Secondary Glomerulonephritis Flashcards
(99 cards)
1
Q
Renal involvement in sle
A
persistent proteinuria > 500 mg/dL/day, 3+ on dipstick, cellular urinary casts
2
Q
Class of SLE: Minimal mesangial LN
A
Class I
3
Q
SLE class: Mesangial proliferative LN
A
Class II
4
Q
Focal LN
A
Class 3 (A/C)
5
Q
Diffuse LN
sclerotic or global
A
Class 4
6
Q
Membranous LN
A
Class V
7
Q
Advanced sclerosing LN
A
Class 6, > 90% globally sclerosed without residual activity
8
Q
wire loop lesions in sle and hyaline thrombi
A
class 3
9
Q
subepithelial deposits producing membranous patter
A
Class V
10
Q
IF in lupus nephritis
A
full house - IgG, IgA, IgM, C1q and C3
11
Q
features of activity index in sle
A
endocapillary hypercellularity glomerular neutrophil infiltration wire loop deposits fibrinoid necrosis cellular and or fibrocellular crescents interstitial inflammation
12
Q
most frequent vascular lesion in sle
A
vascular immune deposition - class 3/4
13
Q
most commin in active class IV
A
noninflammatory necrotizing vasculopathy - fibrinoid necrotizing lesion without leukocyte infiltration that narrows or occludes the arteriolar lumen
14
Q
neonatal lupus antibodies
A
maternal anti Ro
15
Q
antihistone antibodies in the absence of antidna antibodies
A
drug induced lupus
16
Q
most common cause of death in sle
A
cardiovascular
17
Q
first line therapy for severe ln
A
Cyclophosphamide or mmf
18
Q
maintenance therapy in sle
A
azathioprine or mmf
19
Q
antibodies associated with increased risk of thrombotic events in APS
A
B2 glycoprotein
20
Q
most common pattern of glomerular involvement in mctd
A
membranous nephropathy
21
Q
sle + scleroderma + polymyositis
A
Mctd
22
Q
small vessel vasculitis
A
pauci immune
23
Q
Wegeners granulomatosis
A
Granulomatosis with polyangitis
24
Q
Churg strauss syndrome
A
Eosinophilic granulomatosis with polyangiitis
25
vasculitis with necrotizing granulomatous inflammation of upper or lower respiratory tracts + gn
GPA
26
earliest lesion in gpa
intracapillary thrombosis with deposition of eosinophilic fibrinoid material
27
sunburst appearance due to massive circumferential destruction of Bowman’s capsule
mpa
28
3 phases of egpa
Allergic - eosinophilic - vasculitic
29
most common associated illness found in patients with classic pan
Hbv infection, hairy cell leukemia
30
systemic necrotizing vasculitis affecting medium sized muscular arteries producing focal aneurysm
PAN
31
segmental transmural inflammation of medium sized and large elastic arteries mixed infiltrates
temporal arteritis
32
Definitive diagnostic test temporal arteritis
temporal artery biopsy
33
rare hiant cell arteritis inflammation and stenosis of medium sized and large arteries
Takayasu arteritis
34
Predilection in takayasu arteritis
aortic arch
35
IgA vasculitis
hsp
36
classic tetrad of hsp
skin, gi, joint and gn
37
chronic inflammatory cell infiltration of the exocrine salivary and lacrimal gland associated with sicca complex of xerostomia and xerophthalmia
Sjogren syndome
38
extra cellular deposition of characteristic fibrils in various organs
amyloidosis
39
amyloidosis - overproduction of immunoglobulin light chains
AL amyloidosis
40
Amyloidosis- continuous overproduction of acute phase proteins in chronic inflammation
AA amyloidosis
41
most common pattern of monoclonal immunoglobulin disease
Light chain deposit disease
42
abnormal circulating monoclonal igm protein in association with b cell lymphoproliferative hematologic disorder
Waldenstorm macroglobulinemia
43
associated with hcv, collagen disease
Mixed cryoglobulinemia
44
variant of alport syndrome with hereditary nephritis, thrombocytopathia, diffuse leimyotosis, icthysosis and hyperprolinuria
Epstein syndrome
45
alport syndrome + nephritis + macrothrombocytopenia + Dohle like inclusion + deafness and cataract
Fechtner syndrome
46
benign familial hematuria and thin gbm nephropathy
Thin basement membrane
47
classic tetrad if nails, elbows, knees and iliac horns anomalies
nail patella syndrome
48
pathognomonic of nps
triangular lunulae
49
moth eaten appearance of thickened basement membrane
nail patella syndrome
50
xlinked inborn error of glycosphingolipid metabolism involving a lysosomal enzyme a-galactosidase A
Fabry disease
51
major em finding in fabry disease
myelin figures or zebra bodies
52
most common organism ptr antibiotic era infection related gn
strep viridans
53
predominant Ig deposited in shunt nephritis
IgM
54
schistosoma causing cystitis
haematobium
55
most common pattern of immune complex mediated gn seen in hiv
mpgn
56
hep c
mpgn with or without assoc mixed crypglobulinemia and membranous
57
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
58
reversible inhibitor of inosine monophosphate dehydrogenase required for purine synthesis and blocks b and t cell proliferation
MMF
59
prophylaxis of asymptomatic patients with antiphospholipid antibodies
Low dose aspirin and hcq
60
Sle with clinical thrombotic event, anticoagulation of choice
warfarin
61
target inr for warfarin treatment in antiphospholipid syndrome
Greater than 3
62
most widely used serologic test to confirm a diagnosis of mctd
Ena with Anti U1RNP
63
most common pattern of glomerular involvement in mctd
membranous nephropathy with typical peripheral capillary wall granular IF staining for igg c3 iga and igm
64
nervous system involvement in GPA
mononeuritis multiplex
65
P anca positive has antibodies directed against
MPO
66
C anca positive antibody directed against
pr3
67
treatment of choice for gpa and other pauci immune rpgn
cyclophosphamide
68
primary treatment for egpa with mild disease
corticosteroids
69
associated with drug abuse with amphetamines
polyarteritis nodosa
70
primary amyloidosis
AL amyloidosis
71
treatment of midd
melphalan and corticosteroids
72
Type of mixed crypglobinemia: single monoclonal immunoglobulin found with Waldernstroms macroglobulinemia or myeloma
Type 1
73
monoclonal immunoglobulin directed against polyclonal IgG and has rheumatoid factor activity
Type II
74
mixed cryoglobulinemia where the antiglobulin is polyclonal in nature with both polyclonal IgG and IgM
Type 3
75
Angiokeratoma Corporus Diffusum Universale
Fabry’s Disease
76
enlarged podocytes, uniform vacuoles causing a foamy appearance
Fabry Diseade
77
Diagnosis for fabry disease based on
A galactosidase A in plasma blood
78
Treatment of Fabry Disease
Recombinant Enzyme replacement therapy
79
rare disease with insulin resistance where there is loss of fat
lipodystrophy
80
anemia + nephrotic syndrome + corneal opacities
LCAT deficiency
81
CLL
Membranoproliferative GN
82
Ca of the stomach pancreas or prostate
HUS
83
most common feature of apas
Dvt
84
intracellular branching tubular structures measuring 24 nm with dilated cisternae of the ER of glomerular and vascular endothelial cells
tubuloreticular inclusions
85
3 tests in apl that when positive states higher risjs for thromboembolic events
Lupus anticoagulant, anticardiolipin antibodies, B2 glycoprotein antibodies
86
widely accepted most effective agent in PAN
Cyclophosphamide
87
most vommin vascultiis of childhood
Hsp
88
mesangial and endocapillaty prolifetatice gn with variable crescent formation
HSP
89
a chronic active interstitial inflammation by a predominantly lymphocytic infiltrate admixed with plasma cells, with variable interstitial fibrosis and tubular atrophy
Sjogren Syndrome
90
EM: characteristic spicular, cockscomb-like projections along the subepithelial aspect of the GBMs.
amyloidosis
91
treatment of amyloidosis
melphalan, lenalidomide, thalidomide, bortezomib, and cyclophosphamide + steroids
92
an autosomal recessive disease caused by pyrin mutation primarily found in Sephardic Jews which lead to the development of amyloidosis
Familial Mediterranean fever
93
cardinal structural abnormality in Alport Syndrome
the variable thickening, thinning, basket weaving, and lam¬ellation of the GBM
94
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
95
deficiency of a-galactosidase in Fabry disease leads to accumulation of
globotriaosylceramide (ceramide trihexoside)
96
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
97
presence of lami¬nated thrombi consisting of lipids within the lumina of dilated glomerular capillaries
lipoprotein glomerulopathy
98
standard therapy for HCV
ribavirin and pegylated interferon
99
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)
