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Rheumatology (Oxford). 2015 Jun;54(6):1025-32. doi: 10.1093/rheumatology/keu445. Epub 2014 Nov 26.

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

Author information

1
Renal and Vascular Inflammation Section, Imperial College London, Multidisciplinary Vasculitis and Lupus Clinics, Imperial College Healthcare NHS Trust, London UK, Department of Nephrology, Ospedale Cardinal Massaia, Asti, Italy, Centre for Complement and Inflammation Research, Imperial College London, and Department of Pathology, Imperial College Healthcare NHS Trust, London UK Renal and Vascular Inflammation Section, Imperial College London, Multidisciplinary Vasculitis and Lupus Clinics, Imperial College Healthcare NHS Trust, London UK, Department of Nephrology, Ospedale Cardinal Massaia, Asti, Italy, Centre for Complement and Inflammation Research, Imperial College London, and Department of Pathology, Imperial College Healthcare NHS Trust, London UK s.mcadoo@imperial.ac.uk.
2
Renal and Vascular Inflammation Section, Imperial College London, Multidisciplinary Vasculitis and Lupus Clinics, Imperial College Healthcare NHS Trust, London UK, Department of Nephrology, Ospedale Cardinal Massaia, Asti, Italy, Centre for Complement and Inflammation Research, Imperial College London, and Department of Pathology, Imperial College Healthcare NHS Trust, London UK Renal and Vascular Inflammation Section, Imperial College London, Multidisciplinary Vasculitis and Lupus Clinics, Imperial College Healthcare NHS Trust, London UK, Department of Nephrology, Ospedale Cardinal Massaia, Asti, Italy, Centre for Complement and Inflammation Research, Imperial College London, and Department of Pathology, Imperial College Healthcare NHS Trust, London UK.
3
Renal and Vascular Inflammation Section, Imperial College London, Multidisciplinary Vasculitis and Lupus Clinics, Imperial College Healthcare NHS Trust, London UK, Department of Nephrology, Ospedale Cardinal Massaia, Asti, Italy, Centre for Complement and Inflammation Research, Imperial College London, and Department of Pathology, Imperial College Healthcare NHS Trust, London UK.

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.

KEYWORDS:

Goodpasture’s syndrome; anti-neutrophil cytoplasm antibody; granulomatosis with polyangiitis; histopathology; microscopic polyangiitis; renal; systemic lupus erythematosus; vasculitis

PMID:
25431483
PMCID:
PMC4476844
DOI:
10.1093/rheumatology/keu445
[Indexed for MEDLINE]
Free PMC Article

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