- presents with haematuria or proteinuria (protein >3.5g/day + hypoalbuminaemia + oedema = nephrotic syndrome)
- IgA Nephropathy
- most common GN, deposition of IgA in glomerulus
- classically haematuria starting with in 1-2 days of URTI
- associated with HSP
- Post strep glomerulonephritis
- classically 1-2 weeks post strep infection
- haematuria, oliguria, odema, HTN
- Minimal Change Disease
- most cases in children, 20% adults with nephrotic syndrome
- assoc atopy in children and possible underlying Hodgkins in adults
- does not progress to ESRF
- Focal Segmental Glomerulonephritis
- haematuria
- CRF with in 10 years, delayed with steroid treatment
- Membranous nephropathy
- nephrotic syndrome in older adults
- idiopthic, SLE, hep B, malignancy, gold, penicillamine
- Mesangiocapillary Glomerulonephritis
- -nephrotic or nephritic syndrome in children and young adults
- Crescentic Glomerulonephritis
- ANCA-positive vasculitis of kidney (idiopathic form)
- progresses to ESRF with in weeks to months with out treatment
- Goodpasture’s Syndrome
- autoantibodies against alpha3 chain of type IV collagen (structural component of glomerular and alveolar basement membrane)
- may also present with haemoptysis from pulmonary haemorrhage
- rapidly progressive renal failure
- prednisolone, cyclophosphamide, plasma exchange to remove antibodies generally effective if started before renal disease is advanced
- relapse rare
- Systemic Diseases
- Systemic Lupus Erythematous
- Primary Systemic Vasculitis
- necrotising inflammation of blood vessels
- Wegener’s Granulomatosis
- Microscopic Polyangiitis
- Churg-Strauss Syndrome
- Haemolytic Uraemic Syndrome
- thrombotic microangiopathy, widespread interglomerular thrombi
- overlap with thrombotic thrombocytopaenic purpure
- in children commonly related to GI E coli infection
- Systemic sclerosis
- obstructive vasculopathy “scleroderma kidney”
- usually have systemic features of scleroderma
- ACE inhibitors improve renal outcomes
- Sarcoidosis
- granulomatous inflammation of numerous organs including lung and kidney
- granulomatous tubulointerstitial nephritis
- Cryoglobulinemia
- immunoglobuline precipitate in cold
- purpuric rash, arthraligia, peripheral neuropathy
- many cases assoc with Hep C
- Myeloma and Monoclonal Dysproteinaemias
- light chain cast nephropathy, light chain depositions
- plasma exchangemay be helpful
- Amyloidosis
- insoluble protein deposits in kidney (amyloid AL and AA)leading to nephrotic syndrome
- commonly liver, spleen heart and nervous system involvement depending on type of amyloid
- Investigations
- -Urine dipstick
- -BP
- -ECG
- -Urine M/C/S, spot protein, 24 hour urine protein
- -FBE, UEC, blood glucose, coagulation screen, LFT, CRP, ESR, CMP
- -Imaging, USS and CT (beware contrast)
- -ANA, ANCA, anti GBM, complement levels (C3, C4), cryoglobulins
- -throat swab, antistreptolysin
- -serum immunoglobulin titres, paraprotein electrophoresis, uriniary Bence Jones protein
- -CK
- -Hep B/C, HIV (if risk factors or dialysis rpeparation)
- Management
- -treat underlying cause
- -most will require hospitalisation and nephrology input
- -most GN treated with prednisolone +/- cyclophosphamide
Nephrotic syndrome
- Clinical
- High protein – 3/4+ on dipstick, Urine PCR > 200
- Frothy appearance urine
- Hypoalbuminemia < 25g/L
- Significant oedema
- Swelling around ankles and eyes
- Associated diseases
- In children most commonly idiopathic
- Minimal change disease
- Focal segmental glomerulosclerosis
- Membranous glomerulonephritis
- SLE
- Henoch Schoenlein Purpura
- Assessment
- Intravascular volume depletion
- Dizziness
- Hypoperfusion
- Tachycardia
- reduced urine output
- hypotension
- Severe oedema
- Discomfort
- Genital
- skin breakdown
- pleural effusion
- ascites
- Infection – increased risk – cellulitis, SBP
- Thrombosis – increased risk
- Systemic symptoms – fever, rash, joint pains
- Treatment
- Usually need admission/specialty care
- Prednisolone
- Albumin and frusemide as needed
- Follow up – daily checking urine protein dipstick – prompt treatment of relapses
Nephritic syndrome
- Clinical
- Haematuria ++
- Protein +
- Hypertension
- Low urine volume <300mL/day
- Mild oedema – often periorbitial
- Causes
- Post streptococcal glomerulonephritis – most common cause for children
- IgA nephropathy – after URTI
- Rapidly progressive glomerulonephritis
- Henoch Schonlein purpura
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