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Necrotizing and crescentic glomerulonephritis presenting with preserved renal function in patients with underlying multisystem autoimmune disease: a retrospective case series.

AbstractOBJECTIVE:
Necrotizing and crescentic GN usually presents with rapidly declining renal function, often in association with multisystem autoimmune disease, with a poor outcome if left untreated. We aimed to describe the features of patients who have presented with these histopathological findings but minimal disturbance of renal function.
METHODS:
We conducted a retrospective review (1995-2011) of all adult patients with native renal biopsy-proven necrotizing or crescentic GN and normal serum creatinine (<120 μmol/l) at our centre.
RESULTS:
Thirty-eight patients were identified. The median creatinine at presentation was 84 μmol/l and the median proportion of glomeruli affected by necrosis or crescents was 32%. Clinicopathological diagnoses were ANCA-associated GN (74%), LN (18%), anti-GBM disease (5%) and HScP (3%). Only 18% of cases had pre-existing diagnoses of underlying multisystem autoimmune disease, although the majority (89%) had extra-renal manifestations accompanying the renal diagnosis. All patients received immunosuppression and most had good long-term renal outcomes (median duration of follow-up 50 months), although two progressed to end-stage renal disease within 3 years. We estimate that renal biopsy had an important influence on treatment decisions in 82% of cases.
CONCLUSION:
Necrotizing and crescentic GN may present in patients with no or only minor disturbance of renal function. This often occurs in patients with underlying systemic autoimmune disease; early referral for biopsy may affect management and improve long-term outcomes in these cases.
AuthorsStephen P McAdoo, Anisha Tanna, Olga Randone, Frederick W K Tam, Ruth M Tarzi, Jeremy B Levy, Megan Griffith, Liz Lightstone, H Terence Cook, Tom Cairns, Charles D Pusey
JournalRheumatology (Oxford, England) (Rheumatology (Oxford)) Vol. 54 Issue 6 Pg. 1025-32 (Jun 2015) ISSN: 1462-0332 [Electronic] England
PMID25431483 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
Copyright© The Author 2014. Published by Oxford University Press on behalf of the British Society for Rheumatology.
Chemical References
  • Immunosuppressive Agents
  • Creatinine
Topics
  • Adolescent
  • Adult
  • Aged
  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis (complications, drug therapy, pathology)
  • Biopsy
  • Creatinine (blood)
  • Female
  • Glomerulonephritis (complications, drug therapy, pathology)
  • Humans
  • Immunosuppression Therapy
  • Immunosuppressive Agents (therapeutic use)
  • Kidney Failure, Chronic (prevention & control)
  • Kidney Glomerulus (pathology)
  • Male
  • Middle Aged
  • Necrosis (pathology)
  • Prognosis
  • Retrospective Studies
  • Young Adult

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