Mar29 M1-Introduction to Autoimmune Diseases

Question 1

most general symptoms that may be signs of auto-immunity

Answer 1

constitutional: fever, weight loss, poor appetite
non-specific: Raynaud’s (fingers white then blue then red), Sicca (dry eyes, dry mouth + CT disease), diffuse lymphadenopathy

Question 2

symptoms that are highly associated with autoimmune disease (to check before doing lab tests)

Answer 2

• arthritis
• skin rash
• mucosal involvement
• lung involvement
• ocular symptoms
• renal dysfunction
• peripheral nerve defect

Question 3

big thing we want to determine in inflam disease dx

Answer 3

• if disease is inflammatory or not

- ostearthritis = non inflam, arthritis = inflam

Question 4

problem of lab tests in autoimmune diseases

Answer 4

are not diagnostic because the diseases in rheumatology are rare. more likely that someone doesn’t have disease

Question 5

non specific tests done in autoimmune disease

Answer 5

• CBC
• creatinine
• liver function
• UA
• urine protein measurement (kidney involvement is common in autoimmune)

Question 6

lab tests for inflammation and for immune activation

Answer 6

for inflammation: ESR (erythrocyte sedimentation rate) and CRP
for immune activation: C3 and C4

Question 7

auto-antibodies in autoimmune diseases

Answer 7

-only ASSOCIATED with these diseases but may not play role in pathophgy
-false - and false + are possible
-level doesn’t reflect how sick pt is
-

Question 8

auto-Ab often found in lupus

Answer 8

anti-nuclear Ab (ANA) (only association. doesn’t cause lupus)

Question 9

last dx step in autoimmune diseases

Answer 9

biopsy of an affected organ like the kidneys after noticed proteinuria for example

Question 10

different kinds of autoimmune diseases

Answer 10

• Connective tissue diseases (SLE, slceroderma limited or diffuse, Sjogren’s syndrome, Mixed CT disease (MCTD))
• Inflammatory myositis
• systemic vasculitis
• inflammatory arthropathies (arthritic psoriasis, etc.)

Question 11

scleroderma charact

Answer 11

• severe fibrosis (sclerosis = fibrosis) of skin, blood vessels, lung, GIT, kidneys and other organs. hard to move
• affects skin in limited (CREST syndrome) or diffuse pattern

Question 12

2 things often found in pts with scleroderma

Answer 12

• Raynaud’s sign

- positive ANA

Question 13

Sjogren’s syndrome charact

Answer 13

• affects exocrine glands

- xerostomia, xerophtalmia (dry mouth and eyes), joints, lungs, kidneys, CNS, etc.

Question 14

2 things often found in Sjogren’s disease patients

Answer 14

anti-Ro (SSa) and anti-La (SSb) auto-Abs

Question 15

MCTD (mixed CT disease) definition

Answer 15

• defined as having positive anti-RNP (rhybonuclear protein) Ab
• autoimmune disease where manifestations are normally seen in 3 other autoimmune diseases can be seen
• SLE
• scleroderma
• inflamatory myositis

Question 16

things often seen in MCTD

Answer 16

• Raynaud’s sign
• lung involvement
• anti-RNP antibody

Question 17

SLE charact

Answer 17

• affects mostly young women

- characterized by immune complex deposition in affected organs

Question 18

how mortality in lupus compares to mortality in healthy individual

Answer 18

2-5x greater mortality rate in lupus

Question 19

how lupus is diagnosed

Answer 19

• there’s a table with 11 criteria but is mostly used to check who to enter in a clinical trial
• real dx is with expert opinion (might dx lupus if lacking signs. might say it’s not lupus even if have a lot of signs)

Question 20

11 manifestations of lupus

Answer 20

1. malar rash 2. discoid rash 3. photosensitivity 4. oral ulcers 5. arthritis 6. serositis 7. renal disorder 8. neurologic disorder 9. hematologic disorder 10. immunologic disorder 11. ANA

Question 21

specific clinical manifestations you really look for in SLE

Answer 21

• cutaneous manifestations
• mucosal ulcers
• arthritis
• lupus nephritis
• serositis
• hemato prob
• auto Ab

Question 22

3 categories of skin rashes in lupus

Answer 22

acute LE = localized (malar) or generalized
subacute LE = annular or papulosquamous
chronic LE = classical DLE (localized, generalized), hypertrophic DLE, lupus panniculitis, lupus tumidus, DLE (lichen planus overlap)

Question 23

most common rash in lupus

Answer 23

malar rash

• butterfly rash below eyes, on the cheeks
• DOESN’T affect the nasolabial fold
• photosensitive (CAUSED BY UV LIGHT)
• non scaring bc affects superficial skin

Question 24

discoid rash charact

Answer 24

• involvement of deeper skin (dermis) = alopecia, depigmentation, scarring
• less photosensitivity than malar rash

Question 25

major cause of lupus morbidity and mortality on the long-term

Answer 25

lupus nephritis (in 60% of lupus patients) - need to monitor SLE pts regularly for kidney fct and UA + protein qt (major reason why lupus mortality decreased) - need bx to dx lupus nephritis

Question 26

serositis meaning (one of the 11 criteria in lupus)

Answer 26

- pleuritis, pericarditis and peritonitis - shortness of breath, pleuritic pain (distinct from MI bc varies with deep breath) - can have tamponade (one cause of lupus mortality)

Question 27

hematologic involvement in lupus: things that can be seen

Answer 27

- chronic anemia (hemolytic for ex) - ITP, TTP - cytopenias

Question 28

main Ab in lupus

Answer 28

ANA (anti-nuclear Ab)

Question 29

how false positives with ANA testing are avoided

Answer 29

serum volume that is collected is diluted in a lot of saline. greater dilution = reduces false + (you get more specificity)