Glomerulonephropathies 1 Flashcards
acute glomerulonephritis
immunologic mechanisms triggers inflammation and proliferation of glomerular tissue
causes damage to kidney
thickening and proliferation in glomerulus
microalbuminuria
excretion of 30-300 mg of protein per day
sign of early renal disease
macroalbuminuria
excretion of >300 mg (3g) per day
nephritic syndrome
abrupt onset of proteinuria 1-3 g/day
+/- hematuria, RBC casts
nephritic syndrome is often accompanied by
HTN
Reduced GFR – oliguria, AKI
Edema/fluid overload
nephrotic syndrome
heavy proteinuria (>3g/day)
hypoalbuminemia, edema, hyperlipidemia
secondary causes of nephrotic syndrome
DM
lupus
amyloidosis
damage done in glomerulonephropathies
immune mediated inflammation and proliferation damages GBM, mesangium or capillary endothelium
deposition of immune complexes and complement in tissues of glomeruli PLUS invasion by PMNs and thickening of GBM
types of GN
focal (<50%)
diffuse (all glomeruli)
segmental (only part of tuft involved)
global (entire glomerular tuft)
GN pathology
thickening and cellular proliferation of glomerulus
GBM becomes leaky and lets proteins into ultra filtrate
persistent inflammation = fibrosis and scarring of GBM
rapid GN
> 50% loss of nephron function over WEEKS
can be permanent
prolonged GN
inflammatory changes over time persistent abnormalities
general clinical findings of GN
edema (decreased onchotic pressure)
weakness, fever, abdominal pain, malaise
HTN
urine is dark and volume reduced
evidence of renal failure
proteinuria, RBCs and RBC canes in U/A
pathognomonic GN finding
RBC casts in U/A
special lab work up to determine GN etiology (8)
- Complement - CH50, C3, C4 (low levels = GN)
- Anti GBM autoAbs
- ANA (SLE, autoimmune)
- anti-dsDNA (SLE, autoimmune)
- ANCA (vasculitides)
- HIV
- viral hepatitis panel
- anti-PLAR2 recetor
diagnostic study of choice in GN
renal biopsy looking for immune deposits
confirm diagnosis and type of GN
minimal, linear, granular
minimal deposits GN (3)
- pauci-immune GN (vasculitides)
- GPA/Wegener’s
- Eosinophillic granulomatosis (Churg-Strauss)
linear deposits GN (1)
- anti GBM GN
granular deposits (8)
- PSGN
- IgA nephropathy
- HSP
- HIV
- HBV
- HCV GN
- cryoglobulinemia
- SLE/autoimmune
general GN treatment
reduction fo HTN and fluid overload
treat underlying cause
salt and water restriction
post infectious GN (PSGN)
due to subtype of group A hemolytic strep, increased caused by staph infection
appears 1-4 WEEKS following impetigo, cellulitis, pharyngitis, UTI - also seen in other bacterial infections
uncommon in developed countries, 60+, 5-12 y/o, M>F
PSGN mechanisms of damage (4)
- circulating immune complex deposits in GBM
- strep antigens deposit in GBM, cause immune reaction
- Abs against strep antigens react with glomerular proteins (molecular mimicry)
- nephritic strain of strep turns IgG against us
s/s of pSGN
oliguria, edema , HTN
colca colored urine with RBCs, RBC casts, proteinuria <3 g/day
LOW serum complement + Strep (or other agent) titers HIGH
diagnosing PSGN + tx
renal biopsy needed to r.o other causes
TX: ABX, salt and fluid restriction, diuretics, anti-HTN (CCB)
oliguiria/fluid retention spontaneously resolve - proteinuria and hematuria can take 6-12 months to resolve
IgA Nephropathy
MC form of acute glomerulonephritis (particularly in asians and caucasians)
teens or young adults, M>F
IgA and C3 deposition in the glomerular mesangium
cause of IgA Nephropathy
unknown- may develop with minimal/no symptoms
associated with hepatic cirrhosis, celiac dz, HIV
IgA Nephropathy symptoms
episodes of gross hematuria (1-3 days) and associated with URI, GI, Flu illnesses
30-40% have microscopic hematuria and mild proteinuria on routine UA
RARE nephrotic syndrome or progressive GN with nephritic pic
clinical course IgA Nephropathy
gross hematuria lasting 1-3 days associated with URI, GI, or fLU illness
urine: red-cola colored 1-3 DAYS after onset of associated illness
flank pain, low grade fever (mimic UTI)
IgA Nephropathy lab findings
IgA levels increased
complement normal
renal biopsy: focal GN, diffuse mesangial IgA C3 deposits, proliferation of cells
- bx is persevered for severe/progressive disease
tx of IgA Nephropathy
HTN or proteinuria = ACE/ARB
fish oil- anti inflammatory
corticosteroid (proteinuria 1-3 g)
immunosuppressant may be used
prognosis fo IgA Nephropathy
most unfavorable prognostic indicator is proteinuria >1g/day
1/3 have remission, some have progressive insufficiency and ESRD, microscopic hematuria and stable GFR
IgA Vasculitis
small vessel vasculitis of unknown cause
MC form of systemic vasculitis in children
MC in children, male predominance, self limited
occurs in fall, winter, spring, preceded by URI
clinical features IgA Vasculitis
- palpable purpura (w/o thrombocytopenia or coagulopathy)
- arthritis (lg joints, swelling and tenderness)
- abdominal pain (colicky, worsen to GIB, intussception)
- renal disease
distinguish IgA Vasculitis and IgA Nephropathy
identical lesions on renal biopsy
IgA Vasculitis is cutaneous, GI, Connective tissue
diagnosis of IgA Vasculitis
low C3/C4, high IgA levels
made if classic clinical pic present - if not, bx skin or renal
managed outpatient (OTC meds)
most recover fully and no recurrence (some have recurrence in 4 months)
rapidly progressing GN
anti-glomerular basement GN
connective tissue disorders
granulomatosis w/polyangiitis
hypersensitivity GN
anti GBM GN
rare disease, bimodal epidemiology (males in 20-30s, female in 50-60)
auto-An to subtype of collagen found only in glomerulus and kidney
goodpastures syndrome
AGBM GN
pulmonary hemorrhage mediated by anti-GBM abs and GN
can have HLA connection
anti-GBM GN clinical findings
URI HEMOPTYSIS Dyspnea HTN Edema Nephritic proteinuria RBC cast
renal biopsy in anti-GBM GN
ANCA - must distinguish between GPA and anti-GBM
crescentic GN and linear IgG deposits along glomerular capillaries (GPA = no complexes)
ANCA
antibody in both GPA and Anti-GBM
autos directed against granules in cytoplasm of neutrophils and monocyte lysosomes
found in 30% of patients with AGBM, indicates positive finding
anti-GBM GN tx
plasmapheresis (remove Abs) immunosuppressive drugs (prevent formation of new Abs and control inflammation)
mortality improved but need to catch early
connective tissue disorders RPGN
Type II
SLE, polyarteritis
glomerular inflammation caused by deposition of complexes in capillaries
GPA RPGN
aka Wegener’s
pacui-immune necrotizing GN
hypersensitivity vasculitis RPGN
rxn to drug or ag
causes widespread inflammation
MC: PTU, hydrazine, allopurinol, minocycline
renal cysts
epithelium lined cavities filled with fluid/semi solid material
common to have one in normal population
must distinguish from abscess, PCKD, malignancy
benign still require periodic monitoring
problematic renal cysts
scattered thru kidney
U/s, CT, surgical exploration
dialysis cyst
commonly develops
can mask adenocarcinoma
can use arteriography to evaluate
PCKD
autosomal dominate, multiple cysts
progress to ESRD
abdominal/flank pain, hematuria, HTN
large kidneys on exam, hepatic, splenic, pancreatic cysts
diagnosed with u/s
PCKD complications
- abdominal or flank pain (lg size + infection, bleeding, stones)
- hematuria (fragile, stretched vessels rupture, - gross = cyst rupture, recurrent = CA)
- renal infection (fluroquinolones x14 days, fever + leukocytosis)
- HTN - RAAS activation 2/2 ischemia
- nephrolithiasis
- Cerebral aneurysm
- other
poor prognosis indicators in PCKD
lg kidneys multiple infxn/hematuria HTN male sex AA ethnicity
