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pathology starting with vasculitis > Systemic Autoimmune Disease > Flashcards

Systemic Autoimmune Disease Flashcards

1
Q

What does ANA mean?

A

Anti-Nuclear Antibody

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2
Q

An immune complex disease is what type of immune injury?

A

Type 3

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3
Q

Type 3 immune injuries hit which organs (2)?

A

Vessels

Kidneys

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4
Q

Type 2 immune injury is involved in what type of reactions? And what do they cause

A

Complement mediated that result in localized tissue infiltrates and damage

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5
Q

Type 4 immune injury present what kind of infiltrates?

A

Lymphocytic

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6
Q

Healing and regeneration requires what two steps?

A

Organization of fibrinoid deposits

Interstitial fibrosis

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7
Q

In SLE what happens to B cells to produce pathogenesis in SLE?

A

Activation of multiple clones of B cells

Possibly due to loss of normal suppressor T cell mechanisms

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8
Q

Most characteristic formulation of Antibodies in SLE?

A

Autoantibodies to double stranded DNA

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9
Q

What causes loss of normal hematological elements in SLE?

A

Autoantibodies that go after RBCs, neutrophils, and platelets

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10
Q

What test is used to show deposition of antibodies in basement membrane of the skin in SLE?

A 
Immunofluorescence techniques (positive band test)
Normal people can show these too so beware
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11
Q

What causes heart problems in SLE?

A

Antibodies to hyaluronic acid

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12
Q

What is specificity rule for deposition of immune complexes in the kidney and small blood vessels of the spleen?

A

Regardless of the specificity of the antibody immune complexes here will lead to type 3 mediated lesions.

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13
Q

What causes the tissue damage in SLE?

A

Combination of Type 2 (cytotoxic) and type 3 (immune complex mechanisms)

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14
Q

Lesions in SLE result in what involving complement?

A

Fixation of the complement

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15
Q

Fixation of complement in SLE results in what?

A

Low total complement level (hypocomplementemia)

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16
Q

SLE primarily affect whom?

A

Women of reproductive age

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17
Q

Most frequent manifestation of SLE?

A

Arthritis and arthralgia

Attacks self limited and DO NOT lead to chronic inflammatory destruction of joint with pannus formation

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18
Q

Progression of SLE?

A

Episodic and remitting attacks, gets more frequent, increases in number of systems involved, organ failure

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19
Q

Death in SLE results normally from?

A

Renal/cardiac failure

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20
Q

Characteristic rash is where in SLE?

A

Malar rash across nose and cheeks

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21
Q

What does every or nearly every patient with SLE test positive for?

A

ANA, but not diagnostic

22
Q

What is diagnostic of SLE?

A

Antibodies to double stranded DNA

23
Q

What ANA staining pattern would show binding to double stranded DNA?

A

Rim or peripheral staining patter

24
Q

Progressive renal failure in SLE is heralded by what?

A

Proteinuria

Decrease in creatinine clearance

25
Q

What are hematoxylin bodies?

A

Inclusion bodies of phagocytes formed by phagocytosis of leukocytes damaged by ANAs

26
Q

What leads to reactive skin lesions from deposited immune complexes in SLE?

A

UV light

27
Q

Four types of Renal disease patterns in SLE?

A

Mesangial (Mildest)
Focal Proliferative
Diffuse Proliferative (Worst)
Membranous

28
Q

What results in Onion skin concentric rings of fibrin deposition around small arterioles

A

Repeated injury resulting from immune complex deposition the walls of small blood vessels in the spleen

29
Q

What is Libman-Sacks endocarditis in SLE?

A

1-3 mm warty deposits on either surface of any of the heart valves

30
Q

What is anti-phospholipid syndrome?

A

Anti-phospholipid or anti-cardiolipin antibodies

31
Q

In anti-phopholipid syndrome in SLE how do the antibodies work?

A

In vitro they interfere with coagulation

In vivo they promote coagulation

32
Q

In chronic discoid lupus what is the characteristic lesion

A

Disc shaped scaling lesions usually above neck

33
Q

Are discoid lesions diagnostic of Chronic discoid lupus?

A

No, identical lesions can appear in systemic lupus

34
Q

Drug induced lupus is what?

A

Immune hypersensitivity to drug (Isoniazid, Penicillamine, and Procainamide)

35
Q

What resolves Drug induced lupus?

A

Remove use of the drug

36
Q

Sjogren’s syndrome destroys what structures?

A

Lacrimal and salivary glands

37
Q

What is Sicca syndrome associated with Sjogren?

A

Dry eyes, dry mouth, difficulty swallowing

38
Q

What causes the decrease of tears and saliva in Sjogrens?

A

Lymphocytic infiltration and fibrosis of lacrimal and salivary glands

39
Q

Which autoantibodies are most important in Sjogren’s?

A

Anti-ribonucelar protein antibodies SS-A and SS-B

High SS-A associated with extra glandular manifestations

40
Q

Important clinical feature of Sjogren’s?

A

Mikulicz’s syndrome: lacrimal and salivary gland enlargement of any cause

41
Q

Sjogren’s involvement in B-Cells?

A

Pseudolymphoma, 40 fold increase in risk of developing lymphoid malignancies

42
Q

What do you do to diagnose for Sjogren?

A

Biopsy the lip to examine minor salivary glands

43
Q

What is Progressive Systemic Sclerosis (Scleroderma)?

A

Excessive fibrosis through body, but skin is primary manifestation

44
Q

What are the two major patterns of Scleroderma?

A

Diffuse with early visceral involvement

Localized with limited skin confined to fingers, forearms, and face with late visceral involvement

45
Q

What is CREST?

A 
Mnemonic for symptoms of Scleroderma:
Calcinosis
Raynaud's
Esophageal dysmotility 
Sclerodactyly
Telangiasctasia
46
Q

Scleroderma associated with what?

A

Deposition of collagen, cause unknown

47
Q

Distinctive feature of Scleroderma?

A

Cutaneous involvement involving hands face

Raynauds precedes other symptoms

48
Q

Progression of Scleroderma?

A

Fibrosis of dermis -> Increased compact collagen -> Thinning of epidermis -> Focal calcifications -> Loss of blood supply to fingers may cause autoamputation

49
Q

Diagnostic feature for Scleroderma?

A

Anti-RNP (Ribonucleoprotein)

SS-A, SS-B

50
Q

Most ominous manifestation of renal involvement in Scleroderma?

A

Malignant hypertension with subsequent renal failure

pathology starting with vasculitis (7 decks)

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