Doença celíaca revisitada

 

S. Rito Nobre¹, T. Silva², J.E. Pina Cabral¹

 

Resumo

A doença celíaca é uma enteropatia auto-imune, induzida pela ingestão de glúten, estando subjacente uma predisposição genética. Existe uma forte associação com as moléculas do complexo major de histocompatibilidade DQ2 e DQ8-DR4 mas outros factores estão seguramente envolvidos. O contexto epidemiológico da doença expandiu-se e, actualmente, é considerada a intolerância alimentar mais comum no mundo. O diagnóstico pode ser estabelecido em idades avançadas. Reconhece-se um amplo espectro de manifestações clínicas, de carácter sistémico e muitas vezes pauci-sintomático. As complicações mais graves são o desenvolvimento de neoplasias, designadamente linfomas. Os marcadores serológicos desempenham um papel importante, mercê da sua elevada sensibilidade e especificidade, na investigação diagnóstica, assim como na monitorização da adesão e resposta à terapêutica. Estão identificados vários padrões histológicos que englobam linfocitose intra-epitelial, hiperplasia das criptas e diversos graus de atrofia vilositária. O tratamento assenta numa dieta sem glúten, indefinidamente, o que conduz na maioria dos casos a remissão clínica, laboratorial e histológica. Nas formas graves poderá estar indicada alimentação parentérica e corticoterapia. A imunossupressão é benéfica nalgumas situações de doença celíaca refractária. Têm sido sugeridas alternativas à restrição de glúten mas ainda se encontram no domínio experimental.

 

Summary

Celiac disease is an autoimmune enteropathy, triggered by the ingestion of gluten, with a genetic predisposition. There is a strong association with major histocompatibility complex class II alleles DQ2 and DQ8-DR4, but there are other factors involved. The epidemiology of the disease has increased and it is now considered the most common food intolerance in the world. Diagnosis may be established in

older patients. There is a wide spectrum of clinical presentation, with a systemic and commonly mild character. The most serious complications are malignant tumours, namely lymphomas. Serological tests are important, given their high sensitivity and specificity, in diagnostic evaluation and to monitor adherence and response to treatment. There are several histological patterns, including intraepithelial lymphocytosis,

crypt hyperplasia, and different stages of villous atrophy. The conventional treatment is a lifelong gluten-free diet, which improves symptoms, laboratorial tests and pathological lesions in most patients. In severe cases it might be necessary parenteral nutrition and corticosteroid therapy. Immunosuppression is beneficial in some situations of refractory celiac disease. Some alternatives to gluten restriction have been suggested, but they are still experimental.

 

 

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⁽¹⁾ Serviço de Gastrenterologia dos Hospitais da Universidade de Coimbra, Coimbra, Portugal.

⁽²⁾ Unidade de Gastrenterologia do Centro Hospitalar de Cascais, Cascais, Portugal.

 

Correspondência:

Sónia Rito Nobre

Serviço de Gastrenterologia

Hospitais da Universidade de Coimbra

Avenida Bissaya Barreto e Praceta Mota Pinto

3000-075 Coimbra

Tel.: 239400483

Fax: 239701517

e-mail: soninobre@hotmail.com

 

Recebido para publicação: 22/02/2007

Aceite para publicação: 12/09/2007