Nephritis Nephrosis TBL Flashcards
How is nephrotic syndrome defined?
- acute onset of edema
- proteinuria (>3.5g/d)
- hypoalbuminemia
- hyperlipidemia
- lipiduria
What is acute nephritic syndrome
- edema
- hypertension
- azotemia (sometimes oliguria)
- nephritic urinary sediment (RBC, WBC, cellular casts)
- mild proteinuria
What is Rapidly Progressive glomerulonephritis (RPGN). How serious is it?
rapid decrease of GRF associated with evidence of GN on urinary sediment
GLOMERULAR CRESENT
-emergency need fast tx
What are the 3 components of the glomerular epithelial barrier
- fenestrated capillary endothelium
- glomerular basement membrane
- podocytes
What prevents plasma proteins from leaking into the urinary space?
Slit diaphragms bridgeing the filtration slits between adjacent podocyte foot processes
What is the etiology of GN?
deposition of immune complexes
leukocyte infiltration
inflammation disruption of the filtration barrier
What is Membranous Nephropathy (MN) or Membranoproliferative glomerulonephritis (MPGN)
abnormal thickening of the GBM
What is the differential diagnosis for nephropathy?
- Focal and segmental glomerulosclerosis (FSGS)
- collapsing GN
- Minimal change disease (MCD)
- Membranous nephropathy (MN)
- Diabetic Nephropathy (DN)
- disorders associated with plasma cell dyscrasias (amyloidosis)
What are causes of MCD?
NSAID, Hodgkin’s lymphoma, idiopathic
What are causes of secondary FSGS?
Reduced nephron mass
Sickle cell disease
Massive obesity
inherited podocytopathy
What are causes of Collapsing GN?
HIV-associated nephropathy Pamidronate use (breast cancer, myeloma pts)
What are causes of Membranous Nephropathy?
SLE –> antirheumatic drugs
Hep B
Solid tumors
What are some deposition disease associated with Nephropathy?
Amyloidosis
Light chain deposition disease
What is the management for Nephropathy? (proteinuria, edema, hyperlipidemia)
Proteinuria: 1. ACE inhib 2. angiotensin II receptor blocker (ARB) Edema 1. salt restriction 2. loop diuretics Hyperlipidemia 1. statins
What are the characteristics of MCD? presentation?
- normal renal biopsy findings by light/immunofluorescency microscopy
- primary cause of nephrotic syndrom in children
- presents with rapid onset of anasarca + hypoalbuminemia, hyperlipidemia, proteinuria
- ELECTRON MICROSCOPY: effacement of foot processes
Characteristics of Primary FSGS?
- leading cause of nephrosis in BLACKS
- most common nephrosis etiology
- accumulation of fibrous scar tissue
- associated with HYPERTENSION
- progress to ESRD
What 2 things are MCD NOT associated with?
- Hypertension
2. severely diminished renal function
How do you distinguish MCD and FSGS?
pts w MCD respond to steroids
How is FSGS Dx’d?
electromicroscopy:
diffuse effacement of foot processes, with AREAS OF SCLEROSIS
How are primary and secondary FSGS different?
Primary: immunosuppressive therapy is indicated
Secondary: more gradual onset, MINIMAL EDEMA, minimal HYPOALBUMINEMIA
—should be thought of more as renal damage (sclerosis due to compensatory effort)
what are the causes secondary FSGS?
damage/loss of glomeruli:
- renal agenesis
- Sickle cell disease
- vesicoureteral reflux
- surgical removal of kidney
- -remaining glomeruli have higher load = hypertrophy/sclerosis
What is the Tx for Secondary FSGS?
ACEI, ARB, BP control, diuretics, statins
What is the etiology of Inherited FSGS?
podocyte protein mutations (nephrin, podocin, a-actinin-4, TRPC6)
= podocyte loss = FSGS
What are the age ranges for the specific mutations in Inherited FSGS?
Nephrin - birth
Podocin - Childhood
a-actining + TRPC6 - adult
What is the presentation of Collapsing Glomerulonephropathy
- Huge proteinuria (>10g/d)
- elevated creatinine
- rapid progression to ESRD
What virus is Collapsing glomerulopathy associated with?
Parvovirus B19
What the the lab results of Collapsing glomerulopathy?
US: large echogenic kidney
enormous WAXY CASTS
What is the Tx of Collapsing glomerulopathy?
HAART
ACEI, ARB
What is the cause of MN?
immune deposts beneath foot processes specific for podocyte protein = GBM thickening
How is Primary and Secondary MN diagnosed?
primary: biopsy, phospholipase A2 receptor Ab
secondary: complement levels, ANA, Hep B Ags, presence of infection
What is MN associated with in older people?
malignancy
GI, prostate, breast, lung
What are signs of amyloidosis causes of renal dysfunction?
- older nondiabetic w/ severe albuminuria (w/ HF, hepatomegaly, peripheral neuropathy)
- CONGO red staining
- MACROGLOSSIA
- circulating/urinary free light chains
What is the main cause of Nephritic syndrome?
inflammation within the glomerulus
What is the lab difference between Glomerulonephritis and nephrotic disorders
C3, C4 is abnormal in glomerulonephritis
What does a low C3 value with a normal C4 value indicate?
Poststreptococcal Glomerulonephritis or Dense Deposit disease
What does a low C3 and low C4 value indicate?
- lupus nephritis
- GN associated with chronic infection (endocarditis)
- hep C associated MembranoProlifGN
- cryoglobinemia
What type of GN is indicated by normal complement levels?
Small vessel vasculitis associated w/ ANCAs and anti-GBM disease
What is the most common cause of GN? what age/race is most common
IgA nephropathy. whites/asians , younger men/boys
What are the Sx of IgAN?
HX OF URI asymptomatic microscopic hematuria mild proteinuria BROWN urine flank pain
What other disease is IgAN associated with?
RA
spondyloarthropathies
GI (celiac, chronic liver disease)
Henoch-Schonlein purpura (lesions on legs/forearms)
what group is SLE related GN common in?
what is Dx?
young women - black or hispanic
check ANA titers
what are Sx of Poststreptococcal Glomerular Nephritis (PSGN)
gross hematuria proteinuria edema SEVERE HTN azotemia, sometimes oliguria
How is PSGN diagnosed
Hx (2-3 weeks after pharyngitis or skin infections cause group A B-hemolytic Strep)
Hypocomplementemia (low C3, normal C4)
anti-streptolysin O (ASO) titers
DNase B, hyaluronidase Abs
What kind of pts have Endocarditis-associated glomerulonephritis
IV drug users
prosthetic heart valves
What are Sx of EAGN?
right heart involvemnt
myalgias, arthralgias, purpura
LOW C3, LOW C4
elevated ESR, CRP
what type of infection is associated with Acute Renal failure
Acute endocarditis from S. Aureus
Where is type 1 MPGN seen most often? what are Sx?
adolescents/young adults severe proteinuria nephrotic syndrome accompanying nephritic features hematuria, RBC casts Azotemia, HTN
In adults, what is type 1 MPGN usually seen secondarily to?
chronic infection process systemic autoimmune disorder lymphoma Hep C Virus (MOST COMMON) mixed cryoglobulinemia
What are the lab results of MPGN?
low levels of complement C3, C4
mixed nephritis-nephrotic presentation
What are histological features of MPGN?
duplication of the GBM (tram tracks)
what is the presentation of Dense deposit Disease?
nephrotic syndrome in childhood/adolescnece
low C3, normal C4
HTN
microscopic hematuria
What are the prominent risk factors of Rapidly progressive glomerulonephritis?
- small-vessel vasculitis
2. auto-Abs against the GBM
What does RPGN renal biopsy look like?
cresentic lesion on renal biopsy
what causes primary FSGS?
idiopathic
What is RPGN caused by?
Goodpasture syndrome (anti-GBM Abs) - “ribbonlike staining”
SLE
Wegemers Granulomatosis (vasculitis)
CRESCENT LESIONS
