Glomerulonephritis Flashcards
Clinical features of IgA nephropathy
Usually young male
Recurrent episodes of macroscopic haematuria
Typically associated with mucosal infections
Rarely has nephrotic range proteinuria
Diagnostic features of IgA nephropathy
Mesangial hypercellularity in histology
Positive immunofluorescence for IgA and C3
Clinical features of post-strep glomerulonephritis
URTI precedes GN - typically an interval of 1-6 weeks
Nephritis syndrome
- haematuria (tea, cola-coloured urine)
- hypertension
- oedema
Associated with LOW C3 and normal C4 levels levels
GN that can recur
IgA nephropathy (most common)
FSGC (most rapid)
Membranoproliferative GN (Mesangiocapillary)
Membranous GN
Features of lupus nephritis
Proteinuria
Haematuria
Hypertension
Oedema
Reduced kidney function
Renal colic or flank pain
Fever and fatigue
What GN is associated with low C3 levels
Poststreptococcal GN
Lupus nephritis
Membranoproliferative GN
Histological features of proliferative lupus nephritis
Subendothelial immune deposits/wire loops
Hypercellularity
Leukocyte infiltration
Fibrinoid necrosis
Hyaline thrombi
Crescents in severe LN
Treatment of lupus nephritis
Immunosuppressants - steroids, cyclophosphamide, MMF, azathioprine
Biologics - rituximab, belimumab
ACE inhibitors
NSAIDs
Anticoagulants
Dietary and lifestyle changes
Dialysis or kidney transplant
Principle of treatment in lupus nephritis
Induction - prednisone, cyclophosphamide, MMF, rituximab (refractory cases)
Maintenance - MMF or azathioprine for at least 2 years post remission
Feature of pauci-immune rapidly progressive GN
Severe form of kidney disease
Characterised by inflammation of glomeruli and rapidly declining kidney function
Associated with autoimmune disorders
Treatment of pauci-immune RPGN
Immunosuppressive therapy - steroids, cyclophosphamide, rituximab
Plasmapheresis
Maintenance therapy
Supportive Care
Kidney transplant
Features of membranoproliferative GN
Nephrotic syndrome
Significant proteinuria
Haematuria with dysmorphic red cells and red cell casts**
Histology features of FSGS
Podocyte detachment leading to segmental sclerosis ***
Collapsing lesions
Hyalinosis
Adhesions
Clinical features of FSGS
Characterised by sclerosis affecting only some segments of glomeruli
Nephrotic syndrome
Poor prognostic markers of IgAN
Elevated serum Cr
HTN
Proteinuria > 1g/day
Clinical features of MN
Proteinuria (nephrotic range or sub-nephrotic range)
Microscopic haematuria
Normal or impaired renal function
HTN
Nephrotic syndrome **
Hyperlipidaemia *
Thromboembolic complications*
Secondary causes for MN
Lupus nephritis
Hepatitis B and C
Malignancy (lung, colon, stomach and kidney)
Medications (NSAIDs, gold, penicillamine)
Biopsy results for MN
LM - immune complex deposition in basement membrane “spike and hold”
Granular staining for IgG
EM - electron dense deposits in GBM in subepithelial regions
Antibodies present in MN
Anti-PLA2R (70-80% of primary cases), correlates with activity
THSD7a antibodies (in 3-5% of pts who were anti-PLA2R negative)
GN most likely to rapidly recur
FSGS
Characterised by massive proteinuria within hours to days after implantation of new kidney
Features of Alport’s syndrome
Progressive renal failure, hearing loss ** and ocular abnormalities **
Associated with corneal opacities, anterior lenticonus **
Genetic mutation of Alport’s
COL4A5 (X-linked) or COL4A3 and COL4A4 (AR)
Results in absence of collagen IV a3a4a5 network
Clinical features of TBMN
Persistent microhaematuria (most common cause of persistent haematuria)
NO proteinuria **
Normal renal function and BP
AD inheritance of haematuria **
Histopathology features of TBMN
Uniform thinning of GBM
Genetic mutation in TBMN
HETEROzygous COL4A3 or COL4A4 mutations
Histological features of type 1 MGPN
Global and diffuse thickening of capillary walls
Caused by mesangial interposition into subendothelial zone of capillary loops - “train track formation”
Types of MGPN
Type 1 - immune mediated
Type 2 - complement mediated, dense deposit disease
Indications for renal biopsy in patients with suspected diabetic nephropathy
Rapid onset of proteinuria
Absence of retinopathy
Presence of haematuria
Active urinary sedment
Rapid decrease of renal function
Suspicion of other nephropathies secondary to systemic disease
Clinical features of Goodpasture’s syndrome/anti-GBM
Pulmonary haemorrhage and rapidly progressive GN with renal failure
Histopathology features of anti-GBM
Crescent formation, uniform age
IM - strong linear ribbon-like appearance of IgG
Possible pathogenesis of hypercoagulable state in nephrotic syndrome
Decreased levels of antithrombin and plasminogen (due to urinary losses), increased platelet activation, hyperfibrinogenaemia, inhibition of plasminogen activation and presence of high molecular weight fibrinogen in circulation
Causes of nephrotic syndrome
Idiopathic membranous nephropathy
Membranoproliferative GN
Minimal change disease
Most common causes of secondary MCD
Medications (NSAIDs, selective COX-2 inhibitors)
Malignancy
Infection
Allergy
Features of collapsing glomerulopathy
Segmental and global collapse of glomerular capillaries
Marked hypertrophy
Hyperplasia of podocytes
Most commonly seen in association with HIV
Also associated with TB, CMV, hepatitis B and hepatitis C
Features of Chinese herbal nephropathy
Mild low-molecular-weight proteinuria
Hypertension
Severe anaemia
Development of uroepithelial atypia
Histopathological features of CHN
Unusual extensive, virtually hypocellular cortical interstitial fibrosis
Associated with tubular atrophy and global sclerosis of glomeruli
Histopathology findings on MCD
Minimal changes
Effacement of podocyte foot processes
Management of MCD
Prednisone - very responsive
Histology features of diabetic nephropathy
Thickening of GBM
Eosinophilic nodular glomerulosclerosis (Kimmelsteil-Wilson nodules)
Mesangial matrix expansion
Histopathology scoring system for IgA nephropathy
MEST
Mesangial hypercellularity
Endocapillary hypercellularity
Segmental glomerulosclerosis
Tubullar atrophy/interstitial fibrosis
Causes of FSGS
Can be idiopathic
Heroin
HIV infection
Sickle cell disease
Obesity
Interferon treatment
Congenital malformations i.e. CMT
NPHS1 and NPHS2 mutations
