Collapsing glomerulopathy in adult still's disease

Am J Kidney Dis. 2004 May;43(5):e4-10. doi: 10.1053/j.ajkd.2003.11.025.

Abstract

Idiopathic collapsing glomerulopathy is a clinically and pathologically distinct variant of focal segmental glomerulosclerosis characterized clinically by a male and Afro-Caribbean racial predominance, proteinuria (often nephrotic range), and rapid progression to end-stage renal failure. Pathologically, the typical changes are global glomerular collapse leading to obliteration of glomerular capillary lumina, hypertrophy and hyperplasia of podocytes, and severe tubulointerstitial changes. A secondary form with almost identical pathologic features is described in association with human immunodeficiency virus infection. We describe a female patient who presented with multisystemic manifestations, including high spiking fever, arthralgias, lymphadenopathy, striking hyperferritinemia, and impaired renal function with proteinuria. Renal biopsy showed classic collapsing glomerulopathy. A diagnosis of adult Still's disease was made on the basis of Yamaguchi's criteria. The patient was treated with steroids, resulting in remission of the rheumatological condition closely paralleled by remission of proteinuria and renal function, thereby strongly suggesting a causative link between adult Still's disease and collapsing glomerulopathy in this patient. We propose that collapsing glomerulopathy ought to be considered in adult Still's disease with unexplained renal insufficiency or proteinuria.

Publication types

  • Case Reports

MeSH terms

  • Biopsy
  • Female
  • Glomerulosclerosis, Focal Segmental / complications*
  • Glomerulosclerosis, Focal Segmental / pathology
  • Humans
  • Middle Aged
  • Proteinuria / complications
  • Still's Disease, Adult-Onset / complications*
  • Still's Disease, Adult-Onset / diagnosis