Minimal change nephropathy (MCD)

Characteristics of minimal change nephropathy

Accounts for 90% of cases of nephrotic syndrome in children and about 20% of
cases in adults
Good response to prednisolone (1 mg/kg)
Does not progress to CKD
Microscopic findings in MCD
Light microscopy: normal
Electron microscopy shows fusion of podocyte foot process
No immune complexes or anti-GBM antibody by immunofluorescence
Associations of MCD
Atopy is present in 30% of cases
HLA-DR7
Hodgkins lymphoma in adult
Clinical features of minimal change nephropathy
Nephrotic syndrome with selective proteinuria
Normal renal function, normal blood pressure
Normal complement levels
Increased risk of infections, especially urinary tract infections and pneumococcal
peritonitis
Management of MCD
Prednisolone 60 mg/m2 daily for 4-6 weeks followed by 40 mg/m2 every other
day for further 4-6 weeks (alternate day maintenance)
Adults usually need 12 weeks daily & then 12 weeks alternate day prednisolone
therapy
Other treatments: vaccination, antibiotics, anticoagulation
Drugs used for frequent relapse or steroid resistant or steroid dependent cases
Cyclophosphamide
Ciclosporin
Levamisole
Children presented with oedema, ascites and nephrotic range proteinuria is
commonly due to Minimal change disease (MCD) and biopsy is not indicated.
Failure to respond to prednisolone is an indication of renal biopsy to exclude
other less common causes e.g. FSGS

NEW TOPIC
Focal segmental glomerulosclerosis (FSGS)
A common cause of nephrotic syndrome in older children and younger adults
It may be associated with haematuria, hypertension and impaired renal function
About 50% of patients may respond to a course of high-dose prednisolone
Some patients may respond to the addition of cyclophosphamide; ciclosporin can
be used to reduce proteinuria
Progresses to end-stage kidney disease over several years in up to 50% of
patients
A variant known as 'collapsing glomerulopathy' is associated with HIV infection
Usually recurs in transplanted kidneys

Causes of FSGS
Idiopathic
HIV
Heroin misuse
Morbid obesity
Previous glomerular injury
Sickle-cell disease
Immunofluorescence may show C3 and IgM deposition. Podocyte foot process
fusion may also be seen in FSGS
Drugs used to treat FSGS
High dose prednisolone (0.5-2 mg/kg/day) is the intial treatment but most
patients show little or no response
The efficacy of ciclosporin, cyclophosphamide or mycophenolate mofetil is
uncertain
FSGS is the most common cause of nephrotic syndrome in HIV and usually
progress to CKD. FSGS frequently recurs after renal transplantation

Rapidly progressive glomerulonephritis (RPGN) or crescentic nephritis

Is characterized by rapid loss of renal function over days to weeks
Renal biopsy shows cresent formation, often associated with necrotizing lesions (necrotizing
glomerulonephritis)
Without treatment, the disease progresses to end-stage kidney disease within a few months
Prednisolone and cyclophosphamide are generally effective in patients before severe renal
damage occurs
Plasma exchange is recommended in patients with advanced renal disease and in
Goodpastures syndrome
Causes of rapidly progressive (crescentic) glomerulonephritis (RPGN)
Goodpastures disease (anti-GBM disease, linear IgG deposits along the GBM)
Small vessel vasculitis (particularly ANCA-positive)
Systemic lupus erythematosus (SLE)
IgA nephropathy (occasionally)
Summary box
IgA nephropathy is the commonest cause of nephritic syndrome worldwide.
Haematuria after minor respiratory infections are characteristic. Typically latency
from infection to haematuria is only few days in IgA nephropathy (where it is >14
days in PSGN)
IgA nephropathy may occurs in cirrhosis
PSGN is self-limiting and associated with hypocomplementemia
HSP is characterized by rash, arthritis and abdominal pain
Mesangial IgA deposition is seen in both IgA nephropathy and in HSP
MCGN type I is associated with viral hepatitis, cryoglobulinemia and low C4 level.
Subendothelial immunecomplex deposition causes tram-track appearance
MCGN type II is associated with low C3 level and partial lipodystrophy (C3
nephritic factor). It is also known as dense deposit disease due to
intramembranous complement deposition
Goodpastures syndrome and ANCA-positive small vessel vasculitis e.g.
microscopic polyangiitis are associated with crescentic nephritis
Crescenting nephritis (left image). Crescentic nephritis on immunofluorescence
microscopy