Nephritic Syndrome Flashcards
What is the clinical presentation of nephritic syndrome
- High blood pressure
- mild oedema
- urine dip - blood/protein
What is glomerulonephritis
it is a pathological diagnosis that encompasses a number of conditions that:
- are caused by a pathology in the glomerulus
- present with proteinuria, haematuria or both
- are diagnosed on a renal biopsy
- cause CKD
- can progress to kidney failure (except minimal change disease)
What is rapidly progressive glomerulonephritis (RPGN)
rapid fall in renal function
In glomerulonephritis what is the difference between early and late stages of the disease
Early
- red cell class
- proteinuria
- haematuria
Late
- hypertension
- oedema
- acute renal impairment
What do you see in an urine dip and microscopy in someone with nephritic syndrome
Urine dip = blood and protein - if positive for blood send for a microscopy
- Microscopy = red blood cell casts
- Other - Bence Jones protein, ACR/PCR
what do red cell casts signal
glomerulenephritis - glumerular inflammation
What are other types of casts
Proteinacious material formed in tubule Tamm-Horsfall protein matrix
Other- Hyaline, Granular, White cell casts
What are the 3 common forms of glomerulenephritis
- Autoimmune
- Infections
- malignancies
Describe what in autoimmune can cause glomerulenephritis
- Small vessel vasculitis e.g ANCA associated vasculitis
- SLE
- Anti-GBM disease[Goodpastures]
- IgA nephropathy
- Cryoglobulinaemia
Describe what in infections can cause glomerulenephritis
- Viral- Hepatitis C, Hepatitis B, HIV
- Post-streptococcal
- Subacute bacterial endocarditis
Describe what in malignancies can cause glomerulenephritis
- Lymphoma
- Multiple myeloma
Describe the mechanism of glomerular inflammation
- immunoglobulin mediated
- deposition of antibodies (IgA, anti GBM, immunecomplexes)
- activation of the complement pathway
- production of chemotactic factors
- leading to the release of chemokines
- which leads to recruitment of leukocyes - neutrophils, macrophages, lymphocytes
- further release of chemokines and cytokines
- glomerular inflammation, necrosis and crescent formation
What investigations should you carry out for nephritic syndrome
- Bloods
- Urine
- imaging
- biopsy
What bloods should be used in an investigation for nephritic syndrome
- FBC, U&Es, LFTs, ESR, CRP
- immunoglobulins
- complement (C3 and C4; Low in SLE)
- AutoAbs (anti-nuclear Ab + anti-dsDNA (SLE), anti-GBM, ANCA)
- Culture
- hepatitis serology: HbsAg (HepB antigen), anti-HCV
- cryoglobulins
What is an ANCA ( Anti-Neutrophil Cytoplasmic Antibodies)
This test looks for antineutrophil cytoplasmic antibodies (ANCA) in your blood. Antibodies are proteins that your immune system makes to fight foreign substances like viruses and bacteria. But ANCAs attack healthy cells known as neutrophils
What are the two forms of ANCA
- cANCA - proteinase 3
- pANCA - myeloperoxidase
When can a false positive ANCA occur
- It can occur with infections (endocarditis)
What are the disease associations with ANCA
– Small vessel vasculitis
• Wegeners[cANCA], Microscopic polyangitis [pANCA]
• Churg- Strauss
– Drug Induced vasculitis
What conditions can a low C3+- C4 with renal disease indicate
- SLE
- MesangioCapillary GN [MCGN] associated with hepatitis C
- Cryoglobulinaemia
- Post streptococcal GN
- Infective endocarditis
- Foreign bodies- e.g.- shunt nephritis Cholesterol emboli
What is the difference between nephrotic or nephritic
- Nephrotic - collection of symptoms due to kidney damage including proteinuria, hypoalbuminaemia, oedema and dyslipidaemia
- Nephritis - syndrome comprising signs of nephritis (e.g. kidney inflammation) including proteinuria and haematuria
name the differences in nephrotic and nephritic syndrome in terms of
- blood pressure
- urine
- GFR
Nephrotic
- Blood pressure = stays the same or increases
- Urine = proteinuria
- GFR = stays the same or decreases
Nephritic
- Blood pressure = increases
- Urine = haematouria
- GFR - decreases
What is the pathological process of nephritic syndrome
This arises due to glomerular inflammation this causes
- damage to glomerulus which restricts blood flow resulting in an increase in blood pressure
- damage to filtration mechanisms allowing protein and blood to enter urine
- loss of usual filtration capacity resulting in AKI
What is the differential diagnosis of nephritic syndrome and there symptoms and what you would use to diagnose them
Nephrolithiasis (renal calculus disease)
- severe pain with haematuria
- IV pyelogram or renal ultrasound
Bladder cancer
- painless haematuria
- patients usually older with history of smoking
- cystoscopy and biopsy of lesion
Renal cancer
- Triad of - flank pain, fever, and haematuria
- CT
What is the presentation of nephritic syndrome
- Haematuria (micro or macro) ++ on dip
- Oedema
- Hypertension
- Mild proteinuria <1-1.5g/day (or none)
- AKI in severe cases
What imaging would you use in someone with nephritic syndrome
- CXR - pulmonary haemorrhage
- Renal ultrasound - size and anatomy for biopsy
Why do you need to do a renal biopsy in nephritic syndrome
- Renal biopsy is required for diagnosis
Name the common types of glomerulonephritis
- IgA nephropathy
- SLE
- Anti-glomerular basement membrane disease (good pasture’s)
- post-streptococcal diffuse proliferative GN
- rapidly progressive GN (RPGN)
What is the most common glomerulonephritis
- IgA nephropathy
Name the symptoms of IgA nephropathy
- micro/macroscopic haematuria and nephritic syndrome
Who does IgA nephropathy tend to effect
- Typically young males with rapidly recovering episodic macroscopic haematuria
Describe the pathology of IgA nephropathy
Increase in IgA forms immune complexes and is deposited in mesangial cells leading to mesangial proliferation
What worsens your prognosis in IgA nephropathy
Worse prognosis:
- increase in blood pressure
- proteinuria
- renal failure at presentation
how many patients with SLE have renal damage
- 1/3 of SLE patients have renal damage
- stages I-IV severity and V membranous
What is anti-glomerular basement membrane disease (Goodpastures)
- this is when you have antibodies to collagen IV which is a component of the Glomerular basement membrane
Describe the symptoms you get in anti-glomerular basement membrane disease (Goodpastures)
- pulmonary haemorrhage is common - this is because type IV collagen is predominant in the lungs
- SOB
- Haemoptysis
- haematuria
- nephritic syndrome
- potential AKI a few days after onset of symptoms
How do you diagnose anti-glomerular basement membrane disease (Goodpastures)
- anti-GBM in circulation/kidney
What is the treatment of anti-glomerular basement membrane disease (Goodpastures)
- plasma exchange
- steroids and cytotoxics that may result in a full recovery
How does post-streptococcal diffuse proliferative glomerulonephritis happen
- 1-12 weeks after post painful skin or throat infection
- streptococcal antigen deposits in the glomerular leading to immune complex formation and host reaction
What is the presentation of post-streptococcal diffuse proliferative glomerulonephritis
- haematuria
- oedema
- increase in blood pressure
- oliguria
How do you diagnose post-streptococcal diffuse proliferative glomerulonephritis
- evidence of streptococcal infection
- increase in ASOT
- increase in anti DNAse B
- decrease in c3
What would you not do in post-streptococcal diffuse proliferative glomerulonephritis
- biopsy only on atypical presentation as it is due to risk of inflammatory damage to mesangial and endothelial cells
what is the treatment of post-streptococcal diffuse proliferative glomerulonephritis
- antibiotics to clean nephritogenic bacteria
How does rapidly progressive GN present
- most aggressive GN and progresses to end stage renal failure in days
- increase in creatine and decrease in eGFR secondary to GN
What are the three categories of rapidly progressive GN
- Immune complex RPGN
- paci-immune RPGN
- anti-GBM/goodpastures RPGN
Describe immune complex RPGN - category of rapidly progressive GN
- Idiopathic
- Associated with other primary GN (may transform to become rapidly progressing) – membranoproliferative GN, IgA nephropathy, membranous glomerulopathy
- Associated with secondary GN – post-infection, SLE, cryoglobulinaemia
Describe pauci-immune RPGN - category of rapidly progressive RPGN
- 80-90% are ANCA positive – ANCA-associated systemic vasculitis
- Granulomatosis with Polyangitis (GPA) / Wegner’s – C-ANCA positive
- Microscopic Polyangitis (MPA) – p-ANCA positive
- Churg-Strauss
What is the clinical presentation of rapidly progressive GN
- AKI
- Systemic features - fever, myalgia, decrease weight, Haemoptysis
What is the most common cause of death in ANCA positive patients in rapidly progressive GN
- pulmonary haemorrhage
How do you treat rapidly progressive GN
- corticosteroids and cyclophosphamide
What Is the management of glomerulonephritis
Control BP
- <130/80
- <125/75 if proteinuria is >1g/day
Include an ACEI or ARB as these reduce proteinuria and are renoprotective
Immunosuppression
- High dose IV steroids
Cytotoxic Agents
- 1st line – cyclophosphamide
- 2nd line – rituximab
ANCA Removal
- Possible plasma exchange
What is the difference between post strep diffuse proliferative glomerulonephritis and IgA nephropathy
IgA nephropathy - presents 1-2 days after a URTI
Post streptococcal diffused proliferative glomerulonephritis - presenta 1-12 weeks after a painful or throat infection
