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Nephrology Fellowship Reviewer > Secondary Glomerulonephritis > Flashcards

Secondary Glomerulonephritis Flashcards

1
Q

Renal involvement in sle

A

persistent proteinuria > 500 mg/dL/day, 3+ on dipstick, cellular urinary casts

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2
Q

Class of SLE: Minimal mesangial LN

A

Class I

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3
Q

SLE class: Mesangial proliferative LN

A

Class II

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4
Q

Focal LN

A

Class 3 (A/C)

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5
Q

Diffuse LN

sclerotic or global

A

Class 4

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6
Q

Membranous LN

A

Class V

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7
Q

Advanced sclerosing LN

A

Class 6, > 90% globally sclerosed without residual activity

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8
Q

wire loop lesions in sle and hyaline thrombi

A

class 3

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9
Q

subepithelial deposits producing membranous patter

A

Class V

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10
Q

IF in lupus nephritis

A

full house - IgG, IgA, IgM, C1q and C3

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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
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12
Q

most frequent vascular lesion in sle

A

vascular immune deposition - class 3/4

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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

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14
Q

neonatal lupus antibodies

A

maternal anti Ro

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15
Q

antihistone antibodies in the absence of antidna antibodies

A

drug induced lupus

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16
Q

most common cause of death in sle

A

cardiovascular

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17
Q

first line therapy for severe ln

A

Cyclophosphamide or mmf

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18
Q

maintenance therapy in sle

A

azathioprine or mmf

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19
Q

antibodies associated with increased risk of thrombotic events in APS

A

B2 glycoprotein

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20
Q

most common pattern of glomerular involvement in mctd

A

membranous nephropathy

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21
Q

sle + scleroderma + polymyositis

A

Mctd

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22
Q

small vessel vasculitis

A

pauci immune

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23
Q

Wegeners granulomatosis

A

Granulomatosis with polyangitis

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24
Q

Churg strauss syndrome

A

Eosinophilic granulomatosis with polyangiitis

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25
Q

vasculitis with necrotizing granulomatous inflammation of upper or lower respiratory tracts + gn

A

GPA

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26
Q

earliest lesion in gpa

A

intracapillary thrombosis with deposition of eosinophilic fibrinoid material

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27
Q

sunburst appearance due to massive circumferential destruction of Bowman’s capsule

A

mpa

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28
Q

3 phases of egpa

A

Allergic - eosinophilic - vasculitic

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29
Q

most common associated illness found in patients with classic pan

A

Hbv infection, hairy cell leukemia

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30
Q

systemic necrotizing vasculitis affecting medium sized muscular arteries producing focal aneurysm

A

PAN

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31
Q

segmental transmural inflammation of medium sized and large elastic arteries mixed infiltrates

A

temporal arteritis

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32
Q

Definitive diagnostic test temporal arteritis

A

temporal artery biopsy

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33
Q

rare hiant cell arteritis inflammation and stenosis of medium sized and large arteries

A

Takayasu arteritis

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34
Q

Predilection in takayasu arteritis

A

aortic arch

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35
Q

IgA vasculitis

A

hsp

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36
Q

classic tetrad of hsp

A

skin, gi, joint and gn

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37
Q

chronic inflammatory cell infiltration of the exocrine salivary and lacrimal gland associated with sicca complex of xerostomia and xerophthalmia

A

Sjogren syndome

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38
Q

extra cellular deposition of characteristic fibrils in various organs

A

amyloidosis

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39
Q

amyloidosis - overproduction of immunoglobulin light chains

A

AL amyloidosis

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40
Q

Amyloidosis- continuous overproduction of acute phase proteins in chronic inflammation

A

AA amyloidosis

41
Q

most common pattern of monoclonal immunoglobulin disease

A

Light chain deposit disease

42
Q

abnormal circulating monoclonal igm protein in association with b cell lymphoproliferative hematologic disorder

A

Waldenstorm macroglobulinemia

43
Q

associated with hcv, collagen disease

A

Mixed cryoglobulinemia

44
Q

variant of alport syndrome with hereditary nephritis, thrombocytopathia, diffuse leimyotosis, icthysosis and hyperprolinuria

A

Epstein syndrome

45
Q

alport syndrome + nephritis + macrothrombocytopenia + Dohle like inclusion + deafness and cataract

A

Fechtner syndrome

46
Q

benign familial hematuria and thin gbm nephropathy

A

Thin basement membrane

47
Q

classic tetrad if nails, elbows, knees and iliac horns anomalies

A

nail patella syndrome

48
Q

pathognomonic of nps

A

triangular lunulae

49
Q

moth eaten appearance of thickened basement membrane

A

nail patella syndrome

50
Q

xlinked inborn error of glycosphingolipid metabolism involving a lysosomal enzyme a-galactosidase A

A

Fabry disease

51
Q

major em finding in fabry disease

A

myelin figures or zebra bodies

52
Q

most common organism ptr antibiotic era infection related gn

A

strep viridans

53
Q

predominant Ig deposited in shunt nephritis

A

IgM

54
Q

schistosoma causing cystitis

A

haematobium

55
Q

most common pattern of immune complex mediated gn seen in hiv

A

mpgn

56
Q

hep c

A

mpgn with or without assoc mixed crypglobulinemia and membranous

57
Q

common regimen for sle dose and duration

A

1 mld prednisone, 4-6 weeks, 30 mg/day or less by end of 3 months of therapy

58
Q

reversible inhibitor of inosine monophosphate dehydrogenase required for purine synthesis and blocks b and t cell proliferation

A

MMF

59
Q

prophylaxis of asymptomatic patients with antiphospholipid antibodies

A

Low dose aspirin and hcq

60
Q

Sle with clinical thrombotic event, anticoagulation of choice

A

warfarin

61
Q

target inr for warfarin treatment in antiphospholipid syndrome

A

Greater than 3

62
Q

most widely used serologic test to confirm a diagnosis of mctd

A

Ena with Anti U1RNP

63
Q

most common pattern of glomerular involvement in mctd

A

membranous nephropathy with typical peripheral capillary wall granular IF staining for igg c3 iga and igm

64
Q

nervous system involvement in GPA

A

mononeuritis multiplex

65
Q

P anca positive has antibodies directed against

A

MPO

66
Q

C anca positive antibody directed against

A

pr3

67
Q

treatment of choice for gpa and other pauci immune rpgn

A

cyclophosphamide

68
Q

primary treatment for egpa with mild disease

A

corticosteroids

69
Q

associated with drug abuse with amphetamines

A

polyarteritis nodosa

70
Q

primary amyloidosis

A

AL amyloidosis

71
Q

treatment of midd

A

melphalan and corticosteroids

72
Q

Type of mixed crypglobinemia: single monoclonal immunoglobulin found with Waldernstroms macroglobulinemia or myeloma

A

Type 1

73
Q

monoclonal immunoglobulin directed against polyclonal IgG and has rheumatoid factor activity

A

Type II

74
Q

mixed cryoglobulinemia where the antiglobulin is polyclonal in nature with both polyclonal IgG and IgM

A

Type 3

75
Q

Angiokeratoma Corporus Diffusum Universale

A

Fabry’s Disease

76
Q

enlarged podocytes, uniform vacuoles causing a foamy appearance

A

Fabry Diseade

77
Q

Diagnosis for fabry disease based on

A

A galactosidase A in plasma blood

78
Q

Treatment of Fabry Disease

A

Recombinant Enzyme replacement therapy

79
Q

rare disease with insulin resistance where there is loss of fat

A

lipodystrophy

80
Q

anemia + nephrotic syndrome + corneal opacities

A

LCAT deficiency

81
Q

CLL

A

Membranoproliferative GN

82
Q

Ca of the stomach pancreas or prostate

A

HUS

83
Q

most common feature of apas

A

Dvt

84
Q

intracellular branching tubular structures measuring 24 nm with dilated cisternae of the ER of glomerular and vascular endothelial cells

A

tubuloreticular inclusions

85
Q

3 tests in apl that when positive states higher risjs for thromboembolic events

A

Lupus anticoagulant, anticardiolipin antibodies, B2 glycoprotein antibodies

86
Q

widely accepted most effective agent in PAN

A

Cyclophosphamide

87
Q

most vommin vascultiis of childhood

A

Hsp

88
Q

mesangial and endocapillaty prolifetatice gn with variable crescent formation

A

HSP

89
Q

a chronic active interstitial inflammation by a predominantly lymphocytic infiltrate admixed with plasma cells, with variable interstitial fibrosis and tubular atrophy

A

Sjogren Syndrome

90
Q

EM: characteristic spicular, cockscomb-like projections along the subepithelial aspect of the GBMs.

A

amyloidosis

91
Q

treatment of amyloidosis

A

melphalan, lenalidomide, thalidomide, bortezomib, and cyclophosphamide + steroids

92
Q

an autosomal recessive disease caused by pyrin mutation primarily found in Sephardic Jews which lead to the development of amyloidosis

A

Familial Mediterranean fever

93
Q

cardinal structural abnormality in Alport Syndrome

A

the variable thickening, thinning, basket weaving, and lam¬ellation of the GBM

94
Q

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.

A

nail patella syndrome

95
Q

deficiency of a-galactosidase in Fabry disease leads to accumulation of

A

globotriaosylceramide (ceramide trihexoside)

96
Q

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

A

lipodystrophy

97
Q

presence of lami¬nated thrombi consisting of lipids within the lumina of dilated glomerular capillaries

A

lipoprotein glomerulopathy

98
Q

standard therapy for HCV

A

ribavirin and pegylated interferon

99
Q

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.

A

Kimura’s disease and angiolymphoid hyperplasia with eosinophilia (ALHE)

Nephrology Fellowship Reviewer (29 decks)

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