Clinical Management Of Nephrotic And nephritic Syndromes Flashcards
Nephritic vs nephrotic syndrome
Nephritic
- acute or chronic
- reversible
- inflammatory process with active urinary sediment
- 3 primary symptoms:
1) proteinuria of <2gm/dL
2) WBCs (neutrouria)
3) RBCs (hematuria) - may show RBC casts but must have glomerular disease present also
- can show (+/-) HTN, edema, renal failure
Nephrotic
- acute or chronic
- noninflammatory proces
- 3 primary symptoms:
1) proteinuria of >3,5 bpm/dL
2) mass edema
3) Hypercholesterolemia with hypoalbuminemia - (+/-) show oval fat bodies and HTN
What does the term “active urinary sediment” mean?
Urine contains
- proteinuria of <2gm/dL
- RBCs >10/HPF
- WBCs
Can contain casts also
is seen in nephritic syndrome only
Types of casts and associated pathology
Red cell casts : glomerulonephritis
White cell casts : pyelonephritis, interstitial nephritis
Waxy casts : advanced renal diseases
Hyaline casts : concentrated urine or use of diuretics only (may not be pathology)
Fatty casts = nephrotic syndrome
Granular Casts = renal failure (ATN if muddy brown)
Epithelial cell/renal tubular cell casts = ATN (acute tubular necrosis)
What does the term “bland urinary sediment mean”
Contains
- heavy proteinuria (>3.5)
- urine fat/oval fat bodies
- fatty/waxy cats
- renal tubular cells with lipid droplets
- NO inflammation
seen in nephrotic syndrome
3 broad catagories of glomerulonephritis
1) ANCA is present
2) anti-GBM antibodies are present
3) immune complexes are present
What are possible causes of glomerulonephritis with ANCA bodies present
ANCA-associated crescentic GN:
- if there is no extra-renal symptoms
Microscopic polyangitis:
- if systemic necrotizing symptoms are present
Granulomatosis polyangitis
- if respiratory symptoms with granulomas are present
Eosinophilic granulomatosis w/ polyangitis
- if asthma and eosinophila are present
What are possible causes of glomerulonephritis if anti-GBM antibodies are present?
Goodpasture syndrome
- if lung hemorrhages are present
Anti-GBM specific GN
- if no lung hemorrhage is present
What are possible causes of glomerulonephritis with immune complex markers present?
Lupus GN
- if ANAs are present and lupus systemic symptoms
Postinfectious GN
- if recent strep/staph infection and antipathogenic antibodies are present
IgA nephropathy
- if IgA complexes are present
Cryoglobulinemic GN
- if cryoglobulins are present
MPGN
- if complement is active
Post infectious Glomerulonephritis (PIGN)
A cause of nephritic syndrome w/ low complement and primarily kidney presentation only
Almost ONLY seen in recent staph/strep infections (1-3 weeks after)
- presents with ASO antibodies if strep
Urine shows hematuria and sub nephrotic proteinuria (<3.5)
Imaging shows granular deposition of IgG and C3 in GBM
- EM will show large dense endothelial deposits (humps)
Membranoproliferative glomerulonephritis (MPGN)
Rare pattern of glomerular injury and can be seen across the entire nephritic spectrum
Can be idiopathic or associated with chronic HCV or autoimmune infections/disorders
Type 1: MOST COMMON
- shows low C3/C4 levels in blood and urine
- all over the spectrum (can be asymptomatic hematuria to gross hematuria to extreme proteinuria)
- biopsy shows “tram-track” apperance with hypercellularity
- shows C3 deposition and immunoglobulin staining on IF stains
Type 2: “dense deposit disease
- shows low levels of C3 only in blood and urine
- is an inherited disease
- kidney EM biopsy shows ribbon-like electron dense deposits on the GBM
How do you treat type one MPGN?
Mild disease = ACE-I/ARB
Severe disease = cyclophosphamide or mycophenolate mofetil + steroids
even with treatment, end stage renal disease (ESRD) is very likely
type 2 treatment is novel therapies and experiment processes, no first line
IgA nephropathy (Berger disease)
Cause of nephritic syndrome with normal complement
most common primary glomerular disease worldwide
classic presentation: acute viral illness with gross hematuria/proteinuria with “coca-cola” urine
Commonly seen in females, children and Asians
Serum complement levels are NORMAL
Biopsy shows focal glomulonephritis w/ IgA and C3 depositions and proliferation
How to treat IgA nephropathy
Mild = just monitor
- NO HTN and within normal limits of GFR and minimal proteinuria
Severe: ACE-I/ARB to reduce HTN (125/75) and proteinuria (< 1g/d)
- HTN, reduced GFR and >1g/d proteinuria
- if this doesn’t solve it, add corticosteroids
IgA + RPGN (“crescent nephropathy”)
- treat with corticosteroids and cyclophosphamide
IgA + ESRD = renal transplant
Alport syndrome (hereditary nephritis)
X-linked nephritic syndrome with normal complement levels
Presents with chronic glomerulonephritis w/ hearing and eye issues
caused by defects in (a)-3/4 type 4 collagen subunits
Kidney biopsy shows split lamina densa of the GBM (hallmark finding)
Treatment = ACE-I/ARB
- * if ESRD is present = renal transplant, however need to be careful with risk of developing anit-GBM disease against the normal type 4 collagen seen in the transplant kidney*
ANCA+ w/ renal-limited glomerulonephritis
Cause of nephritic syndrome w/ normal compliment levels
Presents idiopathic crescentic GN and is essentially RPGN w/:
- HTN
- nephritic urine
- ANCA antibodies present
Kidney biopsy shows NO immunoglobulin or compliment deposition
Treatment = cyclophosphamide and high dose corticosteroids
SLE
Is a cause of nephritic syndrome w/ low complement
Has 6 separate classes
- 1 ) normal mesangial
- 2) mesangial
- 3) focal proliferative
- 4) diffuse proliferative
- 5) membranous
- 6) ESRD w/ sclerosis
Treatment = aggressive high dose corticosteroids + cyclophosphamide or mycophenolate mofetil
- can also use rituximab in refractory cases