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ASSESSMENT AND MANAGEMENT OF IgA NEPHROPATHY John Feehally

ASSESSMENT AND MANAGEMENT OF IgA NEPHROPATHY John Feehally. IgA NEPHROPATHY The commonest pattern of glomerulonephritis in the world. CLASSIFICATION OF GLOMERULONEPHRITIS. Histopathology. Clinical. Immune mechanisms. CLASSIFICATION OF GLOMERULONEPHRITIS. Histopathology. Clinical.

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ASSESSMENT AND MANAGEMENT OF IgA NEPHROPATHY John Feehally

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  1. ASSESSMENT AND MANAGEMENT OFIgA NEPHROPATHYJohn Feehally

  2. IgA NEPHROPATHYThe commonest pattern of glomerulonephritis in the world

  3. CLASSIFICATION OF GLOMERULONEPHRITIS Histopathology Clinical Immune mechanisms

  4. CLASSIFICATION OF GLOMERULONEPHRITIS Histopathology Clinical Immune mechanisms Patterns established on light microscopy Membranous Membranoproliferative Focal segmental glomerulosclerosis etc……

  5. CLASSIFICATION OF GLOMERULONEPHRITIS Histopathology Clinical Immune mechanisms Patterns established on light microscopy Membranous Membranoproliferative Focal segmental glomerulosclerosis etc…… ‘Patterns’ not ‘diseases’

  6. IgA1 deposition In the glomerular mesangium

  7. IgA NEPHROPATHY

  8. ASSESSMENT AND MANAGEMENT OFIgA NEPHROPATHYIs IgA nephropathya single ‘disease’ ?

  9. IgA NEPHROPATHYA pattern of glomerulonephritis with many variations

  10. Recurrent visible haematuria Coincides with mucosal infection

  11. Nephrotic syndrome

  12. Asymptomatic Haematuria / proteinuria

  13. CKD Proteinuria Hypertension Renal impairment

  14. HENOCH-SCHȌNLEIN NEPHRITIS

  15. Henoch-Schőnlein purpura

  16. ‘SECONDARY’ IgA NEPHROPATHY COMMONLY REPORTED ASSOCIATIONS Alcoholic liver disease Celiac disease Ankylosing spondylitis Reiter’s syndrome Uveitis Dermatitis herpetiformis

  17. RECURRENT IgA NEPHROPATHY

  18. RECURRENT IgA NEPHROPATHY Pooled published data – 5 year follow up Recurrence 38-60% Graft dysfunction due to recurrence 15% Graft loss due to recurrence 7%

  19. RECURRENT IgA NEPHROPATHY Pooled published data – 5 year follow up Recurrence 38-60% Graft dysfunction due to recurrence 15% Graft loss due to recurrence 7% Why does IgA nephropathy NOT always recur ?

  20. 15-21% 4.7% <5% Percentage of patients with primary glomerular disease

  21. 15-21% Male > Female Male = Female 4.7% <5%

  22. IgA NEPHROPATHYVariations in: Pathological pattern Clinical pattern Transplant recurrence Epidemiological patternPathogenesis

  23. IgA NEPHROPATHY Not expect a single pathogenic mechanism to lead to mesangial IgA deposition and injury No proof that IgAN is a single ‘disease’ No proof that IgAN is the same ‘disease’ in all parts of the world

  24. ASSESSMENT AND MANAGEMENT OFIgA NEPHROPATHY Can you predict which patients with IgA nephropathywill get kidney failure?

  25. ASSESSMENT AND MANAGEMENT OFIgA NEPHROPATHYCan you predict which patients with IgA nephropathywill get kidney failure?CLINICAL evidence

  26. PROGNOSIS IN IgA NEPHROPATHY Rodicio 1982

  27. PROGNOSIS IN IgA NEPHROPATHY 20% ESRD @ 20 years Rodicio 1982

  28. IgA NEPHROPATHY IN INDIA CMC Vellore 1994-2003 Chacko B et al. Nephrology 2005; 10: 496

  29. IgA NEPHROPATHY IN INDIA CMC Vellore 1994-2003 478 adults 55% - Nephrotic syndrome at presentation 56% - Serum creatinine > 123 μmol/L at presentation Chacko B et al. Nephrology 2005; 10: 496

  30. MACROSCOPIC HAEMATURIA AND PROGNOSIS IN IgA NEPHROPATHY Beukhof 1983

  31. LEAD TIME BIAS IN DIAGNOSIS OF IgA NEPHROPATHY Geddes CC et al. NDT 2003; 18: 1541

  32. ISOLATED NON-VISIBLE HAEMATURIA IN IgA NEPHROPATHY How benign is it ? Cohort study – Toronto – 286 patients Proteinuria < 0.2 g/24hr Normal BP Non-visible haematuria plus Bartosik et al. AJKD 2001; 38: 728

  33. ISOLATED MICROSCOPIC HAEMATURIA IN IgA NEPHROPATHY How benign is it ? Cohort study – Toronto – 286 patients Proteinuria < 0.2 g/24hr Normal BP Microscopic haematuria plus 10 year risk of deterioration in renal function = ZERO Bartosik et al. AJKD 2001; 38: 728

  34. ISOLATED NON-VISIBLE HAEMATURIA IN IgA NEPHROPATHY How benign is it ? Cohort study – Hong Kong Non-visible haematuria Proteinuria < 0.4 g/24hr plus During 7 years follow up, 44% had a ‘clinical event’ 33% proteinuria 26% hypertension 7% renal impairment Szeto C et al Am J Med 2001; 110:434

  35. OUTCOME AND AVERAGE FOLLOW-UP PROTEINURIA IN IgA NEPHROPATHY

  36. REMISSION OF PROTEINURIA IMPROVES PROGNOSIS IN IgA NEPHROPATHY • Time-average proteinuria • 1 - < 1g/24h • 2 – 1-2 g/24h • 3 – 2-3g/24h • 4 - >3g/24h Reich H et al. JASN 2007; 18: 3177

  37. ASSESSMENT AND MANAGEMENT OFIgA NEPHROPATHYCan you predict which patients with IgA nephropathywill get kidney failure?PATHOLOGICAL evidence

  38. A CLINICO-PATHOLOGICAL CLASSIFICATION FOR IgA NEPHROPATHY • Does pathology add prognostic information • .. to clinical data at time of biopsy ? • .. to clinical data during follow up ?

  39. A CLINICO-PATHOLOGICAL CLASSIFICATION FOR IgA NEPHROPATHY • Does pathology add prognostic information • .. to clinical data at time of biopsy ? • .. to clinical data during follow up ? Perhaps the biopsy is only useful to establish the diagnosis of IgAN ?

  40. PATHOLOGICAL CLASSIFICATIONS IN RENAL DISEASE Are usually based on expert opinion ... and pre-conceived ideas of what lesions are important

  41. OXFORD CLASSIFICATION OF IgA NEPHROPATHY A different way Approach the problem with an open mind With an international consensus group • Study all histological lesions • Test reproducibility & independence • Then test correlations with outcome

  42. SCORING OF SELECTED PATHOLOGY FEATURES Mesangial hypercellularity - in > or <50% of glomeruli M0 or M1 Endocapillary hypercellularity – present/absent E0 or E1 Segmental sclerosis/adhesions – present/absent S0 or S1 Tubular atrophy/interstitial fibrosis – 0-25%, 26-50%, >50% T0 or T1 or T2 Each can be scored easily in routine clinical practice

  43. PREDICTIVE SIGNIFICANCE OF PATHOLOGY FEATURES IN IgA NEPHROPATHY • M E S T • Each adds predictive value to …. • Initial clinical features • Follow up clinical features In all ages and races studied

  44. VALIDATION STUDIES FOR THE OXFORD CLASSIFICATION OF IgAN

  45. WHAT NEXT ? Validation studies Work towards combining pathology and clinical elements – to produce a single ‘risk score’ There is now the opportunity to design smaller, shorter RCTs

  46. ASSESSMENT AND MANAGEMENT OFIgA NEPHROPATHYHow good is the evidence to guide the treatment of IgA nephropathy ?

  47. KI Supplements 2012 2(2): 1-274 CLINICAL PRACTICE GUIDELINE FOR GLOMERULONEPHRITIS

  48. Examples of Rating Guideline Recommendations QUALITY of Supporting Evidence is shown as A, B, C or D

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