Hypocomplementemic Urticarial Vasculitis Syndrome With Crescentic Glomerulonephritis.
Am J Med Sci
; 355(2): 195-200, 2018 Feb.
Article
em En
| MEDLINE
| ID: mdl-29406049
ABSTRACT
Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a rare autoimmune disease characterized by multiple organ system involvement, including renal disease, with low complement levels. We report the case of a 31-year-old woman who presented with nonspecific symptoms including fatigue, diarrhea, macular rash and abdominal pain with acute renal failure leading to end-stage kidney disease. Laboratory results showed hematuria, nephrotic range proteinuria, worsening creatinine and low C1q levels. Left kidney biopsy showed proliferative glomerulonephritis with crescent formation. She was treated with 6 months of intravenous cyclophosphamide, followed by 2 doses of intravenous rituximab (1g each), thereafter maintained on mycophenolate mofetil and glucocorticoid-based therapy. She experienced a full recovery of renal function after 12 months of dialysis dependence. Hypocomplementemic urticarial vasculitis syndrome with crescentic glomerulonephritis is a rare disease with only 5 other reported cases in literature. In our case, we document a delayed but excellent renal recovery during a 2-year follow-up.
Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Contexto em Saúde:
3_ND
Base de dados:
MEDLINE
Assunto principal:
Urticária
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Vasculite
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Glomerulonefrite Membranoproliferativa
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Ciclofosfamida
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Rituximab
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Ácido Micofenólico
Limite:
Adult
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Female
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Humans
Idioma:
En
Revista:
Am J Med Sci
Ano de publicação:
2018
Tipo de documento:
Article