Glomerular Diseases Flashcards
Renal biopsy findings in post streptococcal glomerulonephritis
Hypercellularity of mesangial and endothelial cells, glomerular infiltrates of polymorphonuclear leukocytes, granular subendothelial immune deposits of IgG, IgM, C3, C4 and C5-9 and subepithelial deposits (which appears as “humps”)
Renal pathology of Subacute Bacterial Endocarditis
Subcapsular hemorrhages, with a “flea-bitten” appearance, and microscopy on renal biopsy reveals focal proliferation around foci of necrosis associated with abundant mesangial, subendothelial, and subepithelial immune deposits of IgG, IgM, and C3
- commonly present with a clinical picture of RPGN and have crescents on biopsy
Most common clinical sign of renal disease in lupus nephritis
Proteinuria
Antibodies that correlate best with the presence of renal disease
Anti-dsDNA
Only reliable method of identifying the morphologic variants of lupus nephritis
Renal biopsy
Lupus nephritis that has normal glomerular histology by any technique or normal light microscopy with minimal mesangial deposits on immunoflourescent or electron microscopy
Class I (Minimal mesangial)
Lupus nephritis that designates mesangial immune complexes with mesangial proliferation
Class II (Mesangial proliferation)
Lupus nephritis that describes focal lesion with proliferation or scarring, often involving only a segment of the glomerulus
Class III (Focal nephritis)
Lupus nephritis that describes global, diffuse, proliferative lesions involving vast majority of the glomeruli
Class IV (Diffuse nephritis)
Class of lupus nephritis that has the worst renal prognosis without treatment
Class IV
Lupus nephritis lesion that describes subepithelial immune deposits producing a membranous pattern
Class V Lesion (Membranous nephritis)
Lupus nephritis class that is predisposed to renal-vein thrombosis and other thrombotic complications
Class V
Lupus nephritis that have >90% sclerotic glomeruli and ESRD with interstitial fibrosis
Class VI (Sclerotic nephritis)
A pulmonary renal syndrome that present with lung hemorrhage and glomerulonephritis, targets Type IV collagen
Goospasture’s syndrome
Renal biopsy show focal or segmental necrosis that later with aggressive destruction of the capillaries by cellular proliferation, leads to crescent formation in Bowman’s space
Anti-GBM disease
Recognized on biopsy by linear immunoflourescent staining for IgG
Anti-GBM disease
Treatment of Goodpasture syndrome patients with advanced renal failure who present with hemoptysis
Plasmapheresis
One of the most common forms of glomerulonephritis worldwide
IgA nephropathy
An immune complex-mediated glomerulonephritis defined by the presence of diffuse mesangial IgA deposits often associated with mesangial hypercellularity
IgA nephropathy
Two most common presentations of IgA nephropathy
- Recurrent episodes of macroscopic hematuria during or immediately following a URTI often accompanied by proteinuria
- Persistent asymptomatic microscopic hematuria
ANCA Small-vessel vasculitis
Granulomatosis with polyangiitis
Microscopic polyangiitis
Churg strauss Syndrome
Renal limited Vasculitis
ANCA that is more common in granulomatosis with polyangiitis
Anti-PR3
ANCA that is more common in microscopic polyangiitis or Churg-Strauss
Anti-MPO
Classically present with fever, purulent rhinorrhea, nasal ulcers, sinus pan, polyarthralgias/arthritis, cough, hemoptysis, shortness of breath, microscopic hematuria, and 0.5-1 g/24h of proteinuria
Granulomatosis with Polyangiitis
CXR finding of Granulomatosis with Polyangiitis
Nodules and persistent infiltrate, sometimes with cavities
Renal biopsy during active disease demonstrate segmental necrotizing glomerulonephritis as focal, mixed, crescenteric or sclerotic without immune deposits
Granulomatosis with Polyangiitis
Disease that is more common in patients exposed to silica dust and thosewith a1 antitrypsin deficiency
Granulomatosis with Polyangiitis
ANCA positive vasculitis with no granulomas on kidney biopsy
Microscopic polyangiitis
Associated with peripheral eosinophilia, cutaneous purpura, mononeuritis, asthma and allergic rhinitis
Churg-strauss syndrome
Commonly associated with persistent hepatitis C infections, autoimmune diseases like lupus or cryoglobulinemia or neoplastic diseases
Type I MPGN
The most proliferative of the three types of MPGN
Type 1 MPGN
Shows mesangial proliferation with lobular segmentation on renal biopsy and mesangial interposition between the capillary basement membrane and endothelial cells producing a double contour sometimes called “tram tracking”
Type 1 MPGN
Low serum C3 and a dense thickening of the GBM containing ribbons of dense deposits and C3
Type 2 MPGN (Dense deposit disease)
Treatment of MPGN in the presence of proteinuria
RAAS inhibitors
Characterized by expansion of the mesangium, sometimes associated with mesangial hypercellularity; thin , single contoured capillary walls, and mesangial immune deposits
Mesangioproliferative glomerulonephritis
Shows no obvious glomerular lesion by light microscopy and is negative for deposits by immunofluorescent microscopy or occasionally shows small amounts of IgM in the mesangium
Minimal change disease
Presents clinically with abrupt onset of edema and nephrotic syndrome accompanied by acellular urinary sediment
Light and immunoflourescent microscopy: negative findings
Electron microscope - effacement of foot processes
Minimal change disease
Treatment of minimal change disease
Steroids
MCD patients who have a complete remission after a single course of prednisone
Primary responders
MCD patients who have two or more relapses in the 6 months following taper
Frequent relapsers
Refers to a pattern of renal injury characterized by segmental glomerular scars that involve some but not all glomeruli , clinically manifests as proteinuria
Focal segmental glomerulosclerosis
Most common cause of nephrotic syndrome in the elderly
Membranous glomerulonephritis
Characterized by uniform thickening of the basement membrane along the peripheral capillary loops.
Electron microscopy reveals electron-dense subepithelial deposits
Membranous glomerulonephritis
Risk factors for the development of diabetic nephropathy
Hyperglycemia
Hypertension
Dyslipidemia
Smoking
Family history of diabetic nephropathy
Genetic polymorphisms affecting the activity of the RAAS
Timeline when morphologic changes appear in the kidney after the onset of clinical diabetes
Within 1-2 years
Eosinophilic , PAS+nodules which develop in some patients with diabetic nephropathy
Nordular glomerulosclerosis or Kimmelstiel-Wilson nodules
A potent risk factor for cardiovascular events and death in patients with Type 2 Diabetes
Microalbuminuria
How long does it take for diabetic nephropathy to reach ESRD from the earliest stages of microalbuminuria
10-20 years
