Renal - Glomerulonephritis: Nephritic syndrome and Asymptomatic Haematuria Flashcards
What are 3 presentations of glomerulonephritis?
- Asymptomatic haematuria
- Nephrotic syndrome
- Nephritic syndrome
Glomerulonephritis Ix: bloods
- Basic: FBC, U+E, ESR
- Complement: C3 and C4
- Antibodies: ANA, dsDNA, ANCA, GBM
- Serum protein electrophoresis and Ig
Glomerulonephritis Ix: urine and imaging
Urine
- Dipstick: look for proteinuria and haematuria
- MC+S
- Bence-Jones protein
Imaging:
- CXR: look for infiltrates (Goodpasture’s and Wegener’s)
- Renal Us +/- biopsy
Glomerulonephritis: general Mx
- refer to nephrologist
- Rx HTN aggressively (=<130/80)
- include ACEi/ARB
What conditions typically present with nephritic syndrome (haematuria, hypertension)?
- Rapidly progressive glomerulonephritis (AKA crescentic Glomerulonephritis) - causes include:
- Goodpasture’s, ANCA positive vasculitis - IgA nephropathy (typically young adult with haematuria following URTI)
- also known as Berger’s disease and mesangioproliferative GN - Alport syndrome: abnormal GMB (defect in type IV collagen)
- Nephritic syndrome: what is rapidly progressive Glomerulonephritis? What are underlying causes
- Syndrome of kidney characterised by rapid loss of renal function with glomerular crescent formation (seen in most pts) of glomeruli on kidney biopsies
- If untreated can rapidly progress to renal failure
Underlying disease:
- Goodpasture syndrome
- SLE
- pAnca vasculitis: Churg-Strauss, microscopic polyangiitis
- cAnca vasculitis: Wegeners/Granulomatosis with polyangiitis)
- idiopathic
- Nephritic syndrome: what is Goodpasture’s syndrome? Features, cause, risk factors
Rare condition:
Features: pulmonary haemorrhage and rapidly progressive glomerulonephritis
-Caused by anti-GBM antibodies against type IV collagen
-Risk factors: smoking, LRTI, pulmonary oedema, being male
- Nephritic syndrome: What is Goodpasture’s syndrome? Investigations and Management
Investigations
- Renal biopsy: linear IgG deposits along BM
- Raised transfer factor secondary to pulmonary haemorrhages
Management
- Plasmapheresis
- steroids
- cyclophosphamide
- Nephritic syndrome: SLE- renal complications: monitoring, class, management
- Lupus nephritis: severe manifestation of SLE - can result in end stage renal disease
- Monitoring: regular urine dips to rule out proteinuria
- Class IV is most severe form - endothelial/mesangial proliferation, thickening of capillary wall, subendothelial immune complex deposits
- Management: treat HTN, corticosteroids if evidence of clinical disease, immunosuppressants (Azathioprine/cyclophosphamide)
- Nephritic syndrome: What are the features of IgA nephropathy?
- Also called Berger’s disease or mesangioproliferative glomerulonephritis (commonest GN in developed world) - accumulation over time of IgA in glomerulus, causing local inflammationa and destruction
- Significant pathological overlap with Henoch-Schonlein purpura (HSP)
- Nephritic syndrome: What are differentiating factors between IgA nephropathy and Post-streptococcal Glomerulonephritis?
IgA neuropathy is part of nephritic syndromes
- macroscopic haematuria
- develops 1-2 days after URTI
- affects young males
Post-strep GN presents as more of a mixed nephrotic/nephritic picture
- main symptom is proteinuria but haematuria can occur
- Post-strep typically occurs 7-14 days following group A beta-haemolytic strep infection (usually strep progenies)
- low complement C3 levels
- pt is generally unwell (headache/malaise)
- Nephritic syndrome: What is Henoch-Schonlein purpura?
-HSP is IgA mediated small vessel vasculitis - usually some degree of overlap with IgA nephropathy (Burger’s disease)
Usually seen in kids
- Palpable purpuric rash over buttocks, extensor surfaces arms/legs
- Abdo pain
- Polyarthritis
- features of IgA neprophathy may occur: haematuria, renal failure
- Nephritic syndrome: What is the treatment and prognosis for Henoch-Schlonein Purpura?
Rx
- Analgesia
- support rx for nephropathy
Prognosis
- usually excellent - self limiting condition esp in kids without renal involvement
- 1/3 pts have a relapse
- Nephritic syndrome: What is Alport syndrome and outline some features
-genetic disorder characterised by Glomerulonephritis, end stage kidney disease and deafness due to a defect in type IV collagen
Features
- haematuria and (as disease progresses), proteinuria
- sensorineural deafness
- Lens dislocation and cataracts
- retinal ‘flecks’
*Can get presence of anti-GBM antibodies (leading to Goodpasture’s syndrome line picture) in pts with a failing renal transplant
Asymptomatic haematuria: what are the features of thin BM disease
- Autosomal dominant - commonest cause of Asymptomatic haematuria (but small risk of ESRF) - only finding is thin BM in glomerulus
- Features: Persistent, asymptomatic microscopic haematuria