Lupus, Scleroderma, Sjogren's Syndrome, MCTD, and Spondyloarthropathies

Question 1

HIgh risk races in SLE

Answer 1

African Americans, Hispanics, Asians

Question 2

Susceptibility genes of SLE

Answer 2

HLA-DR2, complement deficiency

Question 3

11 criteria for SLE

Answer 3

SOAPBRAINMD - Serositis, Oral ulcers, Arthritis, Photosensitivity, Blood disorders (cytopenias), Renal, ANA (FANA+), Immunologic (anti-Smith, anti-dsDNA, anti-phospholipid, anti-cardiolipin, lupus anticoagulant), Neuro (psychosis, seizures), Malar rash, Discoid rash

Question 4

Significance of ANA in lupus

Answer 4

ANA is really sensitive, BUT NOT SPECIFIC (useful if high suspicion of lupus)

Question 5

Where else do you see + ANA

Answer 5

Scleroderma

Question 6

Anti dsDNA and anti-Sm antibodies

Answer 6

High specificity for lupus, anti-dsDNA associated with nephritis

Question 7

Significance of anti-SSA and anti-SSB

Answer 7

Subcutaneous lupus, neonatal lupus, complete heart block in utero

Question 8

Complement pathway in SLE

Answer 8

Both classical and alternative activated–consumes C3 and C4

Question 9

Recurrent fetal loss in SLE is associated with what?

Answer 9

Antiphospholipid antibody

Question 10

Major cause of mortality in SLE

Answer 10

Atherosclerosis of the coronary arteries

Question 11

Criteria for antiphospholipid syndrome

Answer 11

Clinical event and persistent presence of antiphospholipid antibody

Question 12

Antibodies in scleroderma

Answer 12

Topo 1 (diffuse), centromere (limited), PM/Scl, U1-RNP

Question 13

Raynaud’s phenomenon

Answer 13

Vasospastic attacks in digits (white to blue to red), can cause digital ulcers

Question 14

Risk factors for sclerodermal renal crisis

Answer 14

Diffuse skin lesion, corticosteroids, anti-RNA polymerase III Ab

Question 15

Most common organ involved in scleroderma

Answer 15

GI dismotility, upper GI tract dysfunction, lower GI (bad prognosis)

Question 16

Lung diseases in scleroderma

Answer 16

Interstitial lung disease and pulmonary arterial hypertension

Question 17

Lab findings in Sjogren’s syndrome

Answer 17

ANA, SS-A/SS-B, rheumatoid factor

Question 18

Signs and symptoms in Sjogren’s syndrome

Answer 18

Dry eyes, dry mouth, dental caries, mouth ulcers, parotid/submandibular gland enlargement

Question 19

Cancer risk in Sjogren’s

Answer 19

Non-hodgkin’s lymphoma

Question 20

Mixed connective tissue disease overlaps with which diseases?

Answer 20

Overlap with lupus, scleroderma, and inflammatory mysoitis

Question 21

Antibody in MCTD

Answer 21

U1-RNP

Question 22

Common findings in MCTD

Answer 22

Puffy, swollen fingers (early), Raynaud’s, Renal, and CNS involvment

Question 23

Most common cause of death in MCTD

Answer 23

Pulmonary hypertension

Question 24

Labs in spondyloarthropathies

Answer 24

Rheumatoid factor NEGATIVE, association with HLA-B27 (sensitive but not specific)

Question 25

Common signs of spondyloarthropathies

Answer 25

Enthesitis, dactylitis (sausage digits),

Question 26

Epidemiology of ankylosing spondylitis

Answer 26

Weird epidemiology - young, males>females

Question 27

Clinical features of AS

Answer 27

Chronic back pain, symptoms worse in morning and IMPROVE with exercise

Question 28

Joint involvement in AS

Answer 28

Hips, shoulders, knees, and ankles, dactylitis, enthesitis with heel pain

Question 29

X-ray findings of AS

Answer 29

Squaring of vertebral bodies

Question 30

Classic triad of reactive arthritis

Answer 30

Arthritis, conjunctivitis, urethritis

Question 31

Psoraitic arthritis patterns

Answer 31

Oligoarticular (less than 4 joints, asymmetric), DIP involvement, axial involvement

Question 32

Enteropathic arthritis

Answer 32

Occurs after GI disease; gut wall is leaky barrier, associated with Crohn’s and ulcerative colitis