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From membranoproliferative glomerulonephritis to a final diagnosis: Hypocomplementemic urticarial vasculitis syndrome

Marques, Joana ; Pereira, Tiago ; Carvalho, Dulce ; Góis, Mário ; Sousa, Helena ; Ribeiro, Francisco ; Nolasco, Fernando

Portuguese journal of nephrology & hypertension, 2021-12, Vol.35 (4), p.254-259

Sociedade Portuguesa de Nefrologia

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  • Título:
    From membranoproliferative glomerulonephritis to a final diagnosis: Hypocomplementemic urticarial vasculitis syndrome
  • Autor: Marques, Joana ; Pereira, Tiago ; Carvalho, Dulce ; Góis, Mário ; Sousa, Helena ; Ribeiro, Francisco ; Nolasco, Fernando
  • Assuntos: anti-c1q antibodies ; hypocomplementemia ; hypocomplementemic urticarial vasculitis syndrome ; membranoproliferative glomerulonephritis ; systemic lupus erythematosus ; UROLOGY & NEPHROLOGY ; urticaria
  • É parte de: Portuguese journal of nephrology & hypertension, 2021-12, Vol.35 (4), p.254-259
  • Descrição: Membranoproliferative glomerulonephritis describes a glomerular-injury pattern common to a heterogeneous group of diseases. Evaluation based on clinical and laboratory presentation and immunofluorescence staining on kidney biopsy allows identification of underlying pathophysiological processes and may facilitate proper diagnosis and treatment. Hypocomplementemic urticarial vasculitis syndrome is a rare autoimmune disease of multi-organ involvement. The diagnosis is based on well-defined clinical and laboratory criteria. The pathophysiology is not completely understood but the presence of anti-C1q antibody seems to be involved. Renal involvement occurs in up to 50% of cases. It can be heterogeneous and can be indistinguishable from lupus nephritis. Serological findings and skin involvement distinguish these two entities. We report the case of a 40-year-old female who presented with urticarial skin lesions, hypocomplementemia and nephrotic syndrome. Kidney biopsy showed membranoproliferative glomerulonephritis with full house immune complex deposits. The diagnosis of hypocomplementemic urticarial vasculitis syndrome was made and the patient was successfully treated with prednisolone and mycophenolate mofetil.
  • Editor: Sociedade Portuguesa de Nefrologia
  • Idioma: Inglês;Português
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  • Realização: ABCD-USP USP   Logos de Redes Sociais: Freepik

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