Abstract | OBJECTIVE: 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.
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Authors | Stephen 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 |
Journal | Rheumatology (Oxford, England)
(Rheumatology (Oxford))
Vol. 54
Issue 6
Pg. 1025-32
(Jun 2015)
ISSN: 1462-0332 [Electronic] England |
PMID | 25431483
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
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Copyright | © The Author 2014. Published by Oxford University Press on behalf of the British Society for Rheumatology. |
Chemical References |
- Immunosuppressive Agents
- Creatinine
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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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