Copyright ©The Author(s) 2015.
World J Nephrol. May 6, 2015; 4(2): 185-195
Published online May 6, 2015. doi: 10.5527/wjn.v4.i2.185
Table 1 Classification of haematuric diseases by histopathological findings
Glomerular endothelial cell layerGBM disordersMesangial depositsPodocytary slit diaphragm disordersSubendothelial/subepithelial depositOthers
ANCAPrimaryIgANMYH9 diseasePrimary GNWRN
EndocapillaryAlportHSPFabry diseaseMBPSCD
Secondary GN
Anti-GBM diseaseCryoglobulinemia
C3 glomerulopathy
CFHR5 nephropathyFibrillar deposit
Table 2 Possible pathogenic mechanisms of haematuria
DiseaseMolecular defectPrevalenceMain glomerular defect Clinical expression
HaematuriaProteinuriaCKD progression
Genetic disorder
GFB structural damage
Structural GBM damage
ALPORTX-linked: COL4A5 AR: COL4A3/COL4A41/50000GBMMHVariable100% approximately 20-30 yr
TBMDCOL4A3/COL4A41%GBMMHUsually absent20% CKD
HANACCOL4A13 familiesGBMMH or grossNot describedVariable
Structural podocyte damage
MYH9Non muscle myosin IIA heavy chain1:100000NoneMHVariableESRD by young adulthood
Storage disorders
Fibronectine GNFibronectin44 casesMesangial/subendoth60% MH93% variable degreeESRD at 20-60 yr
Fibrillary10-30 nm fibrilsRareMesangial /GBMMH 47%-73% Gross 5%Present 41%-55% nephrotic50% ESRD in few years
Immunotactoid> 30 nm fibrils10-fold rarer than FGNMesangial/subepith/subendothMH 80%100%17% ESRD in 3 yr
Fabry’s diseaseLysosomal storage1:3100- 1:1600All the cellsMHUsually nephroticESRD after age 50 yr
Complement mediated
C3 glomerulopathyAlternative pathway1-2 × 106Mesangial/GBMMH 87%38%Variable
Inflammatory disorders
ANCAAb vs endothelium10-20 × 106EndotheliumMHVariableVariable
Anti GBMAb vs COL40.5-1 × 106/yrGBMMHVariableVariable
Infections (endocapillary)
Primary GN (IgAN, membranoproliferative, crescentic)
IgANGalactose-deficient IgA110%-16%MesangialMH always 75% grossRare nephrotic Usual proteinuria20% ESRD 20 yr after diagnosis
WRNUnknown16.5% non-CKD 33% CKDNoneUsually MHNoneAccelerated CKD progression
LPHSUnknownUnknownGBM (?)MH or grossAbsent or minimalGFR > 60