Glomerular Nephritis vs Nephrotic Flashcards
what is the most common cause of end-stage-renal disease worldwide?
glomerular disease
what is the difference between the nephritic spectrum and nephrotic spectrum?
nephritic = basement membrane damage = leaky
nephrotic = podocyte damage = lose proteins
what are the 7 characteristics of nephritic syndrome?
hematuria (smoky/cola)
< 3.5 g/day proteinuria
hypertention
oliguria
general edema
decreased GFR
elevated BUN/SCr
what are the 5 characteristics of nephrotic syndrome?
severe proteinuria > 3.5 g/day
hyperlipidemia
generalized edema
hypercoag
osteomalacia
what are the 5 nephritic syndromes?
poststreptococcal GN
IgA nephropathy (Berger disease)
Henoch-Schonlein Purpura (IgA vasculopathy)
Lupus Nephritis
Pauci-immune Necrotizing GN
Anti-glomerular basement membrane GN (Goodpasture’s disease)
what is the most common nephritic disease in children?
post-strep glomerulonephritis
what is the patho of post-strep glomerulonephritis?
antibodies and antigens go into mesangium + subendothelium = hypersensitivity reaction
a patient presents with a history of an infection 2 weeks ago. on physical exam, they are hypertensive, report hematuria, and have edema. Dx?
post-strep (infection related) glomerulonephritis
what will be present in UA in post-step glomerulonephritis? (2)
GFR and BUN/Cr?
cola colored/brown/smoky
proteinuria
decreased GFR
elevated BUN/Cr
electron microscope shows large, dense subepithelial deposits or “humps” and immunofluorescent shows a “starry sky appearance”. Dx?
post-strep glomerulonephritis
what is the mainstay treatment for post-strep glomerulonephritis?
supportive treatment
what to give for HTN and proteinuria in PSGN? edema? what if it develops rapidly progressive glomerulonephritis?
ACEI / ARB
lasix / furosemide +/- thiazide
dialysis
what is the most common primary glomerular disease globally?
IgA nephropathy (Berger Disease)
patho of IgA nephropathy (Berger Disease)?
IgG attached to abnormal IgA = immune complexes deposit into mesangium = hypersensitivity reaction
a patient presents with a history of a URI/cough/sore throat. they report hematuria, hypertension, edema to hands and feet, flank pain, fever, and malaise. Dx?
IgA nephropathy (Berger Disease)
what will be present in UA and labs for IgA nephropathy?
cola/tea urine
increase IgA
light microscopy shows focal mesangial proliferation, immunofluorescent shows diffuse mesangial IgA and C3 deposits. Dx?
IgA nephropathy (Berger Disease)
what is the management for IgA nephropathy (Berger) in a patient that is low risk w/ minimal hematuria and proteinuria, no hypertension, and normal GFR?
monitor yearly
what is the management for IgA nephropathy (Berger) in a patient that is high risk w/ gross hematuria, protein >1g/day, hypertension, and decreased GFR?
what should be added to treatment if protein remains elevated after 3-6 mo and GFR >30?
ACI/ARB
lasix/furosemide
steroids
what is the treatment for IgA nephropathy (Berger) that progressed to rapidly progressive GN? (2)
cyclophosphamide + corticosteroids
what is the most common vasculitis in children?
Henoch-Schonlein Purpura (IgA vasculitis)
a patient presents with a history of a URI or drug exposure, they have a palpable purpura rash to LE and buttocks, abdominal pain, and arthalgias. Dx?
henoch-schonlein purpura (IgA vasculitis)
what is the patho of henoch-schonlein purpura (IgA vasculitis)?
IgG-IgA immune complexes attach blood vessels
what is the diagnostic of choice for henoch-schonlein purpura (IgA vasculitis)? what will it show?
kidney biopsy
mesangial proliferation like IgA nephropathy
what does skin biopsy show in henoch-schonlein purpura (IgA vasculitis)?
leukocytoclastic vasculitis
what is the treatment for henoch-schonlein purpura (IgA vasculitis)? (4)
rituximab
prednisone
analgesics
wound care
what are the 2 different types of Pauci-immune necrotizing glomerulonephritis?
perinuclear-ANCA
cytoplasmic -ANCA
what are the 3 different p-ANCAs?
“PEM”
Polyarteritis Nodosa
Eosinophilic granulomatosis with polyangiitis (Churg Strauss)
Microscopic polyangiitis
affects small and medium arteries of the abdomen and kidneys resulting in aneurysms
polyarteritis nodosa (PAN)
allergic reaction with asthma and eosinophilia affecting the respiratory tract and kidneys
eosinophilic granulomatosis w/ polyangiitis (Churg Strauss)
necrotizing vasculitis primarily affecting the kidneys, but can affect pulmonary capillaries
microscopic polyangiitis
what is a type of c-ANCA?
granulomatosis with polyangiitis (Wegener granulomatosis)
granulomatous inflammation involving the respiratory tract and renal necrotizing vasculitis
granulomatosis with polyangiitis (Wegener granulomatosis)
a patient presents with oliguria-anuria, hypertension, edema, hematuria, hemoptysis, fever, malaise, weight loss, and purpura. Dx?
pauci-immune necrotizing GN
which pauci-immune necrotizing GN targets myeloperoxidase antigens (anti-MPO)?
p-ANCAs
which pauci-immune necrotizing GN targets proteinase 3 (PR3) antigens?
c-ANCAs
what will help diagnose the specific type of pauci-immune necrotizing GN?
ANCA serology for anti-MPO or anti-PR3
what is the treatment for pauci-immune necrotizing GN? (5)
high-dose steroids x 3 days
+/- plasmapheresis
cyclophosphamide
azathioprine
prophylactic bactrim (pneumo. jirovecii)
what is the patho of anti-glomerular basement membrane GN (goodpasture’s disease)?
autoantibodies target basement membrane in kidneys and lungs = glomerular crescents + pulmonary hemorrhage
a patient presents with rapid decline in renal function, +/- pulmonary hemorrhage, cough, SOB, DOE, and hemoptysis. Dx?
anti-glomerular BM GN (goodpasture’s disease)
what will be seen in anti-glomerular BM GN (goodpasture’s disease)? (2)
elevated anti-GBM antibodies
negative ANCAs
what does biopsy of anti-glomerular BM GN (goodpasture’s disease) show of LM and IF (2)?
crescent formation
linear deposition of IgG + GBM
what is the treatment for anti-glomerular BM GN (goodpasture’s disease)? (3)
intensive plasmapheresis
prednisone
cyclophosphamide
what is the patho of lupus nephritis?
ANA attacks antigens = immune complexes = hypersensitivity reaction
a patient presents with fever, edema, hypertension, butterfly rash/malar rash, alopecia, joint/muscle pain, frothy urine. Dx?
lupus nephritis
identify the class: minimal mesangial lupus nephritis
class 1
identify the class: mesangial proliferative lupus nephritis
class 2
identify the class: focal lupus nephritis
class 3
identify the class: diffuse lupus nephritis
class 4
identify the class: membranous nephropathy
class 5
identify the class: advanced sclerosing lupus nephritis
class 6
which 2 classes of lupus nephritis are the most active and are further classified as acute or chronic and MUST be treated?
classes 3 + 4 (focal + diffuse)
what is the treatment for class 1 and 2 LN?
ACEI / ARB
what are 2 treatment options for class 3 and 4 LN?
corticosteroids + mycophenolate mofetil
OR
cyclophosphamide
what is the treatment for class 5 LN?
calcineurin inhibitors
what is the treatment for class 6 LN?
dialysis + transplant
what is 3 supportive therapies for LN?
ACEI / ARB
statin
antiplatelet
what is the patho of nephrotic syndrome?
glomerular damage allows albumin into filtrate = decreased oncotic pressure = edema = RAAS = more cholesterol by liver = excess lipids in urine
what is the most common complication of nephrotic syndrome?
spontaneous blood clot
what will LM and IF show in nephrotic syndrome?
LM = oval fat bodies
IF = maltese cross pattern
what will be present in labs in nephrotic syndrome?
microcytic, hypochromic anemia
what does renal biopsy show in nephrotic syndrome?
podocyte effacement
what is the mainstay of nephrotic syndrome treatment?
treat underlying cause
how to treat proteinuria in nephrotic syndrome?
ACEI / ARB
how to treat edema in nephrotic syndrome? (3 line treatments)
1: loop diuretic / lasix
#2: thiazide
#3: IV albumin
how to treat dyslipidemia in nephrotic syndrome? (2)
statins
exercise
how to treat hypercoagulability in nephrotic syndrome?
warfarin 3-6 months
what should be done with patients with nephrotic syndrome?
refer to nephrology
what are the 3 types of primary glomerulonephrosis?
minimal change disease
focal segmental glomerulosclerosis
membranous GN
what are the 2 types of secondary glomerulonephrosis?
diabetic nephropathy
amyloidosis
what are the type of hereditary glomerulonephrosis?
thin basement membrane
what is the most common cause of nephrosis in children and the 3rd most common cause of nephrosis in adults?
minimal change disease
a patient presents with an abrupt onset of generalized swelling, massive proteinuria > 6g/day, hypoalbuminemia, albuminuria, and lipiduria. EM shows widespread effacement of podocytes. Dx?
minimal change disease
what is the treatment for minimal change disease?
high-dose prednisone
what is the patho of focal segmental glomerulosclerosis?
podocyte detachment / cell death = spreads to adjacent podocytes
a patient could have asymptomatic proteinuria or nephrotic syndrome. Dx?
focal segmental glomerulosclerosis
biopsy LM shows segmental sclerosis and IF shows trapped IgG and C3, and EM shows podocyte effacement. Dx?
focal segmental glomerulosclerosis
what is the treatment for primary focal segmental glomerulosclerosis?
prednisone
+/- cyclosporine / tacrolimus if resistant
what is the treatment for secondary focal segmental glomerulosclerosis?
treat cause
what is the most common cause of primary nephropathy in nondiabetic adults?
membranous nephropathy
what is the patho in membranous nephropathy?
immune complex deposit attacks podocyte = GBM thickens and expands = causes spike and dome
what is secondary membranous nephropathy associated with?
cancer
a patient presents with progressive weight gain over weeks, an unprovoked blood clot. UA show albuminuria and lipiduria. labs show a phospholipase A2 receptor antibody and LM shows “spike and dome pattern”. Dx?
membranous nephropathy
what is the treatment for mild primary membranous nephropathy? (3)
ACEI / ARB
lasix
anticoagulants
what is the treatment for moderate primary membranous nephropathy? (3)
rituximab
OR
corticosteroids + cyclophosphamide
what is the most common cause of end-stage renal disease in the US and western societies?
diabetic nephropathy
a patient presents with hypertension, heavy proteinuria, and retinopathy. Dx?
diabetic nephropathy
what is a pathognomonic finding in diabetic nephropathy?
Kimmelstiel-Wilson nodules
what is the treatment for diabetic nephropathy? (5)
Na + fluid restriction
strict glycemic control (SGLT2 inhibitor)
statin (HLD)
ACEI / ARB
+/- diuretics
group of rare diseases caused by extracellular deposits of insoluble fibrils within tissues
renal amyloid
a patient presents with nephrosis with significant proteinuria . 20g/day, and swelling in face/ankle/tongue/legs. labs show a monoclonal spike and biopsy shows a congo red stain. LM natural light appears pink and LM polarized light appears apple green. Dx?
renal amyloid
what is the treatment for primary renal amyloid in less severe disease?
melphalan + stem cell transplant
what is the treatment for primary renal amyloid in severe disease?
melphalan
high-dose steroids
bortezomib
what are 3 treatment options for secondary renal amyloid?
antibiotics
eprodisate
renal transplant
what is the most common cause of asymptomatic hematuria?
thin basement membrane nephropathy
what is the treatment for thin basement membrane nephropathy?
trial with ACEI to reduce hematuria