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Rúpus : como e quando diagnosticar?
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Introdução: O rúpus é um síndrome pouco frequente no qual os doentes têm manifestações de Lúpus Eritematoso Sistémico (LES) e de Artrite Reumatóide (AR), tendo sido discutido se corresponde a um verdadeiro overlap destas duas doenças, se representa um subgrupo de doentes com LES com poliartrite erosiva ou se é uma entidade própria. Casos clínicos: São descritos 2 casos clínicos de rúpus, em mulheres de 30 e 57 anos, respetivamente. À data do diagnóstico, ambas apresentavam critérios diagnósticos de LES e AR. Ambas aparentavam ter pouco tempo de doença e nenhuma apresentava erosões ósseas. Conseguiu-se remissão completa do quadro no 1º caso após a introdução do metotrexato e parcial significativa no 2º caso apenas com hidroxicloroquina, estando o metotrexato a ser considerado. Em termos prognósticos, a 1ª doente terá pior prognóstico, visto ter mais critérios de classificação de LES. Discussão: No rúpus, as manifestações de LES e de AR podem distanciar no tempo, obrigando a considerar a hipótese de que uma revisão nos critérios diagnósticos desta síndrome pode resultar no diagnóstico dum maior número de casos, bem como no diagnóstico mais precoce. Um maior número de casos identificados pode resultar num melhor conhecimento da doença e em estabelecimento de protocolos terapêuticos bem fundamentados. O fármaco de eleição no rúpus é o metotrexato, segundo a maioria dos autores, embora estejam descritos doentes tratados com outros fármacos, com boa resposta. Nos casos apresentados, uma doente apenas conseguiu remissão completa após a introdução do metotrexato; a outra doente conseguiu uma remissão parcial muito significativa apenas com a hidroxicloroquina. Conclusões: O rúpus é uma doença que cursa com manifestações de LES e AR. Apesar de pouco frequente, é importante reconhecê-la pois isso pode ter implicações terapêuticas e, não menos importante, pode ter benefícios no impacto psicológico do diagnóstico.
Introduction: Rhupus is an uncommon syndrome in which patients have manifestations of both, systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA). The debate remains on whether it’s a true overlap of these two diseases, whether it represents a subgroup of SLE patients with erosive polyarthritis or if it is an entity of its own. Clinical cases: We report two clinical cases of rhupus, the first of a 30-year-old woman and the second of a 57-year-old woman. At the time of diagnosis, both had classification criteria of SLE and RA. Both appeared to have the disease for a short time and none had bone erosions. The first patient achieved complete remission with methotrexate. The second achieved a significant response with hydroxychloroquine alone. Concerning the prognosis, the first patient has more SLE classification criteria and therefore a worse prognosis. Discussion: In Rhupus there may exist significant time lapses between SLE and RA manifestations. It therefore seems reasonable to consider reviewing the diagnostic criteria of rhupus, which may result in the diagnosis of a greater number of cases, as well as in the earlier diagnosis of the disease. The identification of more rhupus patients may result in a better knowledge of the disease and in the establishment of therapeutic protocols. A covering to most authors, the drug of choice for rhupus is methotrexate, however, there are patients treated with other drugs who also have a favourable outcome. In the presented cases, one patient achieved full remission with methotrexate and the other a very significant response with hydroxychloroquine alone. Conclusions: Rhupus is a disease that presents with SLE and RA manifestations. Although uncommon, it is important to recognize it early on, since this may have therapeutic implications and, not least important, it may have benefits in terms of the psychological impact of the diagnosis.
Introduction: Rhupus is an uncommon syndrome in which patients have manifestations of both, systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA). The debate remains on whether it’s a true overlap of these two diseases, whether it represents a subgroup of SLE patients with erosive polyarthritis or if it is an entity of its own. Clinical cases: We report two clinical cases of rhupus, the first of a 30-year-old woman and the second of a 57-year-old woman. At the time of diagnosis, both had classification criteria of SLE and RA. Both appeared to have the disease for a short time and none had bone erosions. The first patient achieved complete remission with methotrexate. The second achieved a significant response with hydroxychloroquine alone. Concerning the prognosis, the first patient has more SLE classification criteria and therefore a worse prognosis. Discussion: In Rhupus there may exist significant time lapses between SLE and RA manifestations. It therefore seems reasonable to consider reviewing the diagnostic criteria of rhupus, which may result in the diagnosis of a greater number of cases, as well as in the earlier diagnosis of the disease. The identification of more rhupus patients may result in a better knowledge of the disease and in the establishment of therapeutic protocols. A covering to most authors, the drug of choice for rhupus is methotrexate, however, there are patients treated with other drugs who also have a favourable outcome. In the presented cases, one patient achieved full remission with methotrexate and the other a very significant response with hydroxychloroquine alone. Conclusions: Rhupus is a disease that presents with SLE and RA manifestations. Although uncommon, it is important to recognize it early on, since this may have therapeutic implications and, not least important, it may have benefits in terms of the psychological impact of the diagnosis.
Descrição
Trabalho Final do Curso de Mestrado Integrado em Medicina, Faculdade de Medicina, Universidade de Lisboa, 2019
Palavras-chave
Rúpus Lúpus eritematoso sistémico Artrite reumatóide Diagnóstico