Secondary Glomerulonephritis Flashcards
Renal involvement in sle
persistent proteinuria > 500 mg/dL/day, 3+ on dipstick, cellular urinary casts
Class of SLE: Minimal mesangial LN
Class I
SLE class: Mesangial proliferative LN
Class II
Focal LN
Class 3 (A/C)
Diffuse LN
sclerotic or global
Class 4
Membranous LN
Class V
Advanced sclerosing LN
Class 6, > 90% globally sclerosed without residual activity
wire loop lesions in sle and hyaline thrombi
class 3
subepithelial deposits producing membranous patter
Class V
IF in lupus nephritis
full house - IgG, IgA, IgM, C1q and C3
features of activity index in sle
endocapillary hypercellularity glomerular neutrophil infiltration wire loop deposits fibrinoid necrosis cellular and or fibrocellular crescents interstitial inflammation
most frequent vascular lesion in sle
vascular immune deposition - class 3/4
most commin in active class IV
noninflammatory necrotizing vasculopathy - fibrinoid necrotizing lesion without leukocyte infiltration that narrows or occludes the arteriolar lumen
neonatal lupus antibodies
maternal anti Ro
antihistone antibodies in the absence of antidna antibodies
drug induced lupus
most common cause of death in sle
cardiovascular
first line therapy for severe ln
Cyclophosphamide or mmf
maintenance therapy in sle
azathioprine or mmf
antibodies associated with increased risk of thrombotic events in APS
B2 glycoprotein
most common pattern of glomerular involvement in mctd
membranous nephropathy
sle + scleroderma + polymyositis
Mctd
small vessel vasculitis
pauci immune
Wegeners granulomatosis
Granulomatosis with polyangitis
Churg strauss syndrome
Eosinophilic granulomatosis with polyangiitis
vasculitis with necrotizing granulomatous inflammation of upper or lower respiratory tracts + gn
GPA
earliest lesion in gpa
intracapillary thrombosis with deposition of eosinophilic fibrinoid material
sunburst appearance due to massive circumferential destruction of Bowman’s capsule
mpa
3 phases of egpa
Allergic - eosinophilic - vasculitic
most common associated illness found in patients with classic pan
Hbv infection, hairy cell leukemia
systemic necrotizing vasculitis affecting medium sized muscular arteries producing focal aneurysm
PAN
segmental transmural inflammation of medium sized and large elastic arteries mixed infiltrates
temporal arteritis
Definitive diagnostic test temporal arteritis
temporal artery biopsy
rare hiant cell arteritis inflammation and stenosis of medium sized and large arteries
Takayasu arteritis
Predilection in takayasu arteritis
aortic arch
IgA vasculitis
hsp
classic tetrad of hsp
skin, gi, joint and gn
chronic inflammatory cell infiltration of the exocrine salivary and lacrimal gland associated with sicca complex of xerostomia and xerophthalmia
Sjogren syndome
extra cellular deposition of characteristic fibrils in various organs
amyloidosis
amyloidosis - overproduction of immunoglobulin light chains
AL amyloidosis
Amyloidosis- continuous overproduction of acute phase proteins in chronic inflammation
AA amyloidosis
most common pattern of monoclonal immunoglobulin disease
Light chain deposit disease
abnormal circulating monoclonal igm protein in association with b cell lymphoproliferative hematologic disorder
Waldenstorm macroglobulinemia
associated with hcv, collagen disease
Mixed cryoglobulinemia
variant of alport syndrome with hereditary nephritis, thrombocytopathia, diffuse leimyotosis, icthysosis and hyperprolinuria
Epstein syndrome
alport syndrome + nephritis + macrothrombocytopenia + Dohle like inclusion + deafness and cataract
Fechtner syndrome
benign familial hematuria and thin gbm nephropathy
Thin basement membrane
classic tetrad if nails, elbows, knees and iliac horns anomalies
nail patella syndrome
pathognomonic of nps
triangular lunulae
moth eaten appearance of thickened basement membrane
nail patella syndrome
xlinked inborn error of glycosphingolipid metabolism involving a lysosomal enzyme a-galactosidase A
Fabry disease
major em finding in fabry disease
myelin figures or zebra bodies
most common organism ptr antibiotic era infection related gn
strep viridans
predominant Ig deposited in shunt nephritis
IgM
schistosoma causing cystitis
haematobium
most common pattern of immune complex mediated gn seen in hiv
mpgn
hep c
mpgn with or without assoc mixed crypglobulinemia and membranous
common regimen for sle dose and duration
1 mld prednisone, 4-6 weeks, 30 mg/day or less by end of 3 months of therapy
reversible inhibitor of inosine monophosphate dehydrogenase required for purine synthesis and blocks b and t cell proliferation
MMF
prophylaxis of asymptomatic patients with antiphospholipid antibodies
Low dose aspirin and hcq
Sle with clinical thrombotic event, anticoagulation of choice
warfarin
target inr for warfarin treatment in antiphospholipid syndrome
Greater than 3
most widely used serologic test to confirm a diagnosis of mctd
Ena with Anti U1RNP
most common pattern of glomerular involvement in mctd
membranous nephropathy with typical peripheral capillary wall granular IF staining for igg c3 iga and igm
nervous system involvement in GPA
mononeuritis multiplex
P anca positive has antibodies directed against
MPO
C anca positive antibody directed against
pr3
treatment of choice for gpa and other pauci immune rpgn
cyclophosphamide
primary treatment for egpa with mild disease
corticosteroids
associated with drug abuse with amphetamines
polyarteritis nodosa
primary amyloidosis
AL amyloidosis
treatment of midd
melphalan and corticosteroids
Type of mixed crypglobinemia: single monoclonal immunoglobulin found with Waldernstroms macroglobulinemia or myeloma
Type 1
monoclonal immunoglobulin directed against polyclonal IgG and has rheumatoid factor activity
Type II
mixed cryoglobulinemia where the antiglobulin is polyclonal in nature with both polyclonal IgG and IgM
Type 3
Angiokeratoma Corporus Diffusum Universale
Fabry’s Disease
enlarged podocytes, uniform vacuoles causing a foamy appearance
Fabry Diseade
Diagnosis for fabry disease based on
A galactosidase A in plasma blood
Treatment of Fabry Disease
Recombinant Enzyme replacement therapy
rare disease with insulin resistance where there is loss of fat
lipodystrophy
anemia + nephrotic syndrome + corneal opacities
LCAT deficiency
CLL
Membranoproliferative GN
Ca of the stomach pancreas or prostate
HUS
most common feature of apas
Dvt
intracellular branching tubular structures measuring 24 nm with dilated cisternae of the ER of glomerular and vascular endothelial cells
tubuloreticular inclusions
3 tests in apl that when positive states higher risjs for thromboembolic events
Lupus anticoagulant, anticardiolipin antibodies, B2 glycoprotein antibodies
widely accepted most effective agent in PAN
Cyclophosphamide
most vommin vascultiis of childhood
Hsp
mesangial and endocapillaty prolifetatice gn with variable crescent formation
HSP
a chronic active interstitial inflammation by a predominantly lymphocytic infiltrate admixed with plasma cells, with variable interstitial fibrosis and tubular atrophy
Sjogren Syndrome
EM: characteristic spicular, cockscomb-like projections along the subepithelial aspect of the GBMs.
amyloidosis
treatment of amyloidosis
melphalan, lenalidomide, thalidomide, bortezomib, and cyclophosphamide + steroids
an autosomal recessive disease caused by pyrin mutation primarily found in Sephardic Jews which lead to the development of amyloidosis
Familial Mediterranean fever
cardinal structural abnormality in Alport Syndrome
the variable thickening, thinning, basket weaving, and lam¬ellation of the GBM
Genetic locus for this syndrome is on chromosome 9 and results from mutations in the LIM homeodomain protein LMX1B gene, which is transmitted in an autosomal dominant pattern.
nail patella syndrome
deficiency of a-galactosidase in Fabry disease leads to accumulation of
globotriaosylceramide (ceramide trihexoside)
tall stature, muscular hypertrophy, hirsutism, macroglossia, abdominal distension, subcutaneous nodules, acanthosis nigricans, hepatomegaly, cirrhosis, clitoral or penile enlarge¬ment, febrile adenopathy, cerebral atrophy, cerebral ventricular dilation, hemiplegia, mental retardation, and cardiomegaly
lipodystrophy
presence of lami¬nated thrombi consisting of lipids within the lumina of dilated glomerular capillaries
lipoprotein glomerulopathy
standard therapy for HCV
ribavirin and pegylated interferon
produce skin lesions that appear as single or multiple red-brown papules or as subcutaneous nodules with a predilection for the head and neck region with eosinophilia and elevated IgE.
Kimura’s disease and angiolymphoid hyperplasia with eosinophilia (ALHE)