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Systemic lupus erythematosus and ANCA-associated vasculites overlap syndrome: A case report and literature review of poliautoimmunity

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Resumo:ABSTRACT Systemic lupus erythematosus and ANCA-associated vasculitis overlap syndrome is rare and complex. Moreover, the mechanisms that explain the interaction between these two conditions are still unclear. The authors describe the case of a patient who had myeloperoxidase ANCA-associated vasculitis as the initial diagnosis, with biopsy-proven pauci-immune crescentic glomerulonephritis, attaining complete remission after immunosuppression. Five years later, Systemic Lupus Erythematosus was diagnosed and a second kidney biopsy showed a pattern of lupus nephritis class III. While on immunosuppression treatment, the patient developed a progressive elevation of liver enzymes and was later diagnosed with primary biliary cholangitis. It seems that in this case, the overlap of Systemic Lupus Erythematosus and ANCA-associated vasculitis may be part of a poliautoimmune syndrome suggested by association with a third autoimmune disease.
Autores principais:Gaspar,Ana
Outros Autores:Lima,Anna; Santos,Afonso; Brás,Catarina; Manso,Rita Theias; Soto,Karina
Assunto:ANCA-associated vasculitis Primary biliary cholangitis Systemic Lupus Erythematosus
Ano:2021
País:Portugal
Tipo de documento:relatório
Tipo de acesso:acesso aberto
Instituição associada:Fundação para a Ciência e Tecnologia
Idioma:português
Origem:SciELO Portugal
Descrição
Resumo:ABSTRACT Systemic lupus erythematosus and ANCA-associated vasculitis overlap syndrome is rare and complex. Moreover, the mechanisms that explain the interaction between these two conditions are still unclear. The authors describe the case of a patient who had myeloperoxidase ANCA-associated vasculitis as the initial diagnosis, with biopsy-proven pauci-immune crescentic glomerulonephritis, attaining complete remission after immunosuppression. Five years later, Systemic Lupus Erythematosus was diagnosed and a second kidney biopsy showed a pattern of lupus nephritis class III. While on immunosuppression treatment, the patient developed a progressive elevation of liver enzymes and was later diagnosed with primary biliary cholangitis. It seems that in this case, the overlap of Systemic Lupus Erythematosus and ANCA-associated vasculitis may be part of a poliautoimmune syndrome suggested by association with a third autoimmune disease.