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Amiloidose hepática : um caso clínico
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Resumo(s)
Resumo: A amiloidose AA é uma patologia multissistémica, associada a um processo inflamatório crónico, na qual ocorre deposição de uma proteína de fase aguda, a proteína amiloide A sérica. Apesar de o envolvimento hepático ser frequente, na grande maioria dos casos tem apresentação subclínica. Hepatomegalia e testes de função hepática no limite superior do normal, em especial a fosfatase alcalina, são os achados mais frequentes. Os autores descrevem o caso clínico de um doente renal crónico com espondilartropatia seronegativa sob terapêutica biológica com sintomatologia inicial inespecífica e alteração das provas hepáticas com padrão laboratorial colestático. Os diversos diagnósticos diferenciais de colestase foram sequencialmente excluídos e o diagnóstico definitivo de amiloidose AA apenas foi possível após biópsia hepática. O atraso diagnóstico e a disfunção renal e hepática progressivas não possibilitaram a estabilização do doente, apesar do tratamento com tocilizumab, tendo este falecido 2 anos após o início da sintomatologia.
Summary: AA amyloidosis is a multisystemic disorder associated with a chronic inflammatory disease in which deposition of an acute phase protein, serum amyloid A protein, occurs. Although hepatic involvement is frequent, most cases have subclinical presentation. Hepatomegaly and liver function tests in the upper limit of normal, especially alkaline phosphatase, are the most frequent findings. The authors describe the clinical case of a chronic renal patient and seronegative spondylarthropathy under biological therapy with nonspecific initial symptomatology associated with a cholestatic laboratory standard. The various differential diagnoses of cholestasis were sequentially excluded and definitive diagnosis of AA amyloidosis was only possible after liver biopsy. Diagnostic delay and progressive renal and hepatic dysfunction did not allow stabilization of the patient despite treatment with tocilizumab, who died 2 years after symptom onset.
Summary: AA amyloidosis is a multisystemic disorder associated with a chronic inflammatory disease in which deposition of an acute phase protein, serum amyloid A protein, occurs. Although hepatic involvement is frequent, most cases have subclinical presentation. Hepatomegaly and liver function tests in the upper limit of normal, especially alkaline phosphatase, are the most frequent findings. The authors describe the clinical case of a chronic renal patient and seronegative spondylarthropathy under biological therapy with nonspecific initial symptomatology associated with a cholestatic laboratory standard. The various differential diagnoses of cholestasis were sequentially excluded and definitive diagnosis of AA amyloidosis was only possible after liver biopsy. Diagnostic delay and progressive renal and hepatic dysfunction did not allow stabilization of the patient despite treatment with tocilizumab, who died 2 years after symptom onset.
Descrição
Trabalho Final do Curso de Mestrado Integrado em Medicina, Faculdade de Medicina, Universidade de Lisboa, 2018
Palavras-chave
Amiloidose AA Espondilite anquilosante Amiloidose hepática Colestase Gastroenterologia