Autoimmune Diseases Ii

Front 1

What causes myasthenia graves?

Back 1

1. Autoantibodies, +/- complement towards Ach receptors

Front 2

What type of HSR is myasthenia gravis?

Back 2

1. Type II

Front 3

What are the ssx of myasthenia gravis?

Back 3

1. Progressive muscle weakness with exercise

2. Gets better with rest

3. Only skeletal muscles affected

4. "Better in the morning"

Front 4

What is neonatal myasthenia?

Back 4

1. Generalized muscle weakness due to passively transferred IgG

Front 5

How do you dx myasthenia gravis?

Back 5

1. Circulating anti-AchR antibodies

2. Thymoma on x-ray and histology

3. Abnormal electrical activity in muscles

4. Administration of edrophonium resolves ssx

Front 6

How do you tx myasthenia gravis?

Back 6

1. Cholinesterase inhibitors

2. Corticosteroids

3. Immunosuppressive drugs

4. IV immune globulin

Front 7

What is Addison disease?

Back 7

1. Adrenocrotical insufficiency

Front 8

What type of HSR is Addison disease?

Back 8

1. Type II

Front 9

What is the presentation of Addison disease?

Back 9

1. Hyperpigmentation of mucous membranes

2. Overproduction of corticotrophin and melanocyte-stimulating hormone

3. Vitiligo

Front 10

How do you dx Addison disease?

Back 10

1. ACTH stimulation test

2. Blood chemistries

3. Adrenal gland atrophy

Front 11

How do you tx Addison's?

Back 11

1. Hydrocrotisone

Front 12

What is bullous pemphigoid?

Back 12

1. Chronic subepithelial blistering skin disease

2. Rare involves mucous membrane

Front 13

What type of HSR are bullous pemphigoid/pemphigus vulgaris?

Back 13

1. II

Front 14

What antigens cause bullous pemphigoid?

Back 14

1. IgG to hemidesmosomal antigens

Front 15

What are the ssx of bullous pemphigoid?

Back 15

1. 65 y/o

2. Blisters are tense

3. Preceded by urticaria

Front 16

How do you dx bullous pemphigoid?

Back 16

1. DIF of lesion bx shows IgG and complement in linear band at dermal-epidermal junction

Front 17

What is involved in pemphigus vulgaris?

Back 17

1. Skin and mucous membranes

Front 18

What causes the lesions in pemphigus vulgaris?

Back 18

1. IgG to keratinocyte desmogleins

Front 19

What is the presentation of pemphigus vulgaris?

Back 19

1. 50-60 y/o

2. Flaccid blisters are painful but don't itch

Front 20

How do you dx pemphigus vulgaris?

Back 20

1. DIF shows IgG or IgM and C3 on surface of keratinocytes

Front 21

What causes Graves disease?

Back 21

1. Autoabs against TSH receptor

Front 22

What are the autoabs in Graves?

Back 22

1. Thyroperoxidase

2. Thyroglobulin

3. Na-I symporter

Front 23

What is the presentation of Graves disease?

Back 23

1. Goiter

2. Exophthalmos

3. Pretibial myxedema

Front 24

What type of HSR is Graves?

Back 24

1. II

Front 25

How do you dx Graves?

Back 25

1. Anti-TSH receptor Ab

2. Low TSH

3. Increased T3 and T4

4. Increased radioactive uptake

5. Pretibial myxedema

Front 26

What causes Hashimoto?

Back 26

1. TH1 infiltration destroys thyroid gland

Front 27

What antibodies are present in Hashimoto?

Back 27

1. Thyroid peroxidase

2. Thyroglobulin

Front 28

What are the ssx of Hashimoto?

Back 28

1. Goiter

2. Ssx of hypothyroidism

Front 29

How do you dx Hashimoto?

Back 29

1. Elevated TSH

2. Antibodies to thyroid peroxidase and thyroglobulin

3. Diffuse lymphocytic and plasma cell infiltrate in follicles

Front 30

How do you tx Hashimoto?

Back 30

1. Levothyroxine

2. Surgery

Front 31

What is RA?

Back 31

1. Destructive disease

2. MC= hands and feet

3. Bone erosion in joints

Front 32

What are the ssx of RA?

Back 32

1. Morning stiffness

2. Arthritis in > 3 joint areas

3. Rheumatoid nodules

Front 33

What is the mechanism behind RA?

Back 33

1. HLA-DR4 mutation

2. CD4 cells stimulate synovial cells, fibroblasts, osteoclasts, PMNs to produce cytokines, ROS, and NO

3. Damage to connective tissue

4. Pannus formation

5. Complement can be fixed

Front 34

What is the target of immunotx in RA?

Back 34

1. TNFa

Front 35

What antibody fixes complement in RA?

Back 35

1. RF

Front 36

How do you dx RA?

Back 36

1. Elevated ESR and CRP

2. Circulating RF

3. Radiographs showing erosions/decalcifications

Front 37

For what is Rituximab specific?

Back 37

1. CD20

2. Use to tx RA

Front 38

What type of HSR is SLE?

Back 38

1. III

Front 39

What causes SLE?

Back 39

1. IC of self DNA, Ab, and others

2. ICs fix complement

3. Lodge in various regions

Front 40

What complement deficiency contributes to development of SLE?

Back 40

1. C1, C2, and C4

Front 41

What are the ssx of SLE?

Back 41

1. Erythema--- butterfly rash

2. Glomerulonephritis

3. Arthritis

Front 42

What are the MCC of death in SLE?

Back 42

1. Bacterial infection

2. Arteriosclerosis

Front 43

How do you dx SLE?

Back 43

1. Lumpy-bumpy pattern on IF of kidney bx

2. Anti-dsDNA antibodies--- kidney damage and disease severity

3. Anti-Smith antibodies--- renal involvement

Front 44

How do you manage SLE?

Back 44

1. Avoid sunlight to prevent flares

2. Methotrexate for chronic

3. Prednisone, cyclophosphamide, etc.

Front 45

What is Sicca Syndrome?

Back 45

1. Chronic inflammatory disease mediated by CD4 infiltrate affecting exocrine glands

2. Principally lacrimal and salivary glands

Front 46

What are the ssx of Sicca?

Back 46

1. Xerophthalmia

2. Xerostomia with poor oral health

3. Xeroderma

4. Parotid swelling

Front 47

What are the risks of Sicca?

Back 47

1. Increases risk of non-Hodgkin lymphoma

2. Maternal antibodies to SS-A/Ro increase risk of neonatal lupus and congenital heart block

Front 48

How do you dx Sicca?

Back 48

1. RF and ANA on 70% of patients

2. Schirmer test for tear produciton

3. Salivary gland bx shows aggregate of CD4 T cells

Front 49

What is Guillain-Barre syndrome?

Back 49

1. Ascending paralysis that affects the peripheral nerves

2. Follows infection or vaccination

Front 50

What are the MC etiologic agents of GBS?

Back 50

1. Campylobacter

2. CMV

3. EBV

4. VZV

5. Mycoplasma

Front 51

What is the mechanism behind GBS?

Back 51

1. Lipid antigens of microbes demonstrate molecular mimicry with GM1 and GM1b in myelin

2. Inflammatory cellular response** leads to pathology

Front 52

How do you dx GBS?

Back 52

1. Abnormal nerve conduction tests

2. Elevated CSF protein w/o WBC elevation

3. Reduction in force vital capacity

Front 53

How do you tx GBS?

Back 53

1. Medical emergency--- closely monitor

2. Plasmapheresis or IVIG

3. PT and speech tx

Front 54

What is MS?

Back 54

1. Chronic or relapsing paralysis that attacks myelinated axons

Front 55

What are the ssx of MS?

Back 55

1. Dysarthria

2. Ataxia

3. Tremor

4. Paresthesias

Front 56

What is the mechanism behind MS?

Back 56

1. TH17 v. T reg for myelin basic protein

2. IL-17 induces killing of oligodendrocytes that comprise myelin sheath

Front 57

How do you dx MS?

Back 57

1. MRI: plaques of demyelination in white matter

2. Oligoclonal IgG bands detected by electrophoresis

3. IgG levels correlated with disease severity

Front 58

How do you tx MS?

Back 58

1. Methylprednisone

1. ABC tx---- avonex, betaserone, copaxone