Autoimmunity

Question 1

What is rheumatoid arthritis?

Answer 1

• an autoimmune mediated inflammatory arthritis that leads to destruction of cartilage and bone
• systemic progressive inflammatory disorder

Question 2

What diseases are associated with rheumatoid arthritis?

Answer 2

• cardiovascular disease
• infections
• malignancies

**shortened life expectancies

Question 3

Mutations in genes for which MHC class is associated with rheumatoid arthritis?

Answer 3

MHC Class II

Question 4

What is the Pannus?

Answer 4

• Inflammatory region in RA
• the area where the synovial membrane meets the bone
• in rheumatoid arthritis, this is where inflammed synovial membrane activates osteoclasts via cytokines
• leads to destruction of the joint

Question 5

What is Rheumatoid Factor?

Answer 5

• antibody against Fc region of IgG
• 85% of patients have RF
• It is not specific for RA - seen in other autoimmune diseases, chronic infections, elderly, and 10% of healthy individuals

Question 6

What are Anti-cyclic citrullinated peptides (aCCP)?

Answer 6

-present in about 65-70% of RA patients

=loook this up

Question 7

What inflammatory cytokines are implicated in causing rheumatoid arthritis?

Answer 7

• IL-1
• IL-6
• TNF-alpha

Question 8

What hormone is associated with an increased risk for lupus?

Answer 8

-estrogen replacement

Question 9

What tend to be the targets for antibodies in Lupus?

Answer 9

-nuclear antigens

DNA and RNA complexes

Question 10

How many of the 11 ACR diagnostic criteria must be present to make a diagnosis of Lupus?

Answer 10

-4/11

Question 11

What is malar rash?

Answer 11

-ACR criteria for Lupus

-Fixed erythema,
Flat or raised,
Over the malar eminences
Spares the nasolabial folds

Question 12

What is Discoid Lupus?

Answer 12

-ACR criteria for lupus

-Erythematous raised patches with adherent keratotic scaling and follicular plugging;
Atrophic scarring in older lesions

Question 13

How is arthritis resulting from Lupus different from arthritis caused by rheumatoid arthritis and other autoimmune arthriti?

Answer 13

-Arthritis with lupus is non-erosive, and thus reversible

Question 14

What is the primary target of Polymyositis?

Answer 14

Muscle

autoimmune

Question 15

What is the primary target of Dermatomysitis?

Answer 15

Muscle & Skin

autoimmune

Question 16

What is the primary target of Systemic sclerosis/Scleroderma?

Answer 16

Skin/vasculopathy

autoimmune

Question 17

What is the primary target of Sjogrens Syndrome?

Answer 17

Salivary and lacrimal glands

autoimmune

Question 18

What is scleroderma?

Answer 18

• Multisystem disease characterized by fibrosis of the skin and internal organs
• Vasculopathy : widespread functional and structural abnormalities in small arteries, arterioles, and capillaries
• Autoimmune

Question 19

What is Systemic Sclerosis?

Answer 19

-Subset of scleroderma

• Raunaud’s phenomenon
• Fatigue
• Musculoskeletal complaints
• Puffy skin progressing to skin tightening and fibrosis
• Internal organ involvement

Question 20

What is Raynaud’s Phenomenon?

Answer 20

• Closure of muscular digital vessels
• Triggered by cold or stress, exacerbated by medications (beta-blockers)
• Triphasic color changes (pallor, cyanosis, erythematous)
• Seen in 4-15% of normal population
• Seen in 95% of patients with scleroderma

Question 21

What is the clinical presentation of Polymyositis?

Answer 21

• Insidious onset of proximal muscle weakness in shoulder and pelvic girdles
• 50% of pts have weakness in neck flexors
• Dysphagia
• Dysphonia
• Myalgia
• Raunaud’s

Question 22

What is the clinical presentation of Dermatomyositis?

Answer 22

• Proximal muscle weakness and associated symptoms similar to polymyositis
• Skin rashes

Question 23

What immune cells are associated with Polymyositis?

Answer 23

-CD8+

Question 24

What immune cells are associated with Dermatomyositis?

Answer 24

• CD4+

* also Complement & antibodies

Question 25

What is Sjögren’s Syndrome?

Answer 25

-autoimmune disorder -lymphocytic infiltration of exocrine glands (salivary and lacrimal) -also has systemic manifestations Sx's: - dry eyes - Dry mouth 25% have internal organ involvement

Question 26

Tell me about Scleroderma Renal Crisis.

Answer 26

- its an emergency - Involves accelerated hypertension and rapid renal failure - ACE inhibitors will save the patient's life

Question 27

What are the ACR diagnostic criteria for lupus?

Answer 27

1. Malar rash (butterfly rash) 2. Discoid Rash 3. Photosensitivity 4. Oral ulcers 5. Non-erosive arthritis 6. Serositis (o=pleuritis or pericarditis) 7. Neurological disorder 8. Renal disorder (proteinuria) 9. Hematologic disorder (hemolytic anemia) 10. ANA positive 11. immunologic disorder