title: Necrotizing and crescentic glomerulonephritis presenting with preserved renal function in patients with underlying multisystem autoimmune disease: a retrospective case series.
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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: 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.
authors.forename: Stephen P
authors.initials: SP
authors.lastname: McAdoo
authors.affiliation: 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.
authors.forename: Anisha
authors.initials: A
authors.lastname: Tanna
authors.affiliation: 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.
authors.forename: Olga
authors.initials: O
authors.lastname: Randone
authors.affiliation: 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.
authors.forename: Frederick W K
authors.initials: FW
authors.lastname: Tam
authors.affiliation: 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.
authors.forename: Ruth M
authors.initials: RM
authors.lastname: Tarzi
authors.affiliation: 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.
authors.forename: Jeremy B
authors.initials: JB
authors.lastname: Levy
authors.affiliation: 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.
authors.forename: Megan
authors.initials: M
authors.lastname: Griffith
authors.affiliation: 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.
authors.forename: Liz
authors.initials: L
authors.lastname: Lightstone
authors.affiliation: 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.
authors.forename: H Terence
authors.initials: HT
authors.lastname: Cook
authors.affiliation: 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.
authors.forename: Tom
authors.initials: T
authors.lastname: Cairns
authors.affiliation: 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.
authors.forename: Charles D
authors.initials: CD
authors.lastname: Pusey
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000150:complications / Q000188:drug therapy / Q000473:pathology*; D001706:Biopsy; D003404:Creatinine / Q000097:blood; D005260:Female; D005921:Glomerulonephritis / Q000150:complications / Q000188:drug therapy / Q000473:pathology*; D006801:Humans; D007165:Immunosuppression Therapy; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007676:Kidney Failure, Chronic / Q000517:prevention & control; D007678:Kidney Glomerulus / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D009336:Necrosis / Q000473:pathology; D011379:Prognosis; D012189:Retrospective Studies; D055815:Young Adult
chemical_list: D007166:Immunosuppressive Agents; D003404:Creatinine
keywords: Goodpasture’s syndrome; anti-neutrophil cytoplasm antibody; granulomatosis with polyangiitis; histopathology; microscopic polyangiitis; renal; systemic lupus erythematosus; vasculitis