Glomerulonephritis Flashcards
What is glomerulonephritis?
- Glomerulonephritis: A group of diseases associated with inflammatory injury of the glomeruli
- Sources of Inflammation
- Infiltration of leukocytes
- Deposition of immune proteins
- Disturbed function of the affected tissue
Is glomerulonephritis nephritic or nephrotic?
- Nephritic
- Can be rapidly progressive and include pulmonary renal syndrome
What 4 cell types may be affected in glomerulonephritis?
- Mesangium
- Podocytes
- Capillaries
- Parietal epithelial cells
T/F: Anti-GBM disease leads to a nephrotic disease process
- False, Anti-GBM leads to Glomerulonephritis which is nephritic
What are the two most common causes of glomerulonephritis with a renal etiology?
Most common renal etiology of glomerulonephritis
- IgA nephropathy
- Mesangial immune complexes containing IgA
- Anti-GBM disease
- Antibody to glomerular antigen
Note: there are many other etiologies but these were in red as the most common
What are the two most common systemic etiologies of glomerulonephritis?
Most common systemic etiologies of glomerulonephritis
- Lupus nephritis
- Immune complex deposition
- ANCA asscoiated vasculitis
- “Pauci-Immune”
- Small vessel vasculitis
Note: there are many other etiologies but these were in red as the most common
What are the clinical findings c/w (consistent with) nephritic syndrome
Nephritic Syndrome Clinical Findings
-
Hematuria
- Dysmorphic RBC’s
- RBC casts
- Proteinuria
- 200mg - 10gm
- Elevated Cr
- Elevated BP
What 3 diseases can cause pulmonary renal syndrome?
- Anti-GBM Disease
- ANCA associated vasculitis
- Lupus
pulmonary-renal syndrome requires aggressive therapy like cytotoxic drugs and plasma exchange
What are the criteria for calling a glomerulonephritis “rapidly progressive?”
- Serum Cr doubles or GFR falls by 50% within a few days to 3 months
What 3 diseases are usually associated with Rapidly Progressive Glomerular Nephritis?
- Anti-GBM disease
- ANCA associated Vasculitis
- Lupus
Note: these are the same 3 diseases that are associated with pulmomary renal syndrome
What is pulmomnary renal syndrome?
Pulmomonary renal syndrome: Glomerulonephritis w/ pulmonary capillaritis
- Presents with nephritic syndrome and hemoptysis
- Capillaries of the kidney are involved and capillaries of the lung are invovled
For the following labs name what diseases they test for:
- ANA, Anti-DS DNA
- C3,C4 - if they come back low
- Blood cultures
- ASO
- Hepatitis C
- Hepatitis B
- Anti-neutrophil cytoplasmic antibodies (ANCA)
- Anti-GBM antibodies
- Lupus
- Several diseases including glomerulonephritis, vasculitis, autoimmune, infection
- Infection: Bacterial endocarditis specifically listed
- Streptococcus
- Cryoglobulinemia
- Membranoproliferative glomerulonephritis
- Pauci-immune vasculitis
- Anti-GBM disease, good pasture’s syndrome
Alex’s Made Up Vignette: 56 y/o female presents to the ED coughing up blood snd complaining of dark colored urine. A urine analysis reveals proteinuria and hematuria with RBC casts.
Question:
- What tests should you order
- Why?
- ANA, ANCA, Anti-GBM
- This is pulmonary renal syndrome:
- The 3 main causes are Lupus, ANCA assoc Vasculitis, and Anti-GBM disease
T/F: You do not need a kidney biopsy to definitively diagnose glomerulonephritis.
- False: Kidney biopsy is required for definitive diagnosis of glomerulonephritis
- A kidney biopsy is performed and cresencents are seen, what disease is this c/w?
- What other diseases are usually associated with this disease?
- What kind of treatment does this disease require?
- “Crescentic” glomerulonephritis
- associated with severe disease, caused by rupture of the capillary wall
-
The trifecta as I call them
- Anti-GBM
- ANCA associated vasculitis
- Lupus
- Aggressive therapy
- Cytotoxic drugs
- Plasma exchange (plasmaphoresis)
- What disease is associated with subepithelial immune complex deposition?
- Membrounous disease
What are the 8 main drugs used to treat glomerulonephritis?
- Prednisone
- Cyclophophamide
- Mycophenolate mofetil (MMF)
- Azathioprine
- Chlorambucil
- Rituximab
- Eculizumab
- Intravenous Immunoglobulin (IVIg)
What is the most common cause of glomerulonephritis world wide?
- IgA nephropathy
- Usually presents with hematurea +/- subnephrotic proteinuria
What disease is c/w this IF slide?
- IgA nephropathy
What are treatments availablefor IgA nephropathy?
- Symptom management: Blood pressure, ACE inhibitors)
- Prednisone (new studies show uncertain benefit though)
Lupis nephritis can cause hematuria, nephritic disease, or nephrotic disease depending on where the immune complexes end up getting deposited.
Question: Immune complex depositions in what cell layers cause
- Hematuria
- Nephritic syndrome
- Nephrotic syndrome
- Hematuria - IC deposits in Mesangium
- Nephritic - IC deposits in Subendothelial
- Nephrotic - IC depotis in Subepithelial
What disease is this based only on these IHC (immunohistochemistry) slides
- Anti-GBM
- Notice in the slide on the left, how the capillaries have formed a “crescent” around the glomerulus
- This should at least tell you its severe disease
- Notice on the right slide the linear deposition of antibodies
- This is deposition into the basement membrane structure
- If untreated can lead to ESRD (end stage renal disease) quickly
- Notice in the slide on the left, how the capillaries have formed a “crescent” around the glomerulus
ANCA associated vasculitis is actually a group of diseases.
Question: What 4 vasculitis diseases make up ANCA associated vasculitis?
- Granulomatosis with Polyangiitis (Wegener’s disease)
- Microscropic polyangiitis
- Eosinophilic granulomatosis with polyangiitis
- Renal limited vasculitis
- Is Granulomatosis with polyangiitis associated with C-ANCAor P-ANCA?
- What are the clinical findings?
-
Granulomatosis with polyangiitis:
-
C-ANCA (PR3)
- Clinical findings: Nephritic syndrome often with extra-renal involvement (airways)
-
C-ANCA (PR3)
- Is Microscopic polyangiitis associated with C-ANCA or P-ANCA?
- What are the clinical findings?
- Microscopic polyangiitis
-
P-ANCA (MPO)
- Clinical findings: Nephritic syndrome, often with extra-renal involvement (airways)
-
P-ANCA (MPO)
- Is Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) associated with associated with C-ANCA or P-ANCA?
- What are the clinical findings?
- Eosinophilic granulomatosis with polyangiitis
-
P-ANCA (MPO) > C-ANCA (PR3)
- Clinical findings: Lung and skin vasculitis, asthma, eosinophilia
-
P-ANCA (MPO) > C-ANCA (PR3)
T/F: Renal limited vasculitis is usually associated with C-ANCA (PR3)
- False, its usually associated with P-ANCA (MPO)
What are the treatment guidelines for ANCA associated vasculitis?
- Cytotoxic drugs
- Cyclophosphamide
- Corticosteroids
- B-cell targeted therapies
- Rituximab
- Plasma exchange (plasmaphoresis)
- Removes pathogenic autoantibodies
