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Portuguese Journal of Nephrology & Hypertension

versão impressa ISSN 0872-0169

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GASPAR, Ana et al. Systemic lupus erythematosus and ANCA-associated vasculites overlap syndrome: A case report and literature review of poliautoimmunity. Port J Nephrol Hypert [online]. 2021, vol.35, n.1, pp.47-50.  Epub 31-Mar-2021. ISSN 0872-0169.  https://doi.org/10.32932/pjnh.2021.04.118.

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.

Palavras-chave : ANCA-associated vasculitis; Primary biliary cholangitis; Systemic Lupus Erythematosus.

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