Jan11 M2,3-Pathology of glomerular diseases and renal tubules Flashcards
give 5 primary glomerular diseases
- membranous nephropathy
- MCD
- FSGS
- IgA nephropathy
- Membranoproliferative GN
give 5 secondary or systemic diseases that affect the kidney (glomerulus)
- DM
- amyloidosis
- SLE
- drugs (NSAIDs)
- infectious (HIV, hepB,C, syphilis)
- malignancy + paraneoplastic
4 clinical presentations for glomerular disease
hematuria, proteinuria, mixed, renal failure
how to know if hematuria is due to a glomerular disease (and not something more downstream)
if glomerular disease, RBC casts present
RBC casts point to what syndrome
nephritic. no RBC casts in nephrotic syndrome
pure nephrotic is caused by what
problem in glomerular filtration barrier (GBM or other)
proteinuria qt in nephrotic
3-3.5g a day or more
2 types of proteinurias in nephrotic + def
selective: albumin only
non-selective: albumin + other prots too
5 charact of nephrotic syndrome (labs)
- heavy proteinuria (3g a day)
- hypoalbuminemia
- hyperlipidemia
- lipiduria
- edema
nephrotic syndrome: urine sediment findings
- protein cast/waxy cast
- fatty casts
- oval fat bodies (desquamated tubular cells filled with reabsorbed lipids)
- RBCs but no cast
can you find RBCs in nephrotic syndrome urine sediment
yes but no RBC casts
nephrotic syndrome: 3 main primary diseases
- MCD
- FSGS
- membranous nephropathy
nephrotic syndrome: 3 main secondary diseases/conditions that can cause it
- DM
- amyloidosis
- pregnancy
nephrotic syndromes (2 primary and 2 secondary) that present with hematuria
primary: 1. membrano-proliferative GN 2. IgA nephropathy
secondary: 1. lupus nephritis 2. Henoch-Schonlein purprue (HSP)
most common PRIMARY nephrotic syndrome disease in children and most common PRIMARY in adult
children: MCD
adults: FSGS
most common SECONDARY nephrotic syndrome diseases in adults
- DM
- amyloidosis
- SLE
MCD: proteinuria type and only thing seen on biopsy
selective
only see effacement of foot processes on EM (LM and IF negative)
MCD treatment
steroids
MCD recurrence and prognosis
2/3 recurrence
very good prognosis
patho characteristics seen in some nephritic but not nephrotic syndrome
crescents, proliferation, hypercell, necrosis, inflammation
why selective proteinuria in MCD (2)
swelling of podocytes and podocyte fusion = loss of negative charge
general pathological feature of DM
deposition of glycosylated molecules on BMs everywhere
pathophgy of FSGS
injury (toxic or immune or genetic) to podocytes and loss of negative charge (hematuria is non selective)
main causes of FSGS (cause of podocyte toxicity)
HIV, heroin, hyperfiltration (high BP), medication
what type of immune toxicity to podocytes in FSGS
immune dysregulation with cytokines/T cells but no immune complexes and no autoimmune rx
prognosis of FSGS
50% dev ESRD in 10 yrs
type of proteinuria in FSGS and hematuria or not
non-selective proteinuria.
hematuria is mild or moderate
FSGS (focal segmental glomerulosclerosis): sclerosis meaning
scarring (fibrosis). less 50% glomeruli and part of glomeruli
why is there a vicious circle in FSGS
hyperfiltration injury. more P in GCs that still function so hyperfiltration injury in the glomerulus and sclerosis of nephrons
FSGS sometimes associated with ____
htn
membranous nephropathy: pathogenesis
antibodies recognize self protein on glomerular epithelial cells (podocytes)
membranous nephropathy: antibody often present (antibody against what)
antibody against PLA2R (podocyte antigen phospholipase A2 receptor)
membranous nephropathy: how fast it progresses + prognosis
slowly
40% progress to renal failure
membranous nephropathy: usual primary cause
a pathology of the GBM (membranous nephropathy is a form of GN)
membranous nephropathy: usual secondary causes (4)
- infections (hep, syph, mal)
- cancer
- autoimmune (SLE)
- drugs,toxins
FSGS: LM, IF and EM findings
LM: sclerosis + loss of capillaries
EM: non specific deposits of IGM and C3 segmentally
EM: effacement of foot processes
membranous nephropathy: LM, IF and EM
LM: diffuse thickening of the glomerular capillary walls
IF: IgG granular along the GBM
EM: sub-epithelial deposits, spikes
Diabetic nephrotic syndrome: at what point is it nephrotic syndrome
when proteinuria goes beyond the usual diabetic proteinuria
diabetic nephrotic syndrome LM, IF and EM
LM: diffuse or nodular GS, increased mesengium (KW lesions), arteriosclerosis, arteriolosclerosis, hyalinosis
IF: non specific
EM: thickening of GBM
Kimmelstiel-Wilson (KW) lesions in what condition and what is it
diabetic nephrotic syndrome
mesengial matrix increase and mesengial nodules
proteinuria and pregnancy usually sign of what
hypertension/pre-eclampsia
which nephrotic syndrome diseases (primary and 2ndary) get a biopsy and which do not
MCD: no FSGS: yes MN: yes DM: no (or ?) pregnancy: no
azotemia and uremia def
azotemia: high BUN
urea: high blood urea
nephritic syndrome typical findings (like the 5 of nephrotic but for nephritic)
hematuria, RBC casts, proteinuria, renal failure (azotemia), htn
3 most common primary nephritic syndrome diseases (primary GNs) + 1 that we have to remember
- acute post-infectious GN
- IgA nephropathy
- membrano proliferative GN (MPGN)
- RPGN
4 most common secondary nephritic syndrome diseases
- lupus nephritis
- Henoch-Schonlein purpura (HSP)
- anti-GBM (Goodpasture syndrome)
- Vasculitis/ANCA related GN
acute post infectious GN affects what pop typically
children
acute post infectious GN: after what infections usually
beta hymolytic streptococcal infections or URT or skin.
other: staph, pneumococcus, viruses
presentation in acute post infectious GN
tea/cola colored urine, RBC casts, proteinuria, hematuria
acute post infectious GN: LM if ever take a biopsy (2-3 things)
diffuse global hypercellularity (prolif of glomerular cells) and infiltration of PMN leukocytes (exudative GN) (+ crescents sometimes)
acute post infectious GN on IF if ever take a biopsy
large granular deposits
C3 and IgG along GBM specifically
how IgG deposits in membranous nephropathy (or membranous GN) and acute post infectious GN differ
in MN, smaller deposits and look like a stream
in acute post infectious GN, granular and larger (but deposit on GBM in both cases)
prognosis and dev to ESRD in acute post infectious GN
good prognosis
children mostly affected and 1% children ESRD
adults (rarely): but 15-50% ESRD
labs in acute post infectious GN (2)
- low C3 (bc massive deposition in the kidney
- positive ASOT (for strep infection)
IgA nephropathy (Berger): when does it happen and who is affected
1-2 days after URTI
children and young adults
IgA nephropathy (Berger): prognosis
25% ESRD in 10 yrs
fix things (2) that are common to all IgA nephropathy (Berger) patients on biopsy
- mesengial prolif
- IgA deposition on mesengium (IF)
variable features of pathophgy of IgA nephropathy (Berger)
highly variable in inflammation, crescents, necrosis and scarring
Henoch-Schonlein purpura (HSP) def
systemic form of IgA nephropathy with vasculitis
Henoch-Schonlein purpura (HSP) presentation
gross hematuria, GI symptoms, palpable purpura (vasculitis)
Henoch-Schonlein purpura (HSP) special thing about the biopsy + finding
can do it on the skin
find vasculitis with IgA deposits
Henoch-Schonlein purpura (HSP) prognosis
even better than IgA nephropathy
RPGN presentation
hematuria, htn, RBC casts, oliguria
RPGN cause
no cause, it’s a syndrome/clinical presentation
RPGN main feature
crescents on biopsy
RPGN often associated with what cause
-lupus
-anti-GBM
-etc
or sometimes idiopathic
treatment of RPGN
corticosteroids
RPGN prognosis
(IF UNTREATED), death from renal failure in weeks to months
crescents in acute vs chronic RPGN
acute: cellular crescent
chronic: fibrous crescent
RPGN biopsy findings
inflam, necrosis, damage to GBM, proinflam mediators make the cells proliferate
T/F: crescents are the cause of damage in RPGN
false. leakage of blood and prot
3 main types of crescentic GNs
- anti-GBM
- immune complex mediated (SLE, IgA nephritis, MPGN, post-infectious
- pauci-immune (ANCA-related)
anti-GBM: pathophgy
antibodies to the type IV collagen (NC1 domain of alpha 3 chain) in the GBM
anti-GBM: when is called Goodpasture syndrome
when lung involvement too (type IV collagen also in the lungs)
anti-GBM treatment
steroids and plasmapheresis
anti-GBM labs
anti-GBM antibodies
anti-GBM prognosis
WITH treatment, 60% ESRD
anti-GBM: LM, IF and EM
LM: crescents and necrosis
IF: linear IgG deposits on GBM
EM: no deposits visible
pauci-immune GN: what population + prognosis
older patients. with treatment, 30% reach ESRD in less than 2 years
pauci-immune GN labs
ANCA (anti neutrophil cytoplasmic antibody)
pauci-immune GN: LM, IF, EM
LM: crescents
EM: no deposits
IF: no deposits (or minimal)
pauci-immune GN: amunt of immune involvement
small
any disease with Ab-Ag immune complex deposition can lead to ________
give 3 examples of diseases
RPGN
lupus, IgA nephropathy, anti-GBM
glomerular diseases with crescents (3)
- immune mediated ones (APIGN, MPGN, SLE, IgA nephritis)
- anti-GBM
- ANCA (pauci-immune)
glomerular diseases with inflammation involvement (exduate)
APIGN
glomerular diseases with scarring (5)
FSGS, diabetes, IGAN (and HSP), RPGN
glomerular diseases associated with htn
FSGS, diabetes, RPGN. Nephritic in general. htn = cause of nephrotic in adults
glomerular diseases that have hypercellularity (2 main)
APIGN, IGAN (and HSP), RPGN and crescentic diseases
what glomerular diseases have necrosis
IGAN (and HSP), anti-GBM
what kidney diseases have RBC casts (has to be glomerular bc RBC cast = glomerular)
IgA nephropathy, post-infectious GN, lupus, vasculitis, anti-GBM
(remember cast = nephritic)
what kidney diseases have RBC casts (has to be glomerular bc RBC cast = glomerular)
IgA nephropathy, post-infectious GN, lupus, vasculitis, anti-GBM
(remember cast = nephritic)
5 tubulointerstitial diseases
- acute pyelonephritis
- chronic pyelonephritis
- reflux nephropathy
- drug induced interstitial nephritis
- ATN (acute tubular injury)
acute pyelonephritis cause
bacterial infection (from UTI or form blood)
acute pyelonephritis symptoms
sudden onset, malaise, fever, chills, CVA pain, dysuria (painful whne urinate), urgency
acute pyelonephritis risk factors
immunosuppresion, women (shorter urehtra), procedures, obstruction, diabetes
pathology of acute pyelonephritis
bacterial involvement (one kidney), pus in interstitium, microabcesses and WBC in urine. pus in tubules.
papillary necrosis def
form of acute pyelonephritis with ischemic necrosis of the pyramids’ papilla in context of diabetes, obstruction, analgesic abuse
acute/drug-induced interstitial nephritis: causes
hypersensitivy/allergy/abnormal immune rx/direct toxicity from a medication (antibiotics, analgesics: penicillins, diuretics, NSAIDs)
acute/drug-induced interstitial nephritis: symptoms + onset
2-40 days after taking the drug.
oliguria, azotemia, fever
acute/drug-induced interstitial nephritis: UA
hematuria, leukocytes, NO RBC CAST, bit of proteinuria
pathology of acute/drug-induced interstitial nephritis
interstitial edema and inflammation
ATI/ATN three types
pre-renal, renal, post-renal
ATI/ATN is the most common cause of __________
AKI
ATI/ATN cause if pre-renal vs if post renal
pre-renal: ischemic damage
post-renal: obstruction by compression causes toxicity
manifestations of ATI/ATN
oliguria/anuria, drop GFR, electrolyte imbalance, acidosis, fluid overload
2 types of injuries to tubules in ATI/ATN
ischemic or toxic
most affected tubules in ATI/ATN
PCT
ATI/ATN biopsy findings
vacuolization of cytoplasm, apical blebbing, dilation of tubules, nuclear loss, denudation
amount of inflammation in ATI/ATN
none
diseases with hematuria
nephritic + acute pyelonephritis
