Nephrotic Syndrome Nephritic Syndr
Disease Amyloidosis Diabetic Nephropathy Lupus Nephritis Henoch-Schönlein Purpura Goodpastur
Epidemiology - Mostly in old patients - Most common cause of ESRD - Common SLE complication - Peak age: 3-15 y.o. Syndrome → 2
- 10 years after disease onset - Most common & severe in female - Mainly in fall & winter
Disease → 60-
- T1DM > T2DM adolescents - Men > Women
- Men > Wome
Etiology - About 23 different proteins In both Type 1 and Type 2 diabetes. - Deposition of circulating immune - Associated preceding infection - Anti-GBM ant
Systemic amyloidoses Risk factors complexes in ½ of cases ● Serum
- SAA → most common - Genetic susceptibility - In situ complex formation ● Upper respiratory tract agains
● Acute phase reactant - Age (↑ risk in older pxs) infection type IV
● Chronic inflammatory - High blood pressure ● MMR vaccine found
conditions - High GFR - Risk factors - Exposure of a
- Light chain amyloidosis - Poor glycemic control ● Genetic
capillaries → pu
● Multiple myeloma - Race ● Immunologic
● Environmental affection
- ATTR → ↑black population - Obesity
● Smoki
Hereditary renal amyloidoses - Smoking
● Infectio
- Apolipoprotein AI & AII, - Oral contraceptive use
● Cocain
Fibrinogen, Lysozyme - Genetic susc
Localized amyloidoses
Pathophysiology Cell secretes protein in soluble - Glomerular hyperfiltration → - Circulating immune complexes → - Immune-mediated vasculitis w/ - Antibody bind
IgA1 deposition basement mem
form → misfolds in βpleated induces glomerulosclerosis complement activation →
- IgA1-containing immune - Only affects g
sheet pattern - Hyperglycemia and AGEs ● Tissue damage alveoli becaus
● Sclerosis of efferent ● Leukocyte infiltration complexes → neutrophils and
→ Extracellular deposition of ● ↑ acce
● Procoagulant factors monocytes → organ damage
misfolded proteins → organ arteriole → hyperfiltration ● Cytokine release ● ↑ α3 c
● Glycation of matrix proteins - Kidney: IgA, C3, fibrin, IgG &
damage some IgM - Immune resp
● Mesangial ECM production
→ Mesangial expansion & injury - Purpura → due to gravity, mostly monocytes, ma
in lower extremities dendritic cells.
- Cytokines: hyperglycemia →
● ↑TGFβ, VEGF, IGF-1 →
Remodeling
Clinical features Systemic - Tightly related w/ diabetic - Nephritic → Class III & IV Tetrad Syndrome - T
- Involves many organs like retinopathy - Arthritis / arthralgia - Diffuse pulmo
- Nephrotic → Class V & VI
kidney, liver, spleen, g.i tract & - Diabetes symptoms or px in - Purpura hemorrhage
Lupus manifestations - SOAP BRAIN
heart treatment - G.i. symptoms - Glomerulone
MD
Hereditary renal → mainly - Enlarged kidneys - Renal disease - Anti-GBM ant
Serositis, Oral ulcers, Arthritis, Other organs affected: scrotum, Disease (20-4
kidney Photosensitivity, Blood (pancytopenia), CNS, PNS, lungs, eyes. - Only GN & An
Localized → organ involved Renal, ANA, Immunologic (dsDNA), antibodies
- Enlarged kidneys Neurologic, Malar rash, Discoid rash.
Lab diagnosis Subcutaneous fat or rectal - Starts w/ microalbuminuria DIAGNOSIS → clinical, then confirm - ↑serum IgA - Anti-GBM Ab
mucosa aspiration for the ● 30-300 mg - ANAs (most sensible) - PT, PTT & platelets are normal - ⅓ of pxs → A
biopsy. - Progresses to nephrotic syndrome - Anti-dsDNA & Anti-Smith (most - Hypocomplementemia - Anemia & leu
specific) - Evidence of recent infection - ↑BUN & crea
Disease Amyloidosis Diabetic Nephropathy Lupus Nephritis Henoch-Schönlein Purpura Goodpastur
Epidemiology - Mostly in old patients - Most common cause of ESRD - Common SLE complication - Peak age: 3-15 y.o. Syndrome → 2
- 10 years after disease onset - Most common & severe in female - Mainly in fall & winter
Disease → 60-
- T1DM > T2DM adolescents - Men > Women
- Men > Wome
Etiology - About 23 different proteins In both Type 1 and Type 2 diabetes. - Deposition of circulating immune - Associated preceding infection - Anti-GBM ant
Systemic amyloidoses Risk factors complexes in ½ of cases ● Serum
- SAA → most common - Genetic susceptibility - In situ complex formation ● Upper respiratory tract agains
● Acute phase reactant - Age (↑ risk in older pxs) infection type IV
● Chronic inflammatory - High blood pressure ● MMR vaccine found
conditions - High GFR - Risk factors - Exposure of a
- Light chain amyloidosis - Poor glycemic control ● Genetic
capillaries → pu
● Multiple myeloma - Race ● Immunologic
● Environmental affection
- ATTR → ↑black population - Obesity
● Smoki
Hereditary renal amyloidoses - Smoking
● Infectio
- Apolipoprotein AI & AII, - Oral contraceptive use
● Cocain
Fibrinogen, Lysozyme - Genetic susc
Localized amyloidoses
Pathophysiology Cell secretes protein in soluble - Glomerular hyperfiltration → - Circulating immune complexes → - Immune-mediated vasculitis w/ - Antibody bind
IgA1 deposition basement mem
form → misfolds in βpleated induces glomerulosclerosis complement activation →
- IgA1-containing immune - Only affects g
sheet pattern - Hyperglycemia and AGEs ● Tissue damage alveoli becaus
● Sclerosis of efferent ● Leukocyte infiltration complexes → neutrophils and
→ Extracellular deposition of ● ↑ acce
● Procoagulant factors monocytes → organ damage
misfolded proteins → organ arteriole → hyperfiltration ● Cytokine release ● ↑ α3 c
● Glycation of matrix proteins - Kidney: IgA, C3, fibrin, IgG &
damage some IgM - Immune resp
● Mesangial ECM production
→ Mesangial expansion & injury - Purpura → due to gravity, mostly monocytes, ma
in lower extremities dendritic cells.
- Cytokines: hyperglycemia →
● ↑TGFβ, VEGF, IGF-1 →
Remodeling
Clinical features Systemic - Tightly related w/ diabetic - Nephritic → Class III & IV Tetrad Syndrome - T
- Involves many organs like retinopathy - Arthritis / arthralgia - Diffuse pulmo
- Nephrotic → Class V & VI
kidney, liver, spleen, g.i tract & - Diabetes symptoms or px in - Purpura hemorrhage
Lupus manifestations - SOAP BRAIN
heart treatment - G.i. symptoms - Glomerulone
MD
Hereditary renal → mainly - Enlarged kidneys - Renal disease - Anti-GBM ant
Serositis, Oral ulcers, Arthritis, Other organs affected: scrotum, Disease (20-4
kidney Photosensitivity, Blood (pancytopenia), CNS, PNS, lungs, eyes. - Only GN & An
Localized → organ involved Renal, ANA, Immunologic (dsDNA), antibodies
- Enlarged kidneys Neurologic, Malar rash, Discoid rash.
Lab diagnosis Subcutaneous fat or rectal - Starts w/ microalbuminuria DIAGNOSIS → clinical, then confirm - ↑serum IgA - Anti-GBM Ab
mucosa aspiration for the ● 30-300 mg - ANAs (most sensible) - PT, PTT & platelets are normal - ⅓ of pxs → A
biopsy. - Progresses to nephrotic syndrome - Anti-dsDNA & Anti-Smith (most - Hypocomplementemia - Anemia & leu
specific) - Evidence of recent infection - ↑BUN & crea
