rituximab, often in combination with immunosuppressants. Objectives: To study the epidemiological characteristics of pemphigus vulgaris and evaluate the therapeutic management. Materials and Methods: A retrospective study was conducted at Santa Maria Hospital in Lisbon, including 99 patients diagnosed with pemphigus vulgaris between 2013 and 2022. Data such as gender, age, topographic distribution of lesions, and prescribed therapy were collected from clinical records. These data were subsequently analyzed and statistically treated. Results and Discussion: Gender distribution was homogeneous and the mean age at diagnosis was 59.66 years. Exclusive cutaneous manifestations were the most common (56.6%), with the trunk being the most affected cutaneous location. 40.4% of patients had oral manifestations, with the buccal mucosa being the most affected region. The average duration of symptoms before diagnosis was 4.65 months. The main comorbidities associated at diagnosis were hypertension, obesity, and diabetes. Corticosteroids were the most prescribed pharmacological group, with prednisolone being used in 84.4% of patients who underwent corticosteroid therapy. Immunosuppressants were the second most used drug group, with azathioprine being the preferred active ingredient. Rituximab was used in 21 patients. Furthermore, antihistamines were prescribed to 31 patients. The most commonly used drug combination was one systemic corticosteroid, one topical corticosteroid, and one immunosuppressant, in 14 patients. The average disease duration was 304 days, and the time without active disease for individuals who experienced relapse (35.4%) averaged 415 days. Conclusions: In this study, corticosteroid therapy was the most prevalent treatment. However, currently, first-line treatment is increasingly directed towards the use of rituximab, employed in approximately a quarter of the study sample.
Introduction: Pemphigus vulgaris is a mucocutaneous autoimmune blistering condition that predominantly affects adults over the age of 40, with no gender predilection. The most common treatment involves the use of systemic corticosteroids and/or cycles of rituximab, often in combination with immunosuppressants. Objectives: To study the epidemiological characteristics of pemphigus vulgaris and evaluate the therapeutic management. Materials and Methods: A retrospective study was conducted at Santa Maria Hospital in Lisbon, including 99 patients diagnosed with pemphigus vulgaris between 2013 and 2022. Data such as gender, age, topographic distribution of lesions, and prescribed therapy were collected from clinical records. These data were subsequently analyzed and statistically treated. Results and Discussion: Gender distribution was homogeneous and the mean age at diagnosis was 59.66 years. Exclusive cutaneous manifestations were the most common (56.6%), with the trunk being the most affected cutaneous location. 40.4% of patients had oral manifestations, with the buccal mucosa being the most affected region. The average duration of symptoms before diagnosis was 4.65 months. The main comorbidities associated at diagnosis were hypertension, obesity, and diabetes. Corticosteroids were the most prescribed pharmacological group, with prednisolone being used in 84.4% of patients who underwent corticosteroid therapy. Immunosuppressants were the second most used drug group, with azathioprine being the preferred active ingredient. Rituximab was used in 21 patients. Furthermore, antihistamines were prescribed to 31 patients. The most commonly used drug combination was one systemic corticosteroid, one topical corticosteroid, and one immunosuppressant, in 14 patients. The average disease duration was 304 days, and the time without active disease for individuals who experienced relapse (35.4%) averaged 415 days. Conclusions: In this study, corticosteroid therapy was the most prevalent treatment. However, currently, first-line treatment is increasingly directed towards the use of rituximab, employed in approximately a quarter of the study sample.
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Teses de mestrado - 2023 Saúde Oral