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Vol. 17. Issue 2.
Pages 85-93 (March - April 2011)
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Vol. 17. Issue 2.
Pages 85-93 (March - April 2011)
Série de casos
DOI: 10.1016/S0873-2159(11)70020-4
Open Access
Infliximab no tratamento da sarcoidose - Experiência de um hospital central
Infliximab for treating sarcoidosis patients, Portuguese experience
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M. Aguiar
Corresponding author
m_aguiar@netcabo.pt

Autor para correspondência.
, N. Marçal, A.C. Mendes, A. Bugalho de Almeida
Serviço de Pneumologia 1, Hospital de Santa Maria, CHLN, EPE, Lisboa, Portugal
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Resumo

Apesar de tratamento agressivo e atempado, a Sarcoidose pode ser, por vezes, uma doença progressiva e bastante debilitante. O papel do factor de necrose tumoral (TNF)-α na génese dos granulomas é ambíguo. Por um lado a sua presença é necessária para a formação e manutenção da inflamação granulomatosa, sendo, portanto, o seu antagonista utilizado com sucesso no tratamento de doentes com sarcoidose; por outro lado e para outras situações, há referência do aparecimento dessa patologia em doentes submetidos a Infliximab.

O Infliximab foi por nós utilizado no tratamento de doentes com sarcoidose que não apresentavam resposta à corticoterapia ou a outras terapêuticas convencionais ou que desenvolviam efeitos secundários inaceitáveis a essa terapêutica. A dose inicial foi de 5mg/Kg de peso e as doses subsequentes foram administradas na 2.a semana, na 6.a semana e depois, durante o período de um ano de 8 em 8 semanas.

Dez doentes com diagnóstico de sarcoidose por biopsia, iniciaram terapêutica com este fármaco. Destes cinco eram homens e cinco mulheres, com uma idade média de 47,1 anos, variando a idade entre 28 e 63 anos. Três doentes apresentavam sintomas graves do sistema nervoso central, dois, cirrose hepática, uma inflamação granulomatosa da glândula lacrimal já submetida a múltiplas cirurgias, uma com envolvimento pulmonar extenso (estádio III), uma, com lúpus pérnio desfigurante e dois, com nódulos cutâneos desfigurantes. Oito doentes apresentavam mais do que um local de doença. Todos os doentes completaram pelo menos sete ciclos de tratamento. Em quatro doentes a dose de corticóides ou de outra terapêutica imunosupressora foi suspensa, em três foi reduzida, e apenas em uma doente foi necessário juntar corticóides à terapêutica com Infliximab. Cinco doentes apresentaram uma melhoria significativa. Um dos doentes com sintomas neurológicos apresentou resolução total, enquanto que a segunda melhorou substancialmente o seu deficit visual permitindo que voltasse a ler. Dois doentes suspenderam terapêutica, uma por falta de melhoria dos sintomas neurológicos e outra por aparecimento de pneumonia organizativa durante o tratamento com Infliximab. Três doentes desenvolveram anticorpos anti-histonas durante o tratamento.

O Infliximab parece ser eficaz no tratamento de doentes com sarcoidose refractária ou que desenvolvem efeitos secundários ao regime terapêutico habitual de corticóides ou outros agentes imunosupressivos. No entanto é necessário manter uma vigilância apertada destes doentes para identificar rapidamente efeitos secundários que possam surgir.

Palavras-chave:
Sarcoidose
Infliximab
Abstract

Despite aggressive treatment, sarcoidosis may be debilitating and progressive. The role of tumor necrosis factor (TNF)-α in the genesis of granulomas is ambiguous. It has proven to be critical in the formation and maintenance of granulomatous inflammation and its antagonist, Infliximab, has therefore been used with success in the treatment of patients with sarcoidosis. There are, however, reports of onset of sarcoidosis in patients in treatment for other conditions and that had no outbursts before submission to this therapy.

We used Infliximab in the treatment of patients with sarcoidosis who either didn’t respond to corticosteroids and other conventional drugs or developed unacceptable side effects to these drugs. The initial dose was 5mg/Kg body weight and subsequent doses were given at weeks 2, 4 and then every other 8 weeks for a total period of one year.

We treated ten patients with biopsy proven sarcoidosis, five men and five women, with a mean age of 47.1 years ranging from 28 to 63 years of age. Three patients had severe neurological symptoms, two had hepatic cirrhosis, one had granulomatous inflammation of the lachrymal gland and had already been submitted to many surgeries, one had extensive pulmonary involvement (stage III), one had disfiguring lupus pernio and two presented disabling cutaneous nodules. In four patients the dosage of corticosteroids or other immunosuppressive drugs was suspended, in three the dosage was reduced and in one, corticosteroids were added to the Infliximab therapy. In five of the patients there was a significant improvement. One of the patients with neurological symptoms displayed a complete recovery, while another had significant improvement of vision deficit enabling her to read again. Two patients withdrew from therapy, one due to lack of improvement of neurological symptoms and the other due to the onset of organizing pneumonia spawned by Infliximab. Two patients developed anti-histone antibodies during treatment.

Infliximab seems effective in treating patients who are either refractory or develop side effects to a standard regimen of corticosteroids and immunosuppressive agents. These patients, treated with Infliximab, should be under tight surveillance in order to quickly identify possible secondary effects.

Keywords:
Sarcoidosis
Infliximab
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Copyright © 2011. Sociedade Portuguesa de Pneumologia
Pulmonology

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