Lupus Pathophysiology Flashcards

1
Q

Define lupus erthemateux

A

illness in a patient with manifestations occuring in the skin

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2
Q

SLE is?

A

Major collagen vascular multisystem disease

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3
Q

SLE hallmark is?

A

development of autoantibodies to cellular nuclear components that leads to a chronic inflammatory autoimmune disease

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4
Q

SLE diagnosis requirements?

Test question

A

If 4 or more of the 11 criteria are documented, diagnosis can be made with 95% specificity and 85% sensitivty

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5
Q

SLE affects who?

A

Young adult woman

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6
Q

Genetic involvement in SLE

A

FOUR susceptibility genes are required for the expression of lupus
First degree relatives are about 20 times more likely
24&-58% concordance rate among identical twins

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7
Q

Targeted environmental agents that may induce SLE

A

Sunlight
Chemicals (hydrazine or aromatic amines)
Infxn (epstein barr virus)

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8
Q

Androgen and esterogen?

A

Androgen inhibits autoimmunity

Estrogen enhances autoimmunity

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9
Q

SLE Pathophysiology

A

Fluctuating multisystem disease with a clinical syndrome
Large spectrum of symptoms and organ involvement
Develop antibodies

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10
Q

Major event in the development of SLE?

A

Excessive autoantibody produces and the formation of immune complexes

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11
Q

What causes excessive autoantibodies?

A

Hyperactive B lymphocytes –> loss of immune self-tolerance and high atigenic load

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12
Q

Define antinuclear antibodies

A

Autoantibodies directed againsts nuclear constituents of the cell

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13
Q

Which antibodies are specific to SLE?

A

dsDNA antibodies

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14
Q

What are the four RNA associated antigens?

A

Smith
Small nuclear ribonucleoprotein
Ro
La

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15
Q

Two other against which antibodies can be formed?

A

Histone
Phospholipid moiety of prothrombin activator complex
Cardiolipin

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16
Q

Antiphospholipid antibodies

A

Lupus anticoagulant &

Anticardiolipin antibodies

17
Q

What precedes the diagnosis of SLE by years?

A

Antinuclear
Anti-La
Anti-Ro
Antiphospholipid ab

18
Q

What precedes SLE by months

A

Anti-Sm and anti-snRNP

19
Q

Clinical Presentation of SLE

A

Major and minor joints with short durations (stiffneff, pain, inflammation)
Butterfly rash (sun exposure)
Filtrates
Pericarditis

20
Q

Increase in coronary artery disease = increase in

A

SLE patients life expectancy

21
Q

Lupus nephritis

A

A poorer outcome association and increased risk of morbidity/mortality

22
Q

Six stages of the disease

A
Minimal Mesangial
Mesangial Proliferative
Focal Lupus Nephritis
Diffuse Proliferative Lupus Nephritis
Membranous Lupus Nephritis
Advance sclerosing Lupus nephritis (global sclerosis)
23
Q

SLE Diagnosis

A

Fluorescent antinuclear antibody test (ANA)

24
Q

ANA test

Test question

A

Peripheral pattern of staining (rim) is characteristics of SLE

25
RIM is?
green around the periphery of the nucleus is characteristic of antibodies to double stranded RNA
26
SLE Treatment?
NSAIDS Antimalarial drugs Corticosteroids Cytotoxic agents
27
Chloroquine and hydroxychloroquine
Manages cutaneous manifests, arthralgia, fatigue and fever | Best used in long-term management
28
Corticosteroids
Pulse therapy | For remission of SLE with life-threatening diseas
29
Cytotoxic agents
Cyclphosphamide and Azathiprine (steroid sparing)
30
Cyclophosphamide
Converted by P450 to active which forms irreversible DNA crosslinks between and within strands --> death
31
Cyclophosphamide Indication and ADME
Lupus nephritis and sever lupus CYP450 Can lead to infxn or suppression of hematopoiesis
32
Azathioprine
Purine synthesis inhibitors 30-90% bioavailability Can cause blood clots Steroid sparing