Atopia e auto-imunidade – A propósito de um caso clínico
Atopy and autoimmunity – A case report
M. Alfaro1,4,
, F. Tapadinhas1,4, A.M. Neves2,4, J. Costa Trindade3,4
Corresponding author
pediatria@hdfaro.min-saude.pt
Correspondência: Hospital Distrital de Faro, Rua Leão Penedo, 8000- Faro
Correspondência: Hospital Distrital de Faro, Rua Leão Penedo, 8000- Faro
1 Interna(o) do Internato Complementar de Pediatria do Hospital Distrital de Faro
2 Assistente Hospitalar Graduada de Pediatria do Hospital Santa Maria
3 Chefe de Serviço de Pediatria do Hospital Santa Maria. Professor Agregado de Pediatria da Faculdade de Medicina de Lisboa. Coordenador da Unidade de Imunoalergologia do Departamento da Criança e da Familia do Hospital de Santa Maria
4 Serviços de Pediatria do Hospital Distrital de Faro e do Hospital de Santa Maria
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A atopia e a auto-imunidade são classicamente referidas como reacções imunológicas distintas. Estudos recentes sugerem a existência de possíveis mecanismos patogénicos comuns.</p><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Descrevemos o caso de uma adolescente com antecedentes familiares de asma brônquica e de <span class="elsevierStyleItalic">miastenia gravis</span> materna (HLA-B8) e com antecedentes pessoais de infecções respiratórias altas de repetição dos dois aos quatro anos, iniciando as pneumonias desde os cinco anos (3-4 por ano, em 3 anos consecutivos), associados a dispneia e hipoxemia, necessitando com frequência de internamento hospitalar. Na investigação efectuada os marcadores de atopia revelaram-se inicialmente negativos, sendo excluídas outras hipóteses, como tuberculose, fibrose quística, défice de α-1 anti-tripsina, cardiopatia congénita, malformações broncopulmonares ou aspiração de corpo estranho. Mais tarde, a repetição das análises confirmou finalmente atopia com IgE, RAST e testes de sensibilidade cutânea positivos (ácaros e pólenes). Foram detectados imunocomplexos circulantes e défice de IgG2, 3 e 4. O estudo dos auto-anticorpos mais frequentes e das precipitinas foi negativo e o estudo dos antigénios de histocompatibilidade revelou a presença de HLA-B8 (idêntico ao materno). A cintigrafia de ventilação-perfusão e as provas de função respiratória foram normais. Fez terapêutica com anti-histamínicos, corticóides tópicos e broncodilatadores, verificando-se a diminuição progressiva do número de infecções respiratórias e boa evolução clínica. Aos 16 anos voltou a ser internada, desta vez com o diagnóstico de eritema nodoso e suspeita clínica de sindroma de Sweet, com boa evolução.</p><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">A relação entre atopia e auto-imunidade é enfatizada pelos autores, não correspondendo a sua ocorrência concomitante a uma associação meramente estatística, mas a um desequilíbrio global do sistema imunológico, com envolvimento simultâneo de diferentes tipos de hipersensibilidade.</p>" ] "en" => array:2 [ "titulo" => "Abstract" "resumen" => "<p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Atopy, immunodeficiency and autoimmunity are manifestations of immune system dysfunction. Classically atopy and autoimmunity are referred as distinct immunological reactions. Recent studies suggest the existence of common pathogenic mechanisms.</p><p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">We report the case of a teenager with familial history of asthma and miasthenia gravis in her mother (HLAB8+) and personal history of recurrent upper respiratory infections from two to four years old, and pneumonia since five years old (3 or 4 episodes/ year, in three consecutive years), with associated dyspnoea and hypoxemia, requiring frequently hospital admission. Investigation was initially negative for atopy markers, and excluded other hypothesis as tuberculosis, cystic fibrosis, α-1 antitrypsin deficiency, congenital heart disease, bronchopulmonary malformations or foreign body aspiration. Latter, further exams finally confirmed atopy with a rised IgE, positive RAST and cutaneous sensitivity tests (for house dust mites and pollen) and revealed circulating immune complexes and IgG 2, 3 e 4 deficit. Most frequent autoantibodies and precipitins study were negative, and histocompatibility antigens study revealed HLA-B8 (as her mother). Ventilation-perfusion scintigraphy and respiratory function tests were normal. Antihistamines, topical corticoids and bronchodilators were done with an excellent clinical response. At 16 years-old she is admitted again with the diagnosis of <span class="elsevierStyleItalic">erythema nodosum</span> and the clinical suspicion of Sweet's syndrome, having a good evolution.</p><p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">The relation between atopy and autoimmunity is enfatized by the authors. This simultaneous occurrence does not correspond merely to a statistical association, but may represent a global immune system impairment, with the involvement of different types of hypersensibility.</p>" ] ] "bibliografia" => array:2 [ "titulo" => "Bibliografia" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:21 [ 0 => array:3 [ "identificador" => "bib1" "etiqueta" => "1." "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Allergic disease and autoimmune effectors pathways" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "M. Rottem" 1 => "M.E. Gershwin" 2 => "Y. 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ISSN: 21735115Original language: Portuguese
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