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<p>Systemic lupus erythematosus is an autoimmune disorder that may be characterized by renal disease, which can manifest as lupus nephritis with haematoproteinuria, and cardiac involvement, ranging from subclinical manifestations to cardiogenic shock. While cardiovascular disease is common, myocarditis is a rarer manifestation with a challenging diagnosis due to atypical presentation and lack of sensitivity of endomyocardial biopsy. The renal involvement may allow for a kidney biopsy, which usually demonstrates glomerulonephritis with immune complex deposition. However, a rare pauci-immune histopathological pattern may be present, especially if associated with positive anti-neutrophil cytoplasm antibodies (ANCA). In this report, we describe a case of cardiogenic shock related to lupus myocarditis, successfully supported with veno-arterial extracorporeal circulatory membrane oxygenation (VA-ECMO) and immunosuppression, associated with renal involvement as a crescentic pauci-immune glomerulonephritis due to lupus nephritis and ANCA-positive vasculitis overlap. This case illustrates the complexity in management and diagnosis in the presence of such rare cardiac and renal manifestations, while further contributing to our knowledge and awareness of uncommon organ affliction caused by this auto-immune disease.</p>

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Cardiogenic shock and crescentic pauci-immune glomerulonephritis associated with systemic lupus erythematosus and ANCA-vasculitis overlap: a case report

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2025年11月12日

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  • Ana Rita Bello,
  • Andreia Cristina Gaspar Carnevale,
  • António Tralhão,
  • Christopher Strong,
  • Catarina Brízido,
  • João Presume,
  • Ivo Laranjinha,
  • Rita Calça,
  • Patrícia Matias,
  • Célia Gil,
  • Margarida Gonçalves,
  • Rita Theias,
  • Patrícia Branco

摘要

Systemic lupus erythematosus is an autoimmune disorder that may be characterized by renal disease, which can manifest as lupus nephritis with haematoproteinuria, and cardiac involvement, ranging from subclinical manifestations to cardiogenic shock. While cardiovascular disease is common, myocarditis is a rarer manifestation with a challenging diagnosis due to atypical presentation and lack of sensitivity of endomyocardial biopsy. The renal involvement may allow for a kidney biopsy, which usually demonstrates glomerulonephritis with immune complex deposition. However, a rare pauci-immune histopathological pattern may be present, especially if associated with positive anti-neutrophil cytoplasm antibodies (ANCA). In this report, we describe a case of cardiogenic shock related to lupus myocarditis, successfully supported with veno-arterial extracorporeal circulatory membrane oxygenation (VA-ECMO) and immunosuppression, associated with renal involvement as a crescentic pauci-immune glomerulonephritis due to lupus nephritis and ANCA-positive vasculitis overlap. This case illustrates the complexity in management and diagnosis in the presence of such rare cardiac and renal manifestations, while further contributing to our knowledge and awareness of uncommon organ affliction caused by this auto-immune disease.