Secondary Glomerulonephritis Flashcards by fernanda moraes

Renal involvement in sle

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

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Class of SLE:

Class I Minimal mesangial LN

Class II Mesangial proliferative LN

Focal LN Class 3 (A/C)

Diffuse LN Focal LN Class 3 (A/C)

Membranous LN Class V

Advanced sclerosing LN Class 6, > 90% globally sclerosed without residual activity

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IF in lupus nephritis

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

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features of activity index in sle

endocapillary hypercellularity
glomerular neutrophil infiltration
wire loop deposits
fibrinoid necrosis
cellular and or fibrocellular crescents
interstitial inflammation

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most frequent vascular lesion in sle

vascular immune deposition - class 3/4

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most commin in active class IV

noninflammatory necrotizing vasculopathy - fibrinoid necrotizing lesion without leukocyte infiltration that narrows or occludes the arteriolar lumen

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neonatal lupus antibodies

maternal anti Ro

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antihistone antibodies in the absence of antidna antibodies

drug induced lupus

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most common cause of death in sle

cardiovascular

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first line therapy for severe ln

Cyclophosphamide or mmf

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maintenance therapy in sle

azathioprine or mmf

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antibodies associated with increased risk of thrombotic events in APS

B2 glycoprotein

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most common pattern of glomerular involvement in mctd

membranous nephropathy

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sle + scleroderma + polymyositis

Mctd

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small vessel vasculitis

pauci immune

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Wegeners granulomatosis

Granulomatosis with polyangitis

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Churg strauss syndrome

Eosinophilic granulomatosis with polyangiitis

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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

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

mpa

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3 phases of egpa

Allergic - eosinophilic - vasculitic

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most common associated illness found in patients with classic pan

Hbv infection, hairy cell leukemia

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systemic necrotizing vasculitis affecting medium sized muscular arteries producing focal aneurysm

PAN

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

temporal arteritis

diagn=temporal artery biopsy

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

predilecao pelo aortic arch

Takayasu arteritis

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IgA vasculitis

hsp tetrade =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

benign familial hematuria and thin gbm nephropathy

Thin basement membrane

classic tetrad if nails, elbows, knees and iliac horns anomalies patognomonico 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 major em finding in fabry disease=myelin figures or zebra bodies

Fabry disease

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 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

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 enlarged podocytes, uniform vacuoles causing a foamy appearance

Fabry’s Disease

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

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

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)

primary amyloidosis

AL amyloidosis

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

treatment of amyloidosis

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

AMILOIDOSE AL

expansao mesangial amorfa, acelular, rosa palido, augodao doce, com spikes longos ocasionais na mbg na prata e acometimento da arteria e arteriola frequente positivo para vermelho do congo fibrilas de 9-11 nm na EM randomica monoclonal, smudgy , cadeia leve corado geralmente lambda cadeia leve