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        "resumen" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Atopia&#44; imunodefici&#234;ncia e auto-imunidade s&#227;o manifesta&#231;&#245;es de disfun&#231;&#227;o do sistema imunol&#243;gico&#46; A atopia e a auto-imunidade s&#227;o classicamente referidas como reac&#231;&#245;es imunol&#243;gicas distintas&#46; Estudos recentes sugerem a exist&#234;ncia de poss&#237;veis mecanismos patog&#233;nicos comuns&#46;</p><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Descrevemos o caso de uma adolescente com antecedentes familiares de asma br&#244;nquica e de <span class="elsevierStyleItalic">miastenia gravis</span> materna &#40;HLA-B8&#41; e com antecedentes pessoais de infec&#231;&#245;es respirat&#243;rias altas de repeti&#231;&#227;o dos dois aos quatro anos&#44; iniciando as pneumonias desde os cinco anos &#40;3-4 por ano&#44; em 3 anos consecutivos&#41;&#44; associados a dispneia e hipoxemia&#44; necessitando com frequ&#234;ncia de internamento hospitalar&#46; Na investiga&#231;&#227;o efectuada os marcadores de atopia revelaram-se inicialmente negativos&#44; sendo exclu&#237;das outras hip&#243;teses&#44; como tuberculose&#44; fibrose qu&#237;stica&#44; d&#233;fice de &#945;-1 anti-tripsina&#44; cardiopatia cong&#233;nita&#44; malforma&#231;&#245;es broncopulmonares ou aspira&#231;&#227;o de corpo estranho&#46; Mais tarde&#44; a repeti&#231;&#227;o das an&#225;lises confirmou finalmente atopia com IgE&#44; RAST e testes de sensibilidade cut&#226;nea positivos &#40;&#225;caros e p&#243;lenes&#41;&#46; Foram detectados imunocomplexos circulantes e d&#233;fice de IgG2&#44; 3 e 4&#46; O estudo dos auto-anticorpos mais frequentes e das precipitinas foi negativo e o estudo dos antig&#233;nios de histocompatibilidade revelou a presen&#231;a de HLA-B8 &#40;id&#234;ntico ao materno&#41;&#46; A cintigrafia de ventila&#231;&#227;o-perfus&#227;o e as provas de fun&#231;&#227;o respirat&#243;ria foram normais&#46; Fez terap&#234;utica com anti-histam&#237;nicos&#44; cortic&#243;ides t&#243;picos e broncodilatadores&#44; verificando-se a diminui&#231;&#227;o progressiva do n&#250;mero de infec&#231;&#245;es respirat&#243;rias e boa evolu&#231;&#227;o cl&#237;nica&#46; Aos 16 anos voltou a ser internada&#44; desta vez com o diagn&#243;stico de eritema nodoso e suspeita cl&#237;nica de sindroma de Sweet&#44; com boa evolu&#231;&#227;o&#46;</p><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">A rela&#231;&#227;o entre atopia e auto-imunidade &#233; enfatizada pelos autores&#44; n&#227;o correspondendo a sua ocorr&#234;ncia concomitante a uma associa&#231;&#227;o meramente estat&#237;stica&#44; mas a um desequil&#237;brio global do sistema imunol&#243;gico&#44; com envolvimento simult&#226;neo de diferentes tipos de hipersensibilidade&#46;</p>"
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        "resumen" => "<p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Atopy&#44; immunodeficiency and autoimmunity are manifestations of immune system dysfunction&#46; Classically atopy and autoimmunity are referred as distinct immunological reactions&#46; Recent studies suggest the existence of common pathogenic mechanisms&#46;</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 &#40;HLAB8&#43;&#41; and personal history of recurrent upper respiratory infections from two to four years old&#44; and pneumonia since five years old &#40;3 or 4 episodes&#47; year&#44; in three consecutive years&#41;&#44; with associated dyspnoea and hypoxemia&#44; requiring frequently hospital admission&#46; Investigation was initially negative for atopy markers&#44; and excluded other hypothesis as tuberculosis&#44; cystic fibrosis&#44; &#945;-1 antitrypsin deficiency&#44; congenital heart disease&#44; bronchopulmonary malformations or foreign body aspiration&#46; Latter&#44; further exams finally confirmed atopy with a rised IgE&#44; positive RAST and cutaneous sensitivity tests &#40;for house dust mites and pollen&#41; and revealed circulating immune complexes and IgG 2&#44; 3 e 4 deficit&#46; Most frequent autoantibodies and precipitins study were negative&#44; and histocompatibility antigens study revealed HLA-B8 &#40;as her mother&#41;&#46; Ventilation-perfusion scintigraphy and respiratory function tests were normal&#46; Antihistamines&#44; topical corticoids and bronchodilators were done with an excellent clinical response&#46; At 16 years-old she is admitted again with the diagnosis of <span class="elsevierStyleItalic">erythema nodosum</span> and the clinical suspicion of Sweet&#39;s syndrome&#44; having a good evolution&#46;</p><p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">The relation between atopy and autoimmunity is enfatized by the authors&#46; This simultaneous occurrence does not correspond merely to a statistical association&#44; but may represent a global immune system impairment&#44; with the involvement of different types of hypersensibility&#46;</p>"
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Vol. 13. Issue 5.
Pages 729-735 (September - October 2007)
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Vol. 13. Issue 5.
Pages 729-735 (September - October 2007)
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Atopia e auto-imunidade – A propósito de um caso clínico
Atopy and autoimmunity – A case report
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M. Alfaro1,4,
Corresponding author
pediatria@hdfaro.min-saude.pt

Correspondência: Hospital Distrital de Faro, Rua Leão Penedo, 8000- Faro
, F. Tapadinhas1,4, A.M. Neves2,4, J. Costa Trindade3,4
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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Resumo

Atopia, imunodeficiência e auto-imunidade são manifestações de disfunção do sistema imunológico. A atopia e a auto-imunidade são classicamente referidas como reacções imunológicas distintas. Estudos recentes sugerem a existência de possíveis mecanismos patogénicos comuns.

Descrevemos o caso de uma adolescente com antecedentes familiares de asma brônquica e de miastenia gravis 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.

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.

Palavras-chave:
Alergia
auto-imunidade
asma
eritema nodoso
criança
Abstract

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.

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 erythema nodosum and the clinical suspicion of Sweet's syndrome, having a good evolution.

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.

Key-words:
Allergy
autoimmunity
asthma
erythema nodosum
child
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