Glomerular Disorders

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Description

• Glomerular diseases presentation;
• Asymptomatic hematuria (glomerular hematuria characterized with dysmorphic RBCs)
• Hematuria can be microscopic or macroscopic (for example PIGN or IGAN)
• Asymptomatic Proteinuria (requires 24 hour protein or UPCR or ACR)
• Nephrotic syndrome
• Nephritic syndromes; acute nephritic syndrome, RPGN, chronic GNC

  • Nephritic syndrome
  • Rapid progressive glomerulonephritis

Nephritic Syndrome

Description

At least 3.5g proteinuria in 24hrs , generalized oedema, low serum albumin, hypercholesterinemia and presence of urine oval fat bodies. Causes include:
• Classified as Primary or secondary or based on histology findings
• Minimal change disease
• Membranous nephropathy
• Focal segmental glomerulosclerosis (FSGS)
• Membranoproliferative glomerulonephritis (MPGN)
• Lupus Nephritis
• Diabetic nephropathy
• Amyloidosis

Signs and Symptoms

• Facial swelling worse in the morning
• Frothy urine
• Oedema
• Usually normal BP
• Urine dipstick >2 + proteinuria, hematuria rare except in FSGS

Investigations

• Kidney biopsy is needed for light microscopy, immunofluorescence (IF) and electron microscopy (EM)
• Early referral to Nephrologist important

Treatment

• Usually normal BP
• Urine dipstick >2 + proteinuria, hematuria rare except in FSGS investigations
• Kidney biopsy is needed for light microscopy, immunofluorescence (IF) and electron microscopy (EM)
• Early referral to Nephrologist important
Treatment
• Treatment should be in line with the KDIGO guidelines for glomerular diseases
• The principal for glomerular disease is to determine if the etiology is primary or secondary in nature.
• Salt restriction
• No need for protein restriction in our setting
• Furosemide 80-120mg/day (aim 0.5-1L/day)
• Proteinuria lowering drugs
• Titrate dose depending on proteinuria
• ACEI/ARBs: -e.g Enalapril/Losartan (refer to hypertension for dosages)
• Lipid-lowering /Anti-cholesterol drugs: A statin or statin/ezetimibe for example Simvastatin 10-40mg daily or atorvastatin 10-20mg daily
• Anti-coagulation (INR 2-3)
• Only if albumin < 20g/l, bedridden, very rapid diuresis, otherwise do not use routinely –Warfarin daily

Rapid Progressive Glomerulonephritis

Description

Rapid Progressive GN (crescentic GN) is renal failure occurring over days/weeks, with proteinuria: usually
<3g/day, hematuria: red cell casts, Blood pressure often normal May have other features of vasculitis. Renal failure progress to CKD if no immediate intervention.
RPGN occurs with severe glomerular injury leading to glomerular capillary rupture and accumulation of
plasma and inflammatory cells into the Bowmans capsule leading to formation of the crescent. It can be
classified as:
• immune complex mediated; lupus Nephritis, HBV
• anti GBM RPG
• Pauci immune RPGN; occurring in ANCA associated vasculitis. ANCA vasculitis is a multi-system
disease consists of anti-neutrophil antibodies targeting neutrophil antigens; Proteinase 3 (PR3) and
myeloperoxidase (MPO). ANCA affects capillaries, venules, arterioles and small arteries. Common cause
of RPGN can be classified as:
o Microscopic polyangiitis (MPO
o Granulomatosis with polyangiitis (GPA)
o Eosinophilic Granulomatosis with Polyangiitis (EGPA)

Treatment

• Anti-GBM RPGN
• Prednisolone 60 mg/day and reducing
• Cyclophosphamide 2mg/kg/day and adjusted for white cell count
• Plasma exchange (50ml/kg to a maximum of 4L daily for 14 days or until antiGBM antibodies are
undetectable).Treat for 6 months
• Immune complex RPGN
• Except for Streptococcal, treat as in 3 except plasma exchange may not be indicated
• ANCA positive RPGN
• Methylprednisolone 7mg/kg for 3days and then prednisolone 1mg/kg/day for weeks then taper with either
• Cyclophosphamide 0.5g/m2 IV monthly for 6 months OR
• Cyclophosphamide 2mg/kg PO for 6-12 months
• Plasma exchange for patients with lung haemorrhage and renal dysfunction
• Co-trimoxazole prophylaxis.

Figure 15:Rapid Progressive Glomerulonephritis

Refer to the guidelines (link)