the role of renal biopsy in glomerular diseases …c1q nephropathy fibrillary glomerulonephritis...
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THE ROLE OF RENAL BIOPSY IN GLOMERULAR DISEASES DIAGNOSTIC
FRANCO FERRARIOFABIO PAGNI
Nephropathology CenterSan Gerardo Hospital-Monza
Milan-Bicocca UniversityItaly
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Percutaneous Renal Biopsy has afundamental role in the clinical practice ofNephrologists to determine an accuratediagnosis, prognosis and consequently thechoice of appropriate treatment.diagnosis, prognosis and consequently thechoice of appropriate treatment.
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Being renal biopsy an “invasive” pro-cedure,with possible risk of com-plications,hisevaluation should be performed in centerswith recognized Nephropathologicalexpertise and supported by allmethodologies (LM, IF, EM, Im-methodologies (LM, IF, EM, Im-munohistochemistry), indispensable for acorrect diagnosis. Moreover needs a strictclinico-pathological correlation.
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Reccomandations of International Rheumatologic and Nephrologic Societies
Among different clinical and laboratoryparameters,ultimately renal biopsy is considered as the“gold standard” for diagnosis,prognostic evaluation andtherapeutic intervention, while clinical features andlaboratory parameters frequently shows a modestcorrelationcorrelation
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Example of two cases of ANCA-associatedvasculitis demostrating the frequentdiscrepancies between clinical andmorphological features.morphological features.
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CASE 1A 78 years old caucasian male with history of 20 years hypertension.
In August 2011 fever, astenia and some migrant arthralgia.
Exams
S. Cratinine 1.58 mg/dl
Hg 9.4 gr/dl , Proteinuria 0.3 gr/die , Microscopic Haematuria
ANA, ENA, nDNA, LAC negative.
Cryoglobuline positive(Cryocrit 1%)
Normocomplementemia
C-ANCA (PR3) negative
P-ANCA positive (MPO 6)
Sonography: normal kidneys
Chest x-ray: NORMAL.
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In spite of mild renal insufficency and low titer of MPO-ANCA histological feature of diffuse
massive periglomerular granulomatous reaction necessitating strong therapy
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Impressive periglomerular granulomatous reaction.Glomerular remnant is evident.
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CASE 2A 78 years old caucasian female with history of
crusted rhinitis.
Normal renal function without urinary
abnormalities.
December 2011: slight fever, astenia, arthralgias andDecember 2011: slight fever, astenia, arthralgias and
tongue ulcerative lesion.
January 2012: she developed marked dispnea with
oliguria and was admitted to nephrology
department.
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Exames
S. Cratinine 6.2 mg/dl
Hg 7.72 gr/dl
Proteinuria 0.6 gr/die Microscopic Haematuria
ANA, ENA, nDNA, LAC, Cryoglobuline, Anti-GBM
antibody : negative
NormocomplementemiaNormocomplementemia
C-ANCA (PR3) negative
P-ANCA positive (MPO>100)
Sonography: normal kidneys
Chest x-ray: Suggestive of haemorrhagic alveolitis
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In spite of severe renal insufficency and hight titer of MPO_ANCA histological feature of
focal segmental necrotizing extracapillary glomerulonephritis.
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C3 FIBRINOGEN
Pauci immune pattern with scanty deposits of C3
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IMPACT OF RENAL BIOPSY IN CLINICAL MANAGEMENT
AUTHORS / n° of cases
PAONE / 100 TURNER / 80 COHEN / 108
NON DIAGNOSTIC RB 13% 3% 4%
CHANGED DIAGNOSIS
CHANGED PROGNOSIS
CHANGED THERAPY
--
--
19%
44%
57%
31%
63%
32%
34%
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Clinical Renal Syndromes are relatively few
Asymptomatic proteinuriaAsymptomatic hematuriaNephrotic syndromeNephritic syndromeRapidly progressive renal failureAcute renal failureChronic renal failure
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Mesangial
Endothelial
Also glomerular cells are relatively few:
Endothelial
Visceral epithelial(Podocytes)
Parietal epithelial
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Conversely the number of totally different
types of nephritis is extremely vaste and
quite every year there is a description of new
forms.forms.
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Disease that tipically cause the nephrotic syndrome
Focal segmental glomerulosclerosis (all variants)Idiophatic membranous glomerulophatyMinimal change glomerulophatyDiabetic glomerulosclerosisType I membranoproliferative glomerulonephritisIdiopathic mesangioproliferative glomerulonephritisAmyloidosisAmyloidosisC1q nephropathyFibrillary glomerulonephritisMonoclonal immunoglobulin deposition diseaseType II membranoproliferative glomerulonephritisPre-eclampsia/eclampsiaImmunotactoid glomerulopathyC3 nephropathyCollagenofibrotic glomerulopathy
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Disease that tipically cause hematuria and nephritis
Lupus nephritisIgA nephropathyIdiopatic immune complex proliferative glomerulonephritisPauci-immune/antineutrophil cytoplasm antibody glomerulonephritisPostinfectious acute diffuse proliferative glomerulonephritisThin basementmembrane lesionAntiglomerular basement membrane antibody glomerulonephritisAntiglomerular basement membrane antibody glomerulonephritisAlport’s
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Disease other than glomerulonephritis that tipically cause acute renal failure
Thrombotic microangiopathy (all types)Acute tubulointerstitial nephritisAcute interstitial nephritis IgG4 relatedAcute tubular necrosisAtheroembolizasionAtheroembolizasionLight chain cast nephropathyCortical necrosis
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Disease other than those already listed that tipically manifest aschronic renal failure
ArterionephrosclerosisChronic sclerosing glomerulonephrtisEnd stage renal disease not otherwise specifiedChronic tubulointerstitial nephritisChronic tubulointerstitial nephritisMiscellaneous other diseasesAdeguate tissue with nonspecific abnormalitiesNo pathologic lesion identified
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For all these reasons a correct diagnostic evaluation of
renal biopsy needs nephropathological expertise
supported by all methodologies.
To confirme this we show some example of the main
histological lesions.histological lesions.
All morphological slides show similar
lesions in four different diseases.
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MESANGIAL PROLIFERATION
Definition: At least 4-5 cells per Definition: At least 4-5 cells per
mesangial area
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A B
C D
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Immunofluorescence
• A: Mesangial deposits of IgA
• B: Segmental deposits of IgM• B: Segmental deposits of IgM
• C: Mesangial deposits of C1q
• D: Granular deposits of C3
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A B
Mesangial deposits of IgA Segmental deposits of IgM
Mesangial deposits of C1q Granular deposits of C3
C D
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Electron microscopy
• A: Mesangial electrondense deposits
• B: Foot process effacement• B: Foot process effacement
• C: Finger prints
• D: Subendothelial and subepithelial deposits
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Mesangial deposits Foot processes effacemement
A B
Finger prints Subendothelial + subepithelial deposits
C D
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Final diagnosis
Mesangioproliferative GN
• A: IgA nephropathy
• B: IgM nephropathy• B: IgM nephropathy
• C: Lupus nephritis(class II mesangial)
• D: C3 nephropathy
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BASEMENT MEMBRANE ALTERATIONS
A: Diffuse regular thickenig of glomerular
basement membranes
B: Diffuse “ spikes “ at silver stainB: Diffuse “ spikes “ at silver stain
C: Huge subendothelial deposits
D: Double contour appearance at silver stain
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Thickenig of GBM Spikes
A B
C D
subendothelial deposits Double contours
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Immunofluorescence
A: Diffuse granular parietal subepithelial deposits of IgG
B: Subendothelial deposits of C3
Electron Microscopy
C: Continous subepithelial deposits
D: Double contour with subendothelial deposits
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Granular parietal subepithelial deposits Subendothelial deposits
A B
Subepithelial deposits Subendothelial deposits(double contour)
C D
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Final Diagnosis
• A-B: Membranous glomerulonephritis
• C-D: Membranoproliferative glomerulonephritis