Question | Answer |
Nephrotic Syndrome | Non-inflammatory
Damage to the basement membrane
Increased permeability
Proteinuria >3.5 g/d |
Increased premeability leads to... | ↓ albumin
↓ oncotic pressure
↓ effective circulating volume
Loss of Antithrombin III |
Nephritic Syndrome | Inflammatory
Immune-mediated damage
Infiltrates of neutrophils and phagocytes
-Crescentic lesions
-Rupture of GBM |
Clinical and lab findings with nephritic syndrome | Hematuria
Oliguria
HTN
RBC casts
Proteinuria <3.5g/d |
Glomerular diseases:
Symptoms | Edema
Weight gain
Malaise
Oliguria
Hematuria
Worsening HTN |
Glomerular diseases:
Past Medical Hx | HTN
DM
Hep B/C
HIV
Hyperlipidemia
DVT/PE |
Glomerular diseases:
Family Hx | Kidney disease
DM
Autoimmune Dz |
Glomerular diseases:
Physical exam | Edema, JVD, crackles
HTN
Conjunctival pallor
Signs of autoimmune dz
Xanthomas |
General labs/workup | Rule out pre/post-renal
Urine protein/Cr ratio
UA specifically for casts
Cholesterol panel
Albumin |
What tests are used to help rule out pre/post-renal ARF? | Bladder scan
UA
Specific gravity
FeNa
Uosm |
Nephrotic syndrome findings | Proteinuria >3.5
Serum albumin <3.5
Cholesterol >300 mg/dL
Oval fat bodies on UA |
Nephrotic syndrome findings
(cont.) | ↓Ca due to ↓albumin
Anemia due to loss of transferrin and erythropoietin
Hypercoaguability due to loss of Antithrombin III |
Nephrotic Syndrome Tx | Statins
ACEI/ARBs
Fluid and salt retention |
Nephrotic Syndrome
**BUZZWORDS** | Proteinuria
Hypoabluminemia
Edema
Hyperlipidemia |
Nephrotic syndromes | Minimal change disease
Focal segmental GS
Membranous GN
Diabetic GS
Amyloid |
Minimal Change Disease | Most common type in kids
90% of nephrotic cases <10
Sudden onset
Usually idiopathic |
Minimal Change Disease:
Association and Damage | A:
Medications
Lymphomas
D:
effacement of podocytes |
Why do you get the nephrotic proteinuria with MCD? | Uniform thinning of GBM
Subepithelial accumulation of immune complexes
Disrupted filtration slits |
Minimal Change Disease
Clinical and Dx | Edema
Proteinuria (up to 9g/d)
Dx: biopsy shows effacement of podocytes |
Minimal Change Disease
Tx and prognosis | Tx:
High dose steroids for 8 weeks
Prognosis:
Very good, few progress to ESRD |
Focal Segmental Glomerulosclerosis
(FSGS) | #1 cause of nephrotic syndrome in African Americans
~30% of adult nephrotic |
FSGS
Mechanism | HTN causes hyperfiltration
Accumulation of ECM
Leads to scarring and tamponade
Epithelial cells damaged |
FSGS
Associations | ***HIV***
IV heroin
Urethral reflux
Massive obstruction
Chronic pyelonephritis
Interstitial kidney dz |
FSGS
Clinical | Usually asymptomatic
Microscopic hematuria initially |
FSGS
Tx and prognosis | Tx
High dose steroids
Treat underlying cause
Prognosis
50% → ESRD in 5 yrs |
Membranous Glomerulonephropathy | From antibody/antigen deposition
Up to 1/3 adult nephrotic
2:1 M:F
Typical age 30-50 |
Membranous Glomerulonephropathy
Mechanism | Damage to mesangial matrix
Immune cells can't cross GBM so it's non-inflammatory |
Membranous Glomerulonephropathy
Causes | Usually idiopathic
**Malignancies**
-Breast, colon, stomach, kidney, lung
Hepatitis B/C
Syphilis, DM, SLE |
Membranous Glomerulonephropathy
Workup and Tx | Exclude SLE, RA
Hepatitis serologies
**age appropriate cancer screenings**
Tx:
Steroids and cytotoxic agents |
Diabetic Glomerulosclerosis
Developes within... | 5-10yrs in DM II
10-20yrs in DM I
~50% diabetics |
Diabetic Glomerulosclerosis
Increased prevalence in... | Smokers
Non-whites
Pts with poor glycemic and BP control |
Diabetic Glomerulosclerosis
Dx | Urine microslbumin test
-microalb/Cr >30
Nodular sclerosis on biopsy
**Kimmelstiel-Wilson nodules** |
Diabetic Glomerulosclerosis
Tx | Glycemic control
BP control with ACEI/ARBs
Intensive cholesterol control
(LDL<100) |
Nephritic disease | Inflammatory responce
Immune mediated damage
Crescentric lesions
Plug up Bowman's Capsule
Necrosis/thinning of GBM
Rupture of GBM |
Nephritic disease
Clinical | ***********
*Hematuria*
*Oliguria *
* HTN *
*********** |
Nephritic disease
Labs | UA:
Hematuria
RBC casts
Protein <3.5g/d |
Nephritic Diseases | IgA Nephropathy
Henoch Schonlein Purpura
Post-infectious GN
Membranoproliferative GN
Anti-GBM/Goodpasture's Syndrome |
What immunoglobulin plays a critical role in mucosal immunity? | IgA |
IgA Nephropathy | #1 cause of GN worldwide
2-3 days post infection |
Causes of IgA nephropathy | URT and GI infxn
Causes defective mucosal immunity
Overproduction and increased serum IgA |
How does a pt with IgA nephropathy present? | 1. Macroscopic or gross hematuria
2. Microscopic hematuria
3. High BP or kidney failure |
IgA nephropathy
Clinical findings, Dx and Tx | Macroscopic hematuria
40% have asymptomatic microscopic hematuria
Normal complement (C3&4)
Tx
ACEI/ARBs, steroids |
Henoch-Schonlein Purpura | Disease of kids
Follows URI
IgA in:
-skin, GI tract, kidney, joints |
Henoch-Schonlein Purpura
Clinical findings and Tx | Lower extremity bruising
Bloody diarrhea
Hematuria/HTN
Arthalgias
Tx: supportive |
Post-Infectious GN | Subendothelial antigen deposition
2-3 weeks post infxn
Low C3 and C4 |
How is streptococcal pharyngitis diagnosed? | Rapid strep test
ASO titer |
Post-Infectious GN
Dx and Tx | Dx:
Hx, ASO titer, anti-DNAase B
Tx:
none, self-resolving |
Membranoproliferative GN | Idiopathic cases uncommon
Usually secondary to other dz |
Membranoproliferative GN
Associated with | Sjogren's Syndrome
SLE
Hep B/C WITH cryoglobulinemia
Infective endocarditis |
Membranoproliferative GN
Labs and Tx | UA with dysmorphic RBCs, RBC casts, low C3 and normal C4
Treat underlying cause, steroids, antiplatelets |
Anti-GB/Goodpasture's Syndrome | 10-20% of cases of rapidly progressive GN
Can be ANCA+
Antibodies to type IV collagen |
Anti-GB/Goodpasture's Syndrome
Clinical | Rapid onset of HTN
Oliguria
**Fatal if not treated promptly** |
Anti-GB/Goodpasture's Syndrome
Kidney and lung involvement | Kidney only
More common in older women (Anti-GBM)
Kidney/lung
More common in young men (Goodpasture's syndrome) |
Goodpasture's Syndrome | 70% will have alveolar hemorrhage
(hemoptosis) |
Anti-GB/Goodpasture's Syndrome
Dx and Tx | Dx
Anti-GBM antibodies, biopsy
Tx
Cyclophosphamide+steroids
Plasmapheresis if pulmonary hemorrhage |
Anti-GB/Goodpasture's Syndrome
Prognosis depends on... | Promptness of Dx and Tx
Degree of kidney involvement |
Small Vessel Vasculitis | Wegener's Granulomatosis
-Lung, kidney, sinus
Microscopic Polyangiitis |
Small Vessel Vasculitis
Clinical | Decreased appetite
Weight loss
Hematuria/proteinuria
Polymyalgia |
Small Vessel Vasculitis
Dx and Tx | c-ANCA+
anti-PR3- Wegener's
anti-MPO- Microscopic
Cyclophosphamide+steroids
Plasmapheresis for Wegener's |
Nephrotic/Nephritic
Lupus Nephritis | WHO classifications
III focal nephritis
IV diffuse nephritis
V Membrane formation
VI >90% of glomeruli sclerotic |
Nephrotic/Nephritic
Lupus Nephritis
Clinical and Dx | Acute onset of hematuria
Proteinuria
Edema
Pancytopenia
Dx: anti-Sm, anti-Ds DNA |
Nephrotic/Nephritic
Lupus Nephritis
Tx and prognosis | >Class III
Cyclophosphamide+/-steroids
Prognosis depends on
Kidney fxn
Histologic type
Crescents |