ASN QBank Pearls - Glomerular and Vascular Disorders Flashcards
have often been associated with increased risk for progression of diabetic
nephropathy
smoking and HTN
which genetic screening test is likely to detect a mutation in more than 5% of sporadic FSGS in adolescents?
mutations in NPHS2 (podocin) gene, including p.R229Q variant
possible kidney biopsy findings in MM nephropathy
- fractured tubular casts
- Congo red–positive glomerular and tubular deposits
- acute tubular injury/necrosis
- plasma cell infiltrates
amyloidosis fibril size
9 to 12 nm
cryoglobulinemic GN microtubule shape and size
- curved
- 25-35 nm
immunotactoid glomerulopathy microtubule pattern and size
- parallel arrays
- > 30 nm
fibrillary GN fibril pattern and size
- RANDOM
- 15-30 nm
lupus nephritis IG pattern
“fingerprint”
treatment for idiopathic MCD
- sodium and fluid restriction
- furosemide
- statins
- ACEI
- high-dose prednisone daily or on alternate days
spontaneous remission rate of nephrotic syndrome in iMCD
1/3, but may take up to 2 years or more
treatment of steroid-resistant FSGS
cyclosporine A often in combination with low-dose prednisone
lesion associated with highest response rate to treatment with steroids and has the BEST prognosis in nephrotic patients with FSGS
tip lesion
has not been shown to be effective treatment in patients with idiopathic membranous glomerulonephritis (iMGN)
prednisone alone
multiple myeloma with acute cast nephropathy clues
- very low anion gap
- hypercalcemia
- anemia
- presence of trace urine protein by dipstick
- elevated UPC on direct measurement
- protein electrophoresis shows IgG kappa paraprotein
HIVAN
collapsing FSGS
hepatitis B–induced membranous GN is usually associated with
hepatitis BeAg immune complexes in the subEPIthelial space
hepatitis BsAg-induced MPGN is usually associated with
subENDOthelial deposits due to the different sizes of molecules
hepatitis C leads to what type of cryoglobulinemia
type 2
hepatitis C leads to what type of MPGN
type 1 MPGN
cryoglobulins activate which complement pathway
classical complement pathway
HIV directly infects renal tissue including the
- podocytes
- mesangial cells, and
- renal tubular cells
associated renal lesion;
- hepatitis A
postinfectious GN (PIGN)
associated renal lesion;
- hepatitis C
MPGN
associated renal lesion;
- hepatitis B
membranous GN
HIVAN or collapsing FSGS is due to
active viral replication in the podocyte –> podocytes change to proliferative macrophages
treatment that may lead to an improvement in renal function and nephrotic syndrome in HIVAN
- HAART
- ACEI or ARB, and
- steroids
HIVAN predominantly diagnosed in patients of what race
black
HIVAN predominantly affects what sex
males > females
HIVAN RUS findings
echogenic, occasionally large-sized kidneys
treatment for crescentic type I membranoproliferative glomerulonephritis (MPGN) secondary to cryoglobulinemia with no evidence of chronicity
- initiation of HCV antiviral therapy
- plasmapheresis
- corticosteroids, and
- rituximab
NSAID use, AKI, and nephrotic syndrome
AIN and MCD
cause of leukocytoclastic skin lesions (can be on legs and ears), AKI, small vessel vasculitis
cocaine use
- leukocytoclastic rash
- nephritis
- and abnormal liver function tests
- undetectable C4
- low to normal C3
cryoglobulinemia
timeline of hematuria from PIGN
2 weeks after onset of infection
sacroiliitis is associated with
human leukocyte antibody B27 (HLAB27) and IgA nephropathy
associated with a high risk of relapse in pauci-immune necrotizing and crescentic glomerulonephritis (MPO-ANCA)
presence of upper respiratory tract disease (such as nasal crusting)
MOST likely to be decreased in MPGN, especially C3 glomerulopathy
factor H (normal inhibitor of the alternative complement pathway)
SLE and TMA, think of
anti-phospholipid antibodies
cryoglobulin is typically what immunoglobulin dominant
IgM
cryoglobulin pattern
short vague substructure or is microtubular
immunotactoid glomerulopathy (GP) pattern
- clonal staining
- microtubular substructure
fibrillary glomerulonephritis (GN) pattern
- polyclonal with proliferation
- randomly arranged fibrils
hallmarks of granulomatosis w/ polyangiitis
- older age
- sinusitis
- intermittent otitis media
- crusting in nose
- nodule on CXR
- dysmorphic rbcs
- AKI
- peripheral edema
- normal complements
auto-Ab in granulomatosis w/ polyangiitis
proteinase 3–positive anti–neutrophil cytoplasmic autoantibody
strongly associated with AR mutations in APOL1 gene in individuals of African ancestry
- FSGS
- HIVAN
- collapsing glomerulosclerosis a/w SLE
- SCD-associated nephropathy
best test with highest sensitivity and specificity to evaluate for IG-related disorders
nephelometric assay for serum free light chains
serum protein electrophoresis will miss cases due to its lower sensitivity
monoclonal diseases
serum immunofixation electrophoresis will miss diseases that only produce
lights chains
urine protein/immunofixation electrophoresis is not as sensitive as serum tests for
monoclonal proteins
bevacizumab MOA
MAB against VEGF (vascular endothelial growth factor)
HTN in setting of bevacizumab, renin and aldosterone levels
LOW
best treatment for HTN and proteinuria 2/2 bevacizumab as long as no TMA
dihydropyridine calcium channel blocker (CCB)
what does pamidronate nephrotoxicity cause?
collapsing FSGS and acute tubular injury
clinical scenario diagnostic of classical polyarteritis nodosa (PAN)
- severe HA
- visual changes
- fatigue
- unintentional weight loss
- fever
- HTN
- schistocytes on PBS
renal arteriogram findings in classical polyarteritis nodosa (PAN):- multiple aneurysms
- irregular constrictions in larger vessels
- occlusion of smaller penetrating arteries
clinical scenario consistent with TMA from gemcitabine
- edema
- livedo-like rash with erythematous ulcer
- HTN
- AKI
- anemia
- elevated LDH
- proteinuria
- hematuria
cause of “anticoagulant nephropathy”
warfarin (glomerular bleeding)
- kappa light chains
- nephrotic proteinuria
- nodular glomerulus, and
- granular deposition along BM and in nodule on EM are diagnostic of
LCDD
- lambda light chains
- nephrotic proteinuria
- nodular glomerulus, and
- fibrillar deposition in the nodule on EM are diagnostic of
renal AL amyloidosis
- vasculitic skin rash
- renal insufficiency
- hematuria
- nephrotic range proteinuria, and
- low C4 complement are diagnostic of
cryoglobulinemia type 2 leading to MPGN with immune deposits
diffuse podocyte effacement on EM and segmental scarring on light microscopy
FSGS
diffuse podocyte effacement on EM and a normal light microscopy
MCD
numerous subepithelial deposits on EM and spikes on silver stain
membranous nephropathy
10 nm fibrils detected on EM and mesangial expansion with amorphous material on light microscopy
amyloid
subendothelial deposits on EM and mesangial hypercellularity on light microscopy
membranoproliferative GN
- screening test for SLE w/ 95% sensitivity
- not diagnostic w/o other Abs or clinical features
ANA
- high specificity for SLE
- sensitivity 70%
anti-dsDNA Ab
- MOST specific Ab for SLE
- sensitivity only 30-40%
anti-Smith Ab
- present in 15% of patients w/ SLE
- seen w/ other connective tissue d/o’s, eg Sjogren syndrome
anti-Ro/SSA
