Systemic Autoimmune Disease

Question 1

What does ANA mean?

Answer 1

Anti-Nuclear Antibody

Question 2

An immune complex disease is what type of immune injury?

Answer 2

Type 3

Question 3

Type 3 immune injuries hit which organs (2)?

Answer 3

Vessels

Kidneys

Question 4

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

Answer 4

Complement mediated that result in localized tissue infiltrates and damage

Question 5

Type 4 immune injury present what kind of infiltrates?

Answer 5

Lymphocytic

Question 6

Healing and regeneration requires what two steps?

Answer 6

Organization of fibrinoid deposits

Interstitial fibrosis

Question 7

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

Answer 7

Activation of multiple clones of B cells

Possibly due to loss of normal suppressor T cell mechanisms

Question 8

Most characteristic formulation of Antibodies in SLE?

Answer 8

Autoantibodies to double stranded DNA

Question 9

What causes loss of normal hematological elements in SLE?

Answer 9

Autoantibodies that go after RBCs, neutrophils, and platelets

Question 10

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

Answer 10

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

Question 11

What causes heart problems in SLE?

Answer 11

Antibodies to hyaluronic acid

Question 12

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

Answer 12

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

Question 13

What causes the tissue damage in SLE?

Answer 13

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

Question 14

Lesions in SLE result in what involving complement?

Answer 14

Fixation of the complement

Question 15

Fixation of complement in SLE results in what?

Answer 15

Low total complement level (hypocomplementemia)

Question 16

SLE primarily affect whom?

Answer 16

Women of reproductive age

Question 17

Most frequent manifestation of SLE?

Answer 17

Arthritis and arthralgia

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

Question 18

Progression of SLE?

Answer 18

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

Question 19

Death in SLE results normally from?

Answer 19

Renal/cardiac failure

Question 20

Characteristic rash is where in SLE?

Answer 20

Malar rash across nose and cheeks

Question 21

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

Answer 21

ANA, but not diagnostic

Question 22

What is diagnostic of SLE?

Answer 22

Antibodies to double stranded DNA

Question 23

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

Answer 23

Rim or peripheral staining patter

Question 24

Progressive renal failure in SLE is heralded by what?

Answer 24

Proteinuria

Decrease in creatinine clearance

Question 25

What are hematoxylin bodies?

Answer 25

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

Question 26

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

Answer 26

UV light

Question 27

Four types of Renal disease patterns in SLE?

Answer 27

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

Question 28

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

Answer 28

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

Question 29

What is Libman-Sacks endocarditis in SLE?

Answer 29

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

Question 30

What is anti-phospholipid syndrome?

Answer 30

Anti-phospholipid or anti-cardiolipin antibodies

Question 31

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

Answer 31

In vitro they interfere with coagulation | In vivo they promote coagulation

Question 32

In chronic discoid lupus what is the characteristic lesion

Answer 32

Disc shaped scaling lesions usually above neck

Question 33

Are discoid lesions diagnostic of Chronic discoid lupus?

Answer 33

No, identical lesions can appear in systemic lupus

Question 34

Drug induced lupus is what?

Answer 34

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

Question 35

What resolves Drug induced lupus?

Answer 35

Remove use of the drug

Question 36

Sjogren's syndrome destroys what structures?

Answer 36

Lacrimal and salivary glands

Question 37

What is Sicca syndrome associated with Sjogren?

Answer 37

Dry eyes, dry mouth, difficulty swallowing

Question 38

What causes the decrease of tears and saliva in Sjogrens?

Answer 38

Lymphocytic infiltration and fibrosis of lacrimal and salivary glands

Question 39

Which autoantibodies are most important in Sjogren's?

Answer 39

Anti-ribonucelar protein antibodies SS-A and SS-B | High SS-A associated with extra glandular manifestations

Question 40

Important clinical feature of Sjogren's?

Answer 40

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

Question 41

Sjogren's involvement in B-Cells?

Answer 41

Pseudolymphoma, 40 fold increase in risk of developing lymphoid malignancies

Question 42

What do you do to diagnose for Sjogren?

Answer 42

Biopsy the lip to examine minor salivary glands

Question 43

What is Progressive Systemic Sclerosis (Scleroderma)?

Answer 43

Excessive fibrosis through body, but skin is primary manifestation

Question 44

What are the two major patterns of Scleroderma?

Answer 44

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

Question 45

What is CREST?

Answer 45

``` Mnemonic for symptoms of Scleroderma: Calcinosis Raynaud's Esophageal dysmotility Sclerodactyly Telangiasctasia ```

Question 46

Scleroderma associated with what?

Answer 46

Deposition of collagen, cause unknown

Question 47

Distinctive feature of Scleroderma?

Answer 47

Cutaneous involvement involving hands face | Raynauds precedes other symptoms

Question 48

Progression of Scleroderma?

Answer 48

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

Question 49

Diagnostic feature for Scleroderma?

Answer 49

Anti-RNP (Ribonucleoprotein) | SS-A, SS-B

Question 50

Most ominous manifestation of renal involvement in Scleroderma?

Answer 50

Malignant hypertension with subsequent renal failure