Primary Glomerulonephritis Flashcards
3 layers of glomerular capillary wall
Endothelial cells, GBM, Epithelial cells
the last barrier resistance encounteted by water and filtered solute
epithelial filtration slit
dominant infiltrating cells in early and late chronic renal injury
macrophages
second most abundantly excreted protein
Immunoglobulin
most common pathogen in nephrotic syndrome
Strep pneumonia and E coli
mild transient proteinuria of less than 1g per day in stressful conditions
isolated proteinuria
absence of proteinuria in recumbent, less than 1-2 g protein during upright posture
orthostatic proteinuria
diagnosis of orthostatic proteinuria in split collection 16h vs 8h recumbent
protein during recumbency <50 mg
normal mesangiun contain about number of mesangial cells
2 to 4
effacemebt of podocyte foot processes
minimal change disease
Dose of prednisone for adult patients witg mcd and duration
1 mg/kg not to exceed 80 mg per day, 15 weeks
predilection for sclerosis in the perihilar regions with hyalinosis, obesity
FSGS
segmental consolidation confined to the segment adjacent to the origin of the proximal tubule
Glomerular tip variant
Segmental collapse of capillaries with hypertrophy and hyperplasia of overlying podocytes and accumulation of protein resorption droplets
Collapsing variant
Most aggressive type of FSGS
collapsing variant
Presence of mesangial immune complex deposuts with staining for C1q in a patient with no clinical and lab evidence for sle
C1q Nephropathy
Membranous nephropathy caused by antibodies specific for what antibodies?
M type Phospholipase A2 receptor
pathologic sine qua non of membranous nephropathy
subepithelial immune complex deposits
mesangial dense deposits in MN
secondary MN
characteristic IF finding in MN
diffuse global granular capillary wall staining
most frequent and most intense IF staining in MN
IgG, IgG4
characteristic histologic abnormality by light microscopy of MN
diffuse global capillary wall thickening in absence of significant glomerular hypercellularity
Caused by immune complex localization in the subepithelial zone of glomerular capillaries
Membranous Nephropathy
2 categories of MPGN
Immune complex mpgn and c3 glomerulopatht (alternative complement pathway)
Type 2 MPGN
dense deposit disease
tran tracking/basement membrane reduplication
MPGN Type 1
lobular glomerulonephritis
Mpgn type 1
aggregates of immune complexes filling capillary lumens possibility of crypglobulinemia or lupus in mpgn
hyaline thrombi
characteristic pattern of if in MpGN
peripheral granular to bandlike staining for c3
mesangial interposition into an expanded subendothelial zone that contain electron dense immune complex deposits
MPGN type 1
Type of MPGN with persistent depression of C3 and presence of nephritic syndrome
Type 1 MPGN
Very rare primary immunodeficiency characterized by clinical triad of high serum ige, recurring staph skin abscesses and recurrent pneumonia with pneumatocoeles
buckley syndrome
resembles type 1 Mpgn but with irregularly thickened gbms with numerous intramembranous deposits
mpgn type 3
mesangiocapillary gn
mpgn type 1
discontinuous electron dense bands within the gbm
dense deposit disease
intense capillary wall linear to bandlike staining for C3
dense deposit disease
most common mechanism in uncontrolled activation of C3 convertase
presence of C3NeF
psgn associated with skin infections
epidemic psgn
psgn with pharyngitides
sporadic psgn
IF pattern, numerous large, closely apposed, granular deposits along capillary walls; nephrotic range proteinuria
Garland Pattern
IF pattern, psgn
More scattered granular staining less severe disease
Starry sky pattern
iF pattern, psgn, resolving phase with a mesangioproloferative light microscopic appearance
Mesangial pattern
hallmark ultrastructural feature of psgn
subepithelial humplike dense deposits
deposition predominantly of IgA in the mesangium with mesangial proliferation
IgA nephropathy
ubiquitous ultrastructural finding of IgA in em
Mesangial electron dense deposits
focal or diffuse mesangial hyper cellularity without more complex endo capillary hypercellularity
Iga nephropathy
four parameters predictive of clinical outcomes in IgA nephropathy
MEST mesangial hypercellularity endocapillary hypercellularity Segmental glomerulosclerosis tubular atrophy and interstial fibrosisb
in MEST, not significantly predictive of the rate of decline of kidney function or survival from eskd
endo capillary hypercellularity
size fibrils in fibrillary gn
20 nm
size of microtubular structures jn immunotactoid gn
30-40 nm
associated with lymphoproliferative disease and monoclonal gammopathy
fibrillary/immunotactoid gn
most common cause of rpgn in children
immune complex crescentic gn
least frequent cause of crescentic gn
Anti-GBM
Linear GBM IF staining + lung hemorrhage
good pasture syndrome
Anca disease + vasculitis with no granuloma or asthma
microscopic polyangitis
Vasculitis with granuloma no asthma
granulomatosis with polyangitis
Vasculitis with granulomas and asthma
Eosinophilic grabulomatosis with polyangitis
hallmark ultrastructural finding in immune complex mediated gn
immune complex type electron dense deposit
proliferative crescentic gn + pulmonary hemorrhage + anti gbm antibodies
good pasture syndrome
anca in anti-gbm
mpo-anca
most commin extrarenal findings in anti-gbm
pulmo ary
most common biopsy in antigbm
diffuse crescentic gn with more than 50% of glomeruli with exuberant circumferential crescents
Intense and diffuse linear staining for igG
Anti-GBM
major prognostic marker for progression to eskd in anti-gbm
serum crea at time of tx
major pathogenic factor for pauci immune crescentic gn
anca igg
endothelial tubuloreticular inclusions seen in
HIV
most frequent genetic cause of steroid resistant nephrotic syndrome
NPHs2 podocin
strongest indicators of progressive disease in MN
persistence of moderate proteinuria
Dose of cyclophosphamide for mcd and duration
2 mk for 8-12 weeks
Dose of cyclosporine for mcd
5 mk
No of glomeruli for adequate diagnosis
glomerular - 5
tubulointerstitial 6-10
transplant: 7
Ponticelli protocol
Methlypred 1g/day for the 1st 3 days of each month with daily oral methylpred 0.4 mkd, prednisone 0.5 mkd alternating monthly with chlorambucil 0.2 mkd
when to use ponticelli protocol
Moderate (4 and 6g protein) or high risk (>8g protein)
deposits in the retina Along brusch membrane
Dense deposit disease
latent period in post pharyngitic cases in psgn
14 to 21 days
Synpharingitic episode latency
less than 1 week
latency period of psgn after skin infection
14 to 21 days
Tx of pauci immune crescentic gn
steroids + cyclophosphamide
mppt 7mkd x 3 days; pred 1 mkd 1st month, dc 4th-5th; cyclophosphamide 0.5 g/m2
most impt determinant of patient survival
pulmonary hemorrhage
most impt predictor of outcome with respect to eskd in pauci immune
crea at time of initiation of tx
Earliest lesions are intracapillary thrombosis with deposition of eosinophilic fibrinoid material with endothelial cell swelling
Granulomatosis with polyangitis
Medication in gpa as induction only if disease is milld and controllable
methotrexate
TNF a blocker in gpa with 80% remission rate but high rate of infections
Infliximab
anti CD52 monoclonal antibody for gpa
alemtuzumab
vasculitis induced by ptu
Mpa
reduction to less than 3.5 g/day protein and 50% decrease from peak proteinuria
Partial remission
cytotoxic agent acts largely through alkylation of purine bases
cyclophosphamide
inhibitor of inosine monophosphate dehydrogenase
Azathioprine
reversible inhibitor of inosine monophosphate dehydrogenase
mycophenolate acid, mycophenolate mofetil
adverse effect of craniofacial malformations
MMF
anti C5 monoclonal antibody
Eculizumab
