Glomerular Disease Flashcards
What are the usual clinical manifestations of Alport syndrome?
hearing loss and anterior lenticonus, family history of hematuria
What is the membranous nephropathy antigen associated with SLE?
Exostosin 1 & 2
what is the most common congenital complement deficiency in C3GN?
Factor H
Name 2 diseases where a nodular pattern is present on renal biopsy pathology. (Bonus: name 4)
Diabetes; amyloidosis; light chain deposition disease; idiopathic nodular glomerulosclerosis (smoker)
What is the lesion of reflux nephropathy?
FSGS
what is the most common lesion a/w sickle cell anemia?
FSGS
Name the 2 most common histologic findings in Sjogren syndrome.
interstitial nephritis; cryoglobulinemia
what is the euro-lupus cyclophosphamide dose?
500 mg cyclophosphamide IV q 2 weeks x 6 doses
which idiopathic glomerulopathy does lupus podocytopathy resemble?
Minimal change disease
which ANCA associated vasculitis relapses more, GPA (PR3+) or MPA (MPO+)
GPA or PR3 positive vasculitis
what infection is polyarteritis nodosa associated with?
chronic hepatitis B
name 3 types of hypocomplementemic GN? (bonus: name 4)
post-strep GN; subacute bacterial endocarditis; Lupus nephritis; C3GN
name 2 renal disease a/w eosinophilia? (bonus: name 3)
EGPA or churg strauss; AIN; atheroembolic disease
True or False: Warfarin nephropathy is limited to warfarin anticoagulation.
False. It’s also seen with NOACs
does warfarin nephropathy typically occur with a therapeutic INR?
No. the usual findings are a high INR and gross hematuria
which drug did the ALMS trial find superior for LN maintenance, azathioprine or MMF?
MMF
what does pauci mean?
having few or none.
Pauci immune means there is little or no staining on IF
does double serologic positivity for Lupus and ANCA improve or worsen the prognosis for SLE?
it worsens the prognosis for SLE
what lesion is a/w VEGF inhibitors?
TMA
the PEXIVAS study for ANCA vasculitis had 2 major findings. NAME them
moderate dose steroids worked as well as high dose steroids; PLEX did not improve renal outcomes
which of the ff is indicative of giant cell arteritis?
a) MPO+
b) PR3+
c) ANCA negative
ANCA negative. Most of the vasculitis larger than GPA and MPA are ANCA-
what is the typical glomerular lesion of parvovirus?
FSGS of the collapsing type or collapsing glomerulopathy
Glomerular disease most likely a/w venous thrombosis?
Membranous nephropathy
which light chain is most likely associated with Amyloidosis?
Lambda
which light chain (kappa or lambda) is most likely a/w LCDD?
Kappa
what hematologic finding is frequently seen in atheroembolic disease?
eosinophilia
an infection related GN with IgA can be seen in
a.) strep infection with nL C’
b.) strep infection with low C’
c.) Staph aureus infection
Staph aureus. IgA dominant postinfectious GN are more common in S. aureus infection and patients with DM who have deep seated infections
IgAN is a.w Abs to which of the ff?
a.) glycosylated albumin
b.) galactose deficient IgA1
c.) glucose deficient IgA1
D.) galactose deficient IgA2
Galactose deficient IgA1
which clinical factor portends the worst prognosis in IgAN?
a.) BP
b.) proteinuria
c.) gross hematuria after an URI
d.) chronic micsocopic hematuria
Proteinuria. Chronic microscopic hematuria portends a worse prognosis than intermittent hematuria, but proteinuria is still the strongest predictor of outcome.
what is the most common GN seen in GVHD?
membranous GN
name the protein that is pathognomonic for fibrillary GN
DNAJB9
the most common mutation causing FSGS involves which of the ff?
a.) nephrin
b.) megalin
c.) podocin
d.) VEGF
Podocin
which of the ff can be caused by ICPIS?
a.) AIN, especially with PPIs
b.) AIN, especially with H2B
c.) hypokalemia from adrenalitis
d.) hyponatremia from cerebral salt wasting
AIN esp with PPIs
Congo Red positivity can be seen win which 2 of the ff?
a) amyloidosis
b) DDD
c) Fibrillary GN
amyloidosis and fibrillary GN
Which of the ff is associated with TMA?
a) quinine
b) chlorthalidone
c) ciprofloxacin
d) propranolol
Quinine. Note that tonic water has quinine added to it
Normal kappa to lambda FLC ratio behaves in which of the ff ways? (nL is 0.26 to 1.65)
a) decreases in CKD
b) increases with CKD
c) increases with liver failure
d) can increase as an acute phase reactant
increases with CKD,
nL ratio in CKD is 0.37 to 3.1
which of the ff is a/w MPGN?
a) IgAN
b) C4 deficiency
c) Quinine
d) Monoclonal gammopathies
monoclonal gammopathies
Which patient with renal antiGBM disease is most likely to have lung hemorrhage?
a) younger patient
b) patient with Alport;s after renal transplantation
c) older patient
younger patient. Patients with Alport’s can form anti-GBM Abs after transplant and get crescentic GN (IgAN), but their lungs lack the antigen, so lung hemorrhage is NOT present.
Older patients have LESS lung hemorrhage
Which glomerulopathy is a/w smallest fibrils on EM?
a) Immunotactoid
b) fibrillary GN
c) cryoglobulinemia
d) amyloidosis
Amyloidosis
name a membranous GN antigen a/w cancer (bonus: name 2)
thrombospondin 7a
NELL1
what is the preferred class of antihypertension drugs for VEGF inhibitor induced hypertension?
calcium channel blockers
what is the typical histologic findings of warfarin nephropathy?
RBC casts
what type of AKI is caused by the ICPI, pembrolizumab?
acute interstitial nephritis
PD-1 inhibitors are thought to cause AIN through suppression of T cell immunity. It is more common in patients on PPI. Mgt: stop the ICPI and start steroids.
name a renal disease for which plasmapheresis is universally accepted?
anti-GBM disease
What needs to be monitored 10-14 days after giving cyclophosphamide?
Monitor CBC, to check for lymphopenia. If there is lymphopenia, CYC dose needs to be decrease
name 2 causes of cortical necrosis
- catastrophic APA
- TMA
- DIC
- Viper bite
- postpartum HUS
- Amniotic fluid embolism
- septic abortion
- placental abruption
name 2 causes of AKI in MM
- cast nephropathy
- acute uric acid nephropathy
- hypercaclcemia
- plasma cell infiltration of the kidneys
what are causes of diuretic resistance in nephrotic syndrome? (3)
- direct activation of ENaC by elevated levels of urinary plasmin (“overfill”)
- decreased intravascular volume from reduced oncotic pressure
- capillary permeability, leading to RAA activation (“underfill”).
what assay is used to help diagnose Fabry disease?
measure alpha-galactosidase activity.
What are the classic manifestations of Fabry disease?
neuropathy, skin telangiectasias, and corneal opacities.
What is the classic EM finding of Alport syndrome?
a lamellated appearance of the GBM
what is the role of IHC of skin biopsy in Alport syndrome?
Immunostaining for type IV collagen alpha chains
can confirm absence of alpha 5 type IV collagen in
X-linked Alport patients, mosaic patchy staining in
carrier females, and presence of alpha 5 type IV
collagen only in Bowman’s capsule and distal tubules but not in GBMs in patients with autosomal
recessive Alport
Acanthocytes __ % of total erthryocytes in the urine microscopy, are suggestive of glomerular disease
Acanthocytes > 5% the total erthryocytes
A uATPR on a spot urine of ___ mg/mg is associated with glomerular disease
A uATPR of > 0.6 mg/mg
What is normal RBCs/hpf in urine microscopy?
1-3 RBCs/hpf OR < 4 RBCs/hpf
Urine is red in color, + blood in the dipstick but negative for RBCs on microscopy.
Hemoglobinuria
What is the overall incidence of AIN among native kidney biopsies for whatever reason?
2-5%
What are the top 3 medications associated with drug induced AIN?
Antibiotics, NSAIDs, PPIs
