A case report of hypocomplementemic urticarial vasculitis presenting with membranoproliferative glomerulonephritis.
BMC Nephrol
; 21(1): 351, 2020 08 18.
Article
em En
| MEDLINE
| ID: mdl-32811472
ABSTRACT
BACKGROUND:
Hypocomplementemic urticarial vasculitis syndrome is an infrequent condition characterized by ocular, renal, gastrointestinal and pulmonary involvement with low serum complement levels and autoantibodies. Renal manifestations vary from microscopic hematuria to nephrotic syndrome and acute kidney injury. Accordingly differing histologic patterns have been reported. CASE PRESENTATION We present the case of a 65 years old woman with a history of chronic uveitis who presented with arthralgias, urticarial rush, nephrotic syndrome, glomerular hematuria and low serum complement. Kidney biopsy revealed an immune-complex membranoproliferative glomerulonephritis. The patient received induction therapy with steroids, cyclophosphamide and hydroxychloroquine followed by rapid clinical improvement and remission of proteinuria. Maintenance treatment consisted of rituximab pulses.CONCLUSIONS:
The majority of hypocomplementemic urticarial vasculitis syndrome cases is idiopathic, although an association to drugs, infections or other autoimmune disorders has been recorded. Given the rarity and heterogeneity of the disease, no standard treatment is established.Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Uveíte
/
Vasculite
/
Proteínas do Sistema Complemento
/
Glomerulonefrite Membranoproliferativa
/
Urticária Crônica
/
Síndrome Nefrótica
Limite:
Aged
/
Female
/
Humans
Idioma:
En
Revista:
BMC Nephrol
Assunto da revista:
NEFROLOGIA
Ano de publicação:
2020
Tipo de documento:
Article
País de afiliação:
Grécia