Nephritic syndrome Flashcards
What is RPGN
Severe glomerulonephritis fulminant course with rapid detoriaration in renal function over days to weeks to months - type of nephritic syndrome
What is nephritic syndrome
Haematruia, proteinuria, renal impairment, HPTN
Cuases of nephritic syndrome
Immune complex deposition (AI, post strep, IgA)
AntiGBM deposition
Small vessel vasculitis (polyangitis)
Causes of immune nephritic syndrome
SLE
Post strep glomerulonephritis
IgA nephropathy
Cryoglobuniaemia
Antibodies ass with small vessel vasculitis
ANCA
anti-GBM -
ANCA realted vasculitis can cause
Microscopic polyangitis - MPA
Grnaulomatosis with polyangitis
Eosinophilic granulomatosis w polyangtitis
Anti GBm targets
GBM antibodies target type I collagen within basement membrane
Linear depositon of IgG in glomerular capillaries
Symptoms of nephritic syndrome
Lethargy
Recent infection - fever, sore throat, coryzal symptoms - post strep glom and IgA nephropathy
Haematuria
Oliguria
Oedema - peripheral, periorbital
SOB - fluid overload
Haemoptysis - due to pulmonary haemorrhage eg anti-GBM ANCA-vasculitis
Signs of nephritic syndrome
Haematuria
HPTN
Oedema - peripheral, periorbital
Fluid overload - raised JVP, bibasal crackles on auscultation, peripheral oedema, ascites
Reduced urine output
Extra renal manifestations
Skin - vasculitis rash - palpable purpura
ENT - rhinosinusitis, nasal discharge, polychondritis (cartliage inflammation)
Eyes - red, painful eyes (eg conjunctivitis, scleritis, uveitis)
Lungs - haemoptysis, pleuritic pain, wheeze
Heart - chest pain - pericardial or MI involvement
Nervous system - mononeuropathies
How is nephritic syndrome diagnosed
Haematuria, proteinuria, deteriorating renal function
Glomerular origin of haematuria evidence
Dysmorphic RNC
Red blood cell casts
What is a cast
Cluster of particles wrapped in uromodulin - secreted by epothelial cells lining loop of Henle, distal tube and collecting duct
Manaegment of nephritic syndrome
General supportive measures for AKI
Temporary RRT
Immunosupressive therapy
Complications nephritic syndrome
Can cause severe AKI and ass complications
Can lead to widespread organ dysfunction
What does post infective glomerulonephritis occur after
Group A beta haemolytic streptococcus
What population is PSGN most common in
Children
post exposure length after infection for PSGN
1-3 weeks
Diagnosis
Haematuria, proteinuria, decline renal function + recent strep infetion
Anti-streptolysin titre - ASOT - evidence for recent infection
Renal biopsy in adults
RSPN management
Managing complications
Resolution normally rpaid and outcome excellent in children
Adults more varibale
Active infection - antibiotic
Renal biopsy findings of post infective glomerulone[hritis
Diffuse proliferative and exudative glomerular hisotry
Dominant C3 staining and subepithelial humps
SLE nephropathy what causes
Anti double stranded DNA deposit in glomeruli, other immune complexes, area of deposition can include mesangium, enothelium or epithelium
Classiications of lupus nephritis
Class I - minimal mesangial
Class II - mesangial proliferative
Class III - focal
Class IV - diffuse
Class V - membranous
Class VI - advance scleorisis
Most common and severe form of SLE nephritis
Class IV diffuse - nephrotic or nephritis syndrome
How do class I, II and III of SLE nephritis present
I - asymptomatic
II - non visible haem and proteinuria
III - variabel, may have haematuria, proteinuria, renal impairment and/or nephrotic syndrome
Class V SLE nephritis vs Class VI presentation
V - nephrotic syndrome similar to membranous nephropathy
VI - advance sclerosis - global sclerosis and slowly worsening renal function
Management SLE nephritis
Class IV and above
High dose systemic immunosuppression require RRT
What causes IgA nephropathy
IgA Deposition of IgA nephropathy in the glomeruli
Treatment of IgA nephropathy
High dose prednisolone
Immunosupressive drugs Good BP control, largely supportive
How does IgA nephropathy present
Nephritic syndrome 24-48 hours after URTI
Investigations for IgA nephropathy
Increased mesangial cells
Increased matrix
IgA deposition in matric
Prognosis of IgA nephropathy
20-30% -> renal failure
Pathology of anti GBM disease
antiGBM attacks basement membrane in lungs and kidney -> glomerulo/pulm haemorrhage
Treatment antiGBM
Potent immunosupression
Plasmapheresis
What is membranoproliferative GN?
Glomerular basement membrane thickening
Proliferative changes on light microscopy
What is membranoproliferative GN ass with
Hepatitis C
AI eg SLE
Appearance of membranoproliferative disease on biospy
Thickened basement membrane
Thickened mesangium
Tram tracking appearance
Subendothelial deposition of IgG - on immunoflurescence
Managmenet of membranoproliferative GN
Dipyridamole + aspirin
Kidney transplantation - end stage renal disease
Managmenet of membranoproliferative GN in children with nephrotic range proteinuria
Corticosteroids
What is RPGN
Acute nephritic syndrome with microscopic glomerular crescent formation
What antibody do patients usually have in small vessel vasculitis
ANCA
What is GPA
Wegners - extremely rare long term systemic disorder - formation of granulomas + inflammation of small and medium blood vessles
URTI, lungs, kidneys
What is GPa caused by AB?
c-ANCA
Biopsy of GPA shows
Leukocytoclastic vasculitis with necrotic changes and granulomatous inflammation
Management of GPA
Immunosupression - remission with rituximab or cyclophosphamide in combination with high dose corticosteroids
What is microscopic polyangtitis
Systemic small vessel vasculitis
Clinical features of microscopic polyangitis
weight loss, fevers, fatigue, renal failure
Managemnet of microscopic polyangitis
Long term prednisolone
Cyclophosphamide therapy - cycled
Plasmapheresis
What is goodpasteurs syndrome
When lung involvement with anti-GBM (haemoptysis)
IgG
Management of anti-GBM disease
IV prednisolone
Cyclophophamide
Plasmapheresis
