27076059
Description
Flashcards by Jenna Paterson, updated more than 1 year ago
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Created by Jenna Paterson
about 4 years ago
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| Question | Answer |
| What are primary cases of glomerular disease? | Primary renal pathology such as IgA nephropathy, minimal change, RPGN |
| What are secondary cases of glomerular disease? | Systemic conditions with injury to glomeruli (vasculitis, amyloidosis, diabetes) |
| The damage of what structures --> GD? | 1) Endothelium - prevents large proteins passing through 2) Basement membrane - contains negative charge - repels negatively charged molecules such as albumin 3) Foot processes of podocytes - intercellular junctions stop proteins passing through |
| What is nephrotic syndrome? | Non-proliferative injury to the structure of the glomerulus resulting in excessive leakage of key plasma molecules such as albumin. - Triad of: ○ Heavy proteinuria >3.5g/day ○ Oedema ○ Hypoalbuminaemia |
| What findings are present in nephrotic syndrome and why? | - Excessive leakage of key plasma molecules such as albumin --> proteinuria and hypoalbuminaemia. - Falls in serum albumin reduces plasma oncotic pressure which causes oedema. - Reduced oncotic pressure also leads to elevated cholesterol. - Loss of proteins including immunoglobulins means greater risk of infection. - Loss of proteins including antithrombotic factors results in risk of thromboembolism. |
| What is nephritic syndrome? | - Usually proliferative changes within the glomerulus resulting in increased cell numbers and inflammatory cell infiltration. - Haematuria - Proteinuria - Oliguria - Hypertension |
| What findings are present in nephritic syndrome and why? | - Proliferative changes within the glomerulus resulting in increased cell numbers and inflammatory cell infiltration --> presence of blood in urine.- Inflammatory infiltrates result in reduced movement across BM (decreased GFR) causing oliguria. - Water and sodium retention --> HTN and degree of oedema. |
| What is a nephritic urinary sediment? | Urine microscopy showing red cell casts, leucocytes, sub-nephrotic range proteinuria and dysmorphic red cells. |
| What conditions are associated with nephrotic syndrome? | Minimal change disease Membranous glomerulopathy Focal segmental glomerulosclerosis |
| What conditions are associated with nephritic syndrome? | - IgA nephropathy - Post-streptococcal GN (PSGN) - Anti-GBM (Goodpasture's syndrome) - Membranoproliferative glomerulonephritis |
| What is RPGN? | Rapidly-Progressive Glomerulonephritis (RPGN) - AKA Crescenteric GN is an acute nephritic syndrome PLUS formation of crescents. Characterised by extensive crescent formation (>50%) and rapidly progressive renal dysfunction to ESRD within weeks-months. |
| Why do crescents form in RPGN? | Crescents result from thrombosis and rupture of glomerular capillaries compressing surrounding glomerular structures (crescent moon shape) |
| What is a poor prognostic factor in RPGN? | Prognosis poor if initial serum creatinine >600umol/L, otherwise 80% patients improve without treatment. |
| What is the most common cause of RPGN? | ANCA-associated vasculitis |
| How does GN present? | - Up to 50% have no symptoms - Proteinuria is the hallmark ± oedema - Isolated haematuria (not specific) - Nephrotic syndrome - Nephritic syndrome - Acute renal failure - Chronic renal failure/ESRD |
| What is the most common cause of nephrotic syndrome in children? | Minimal Change Disease |
| What is the most common cause of nephrotic syndrome in adults? | Focal segmental glomerulosclerosis (FSGS) |
| What are the investigation findings in minimal change disease? | - Immunofluorescence and light microscopy unremarkable - Electron microscopy - fusion of podocyte foot processes |
| What are the investigation findings in FSGS? | - Immunofluorescence and light microscopy unremarkable - Electron microscopy - segmental scarring of certain glomeruli and fusion of podocyte foot processes |
| What are the investigation findings in membranous glomerulopathy? | - Immunofluorescence - diffuse uptake of IgG - Microscopy - thickened glomerular BM with spikes. |
| How is minimal change disease managed? | - Supportive - nutritional support, salt and fluid restriction - Corticosteroids first line - more evidence for use in children - Other immunosuppressants e.g. tacrolimus (1st line), rituximab |
| How is FSGS managed? | - Generally supportive, especially if secondary - Around 50% respond to steroids, role for other agents |
| How is membranous glomerulopathy managed? | Steroids for progressive disease |
| What causes membranous glomerulopathy? | Immune complex deposition resulting in complement activation against GBM proteins. Commonly idiopathic - can be associated with hep B, malaria, lupus. |
| What % of people with membranous glomerulopathy progress to ESRF? | 1/3 have chronic membranous GN, 1/3 enter remission, 1/3 progress to ESRD. |
| Who is typically affected by IgA nephropathy? | Commonest intrinsic renal disease in adults, typically young males |
| How might IgA nephropathy present? | - May present with frank haematuria within 24-48 hours of upper respiratory tract infection (termed synpharyngitic haematuria) - May cause either a nephritic or nephrotic presentation or be clinically silent. |
| What are the investigation findings in IgA nephropathy? | - Microscopy - increased numbers of mesangial cells (proliferation) - IHC - IgA deposition within mesangial cells |
| What are the investigation findings in post-streptococcal GN? | - Diffuse proliferative and exudative glomerular histology - Dominant C3 (complement) staining and subepithelial humps - Raised serum streptococcal titres. |
| What are the investigation findings in anti GBM? | - Serum anti-GBM antibodies - IHC - IgG deposits along BM |
| What are the investigation findings in membranoproliferative GN? | - Microscopy - thickened BM AND thickened mesangium (tram-tracking appearance). - Immunofluorescence - subendothelial deposition of IgG |
| What is PSGN and how might it present? | - Immunologically-mediated glomerular injury, triggered by infection (usually streptococcal) - Typically presents 2 weeks later with nephritic syndrome. |
| How is PSGN managed? | Supportive - self limiting |
| How is IgA nephropathy managed? | - Steroids/immunosuppression for rapidly progressive disease with crescents, or heavy proteinuria. - Evidence base is very poor. |
| How is membranoproliferative glomerulonephritis managed? | - Steroids in children - Antiplatelets in adults |
| How is Anti-GBM managed? | High dose immunosuppression - steroids + cyclophosphamide unless HD dependent at presentation. |
| If someone presents with nephritic syndrome and haemoptysis, what might you suspect? | Anti-GBM (Goodpasture's syndrome) or Granulomatosis with Polyangiitis (GPA) Haemoptysis is indicative of pulmonary haemorrhage |
| A finding of c-ANCA would lead you to what diagnosis? | Granulomatosis with Polyangiitis (GPA) |
| A finding of p-ANCA would lead you to what diagnosis? | Microscopic Polyangiitis (MPA) |
| How is Granulomatosis with Polyangiitis (GPA) treated? | Immunosuppression induction with high dose steroids + rituximab/cyclophosphamide then maintenance therapy (steroids/MTX) |
| How is (GPA) Microscopic Polyangiitis (MPA) treated? | Long term steroids/cyclophosphamide - often cycled (plasmapheresis) |
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