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
Q

RIM is?

A

green around the periphery of the nucleus is characteristic of antibodies to double stranded RNA

26
Q

SLE Treatment?

A

NSAIDS
Antimalarial drugs
Corticosteroids
Cytotoxic agents

27
Q

Chloroquine and hydroxychloroquine

A

Manages cutaneous manifests, arthralgia, fatigue and fever

Best used in long-term management

28
Q

Corticosteroids

A

Pulse therapy

For remission of SLE with life-threatening diseas

29
Q

Cytotoxic agents

A

Cyclphosphamide and Azathiprine (steroid sparing)

30
Q

Cyclophosphamide

A

Converted by P450 to active which forms irreversible DNA crosslinks between and within strands –> death

31
Q

Cyclophosphamide Indication and ADME

A

Lupus nephritis and sever lupus
CYP450
Can lead to infxn or suppression of hematopoiesis

32
Q

Azathioprine

A

Purine synthesis inhibitors
30-90% bioavailability
Can cause blood clots
Steroid sparing