Seropositive spondyloarthropathic disorders (obj 22)

Question 1

thickening of skin confined to face, neck, and distal extremities

Answer 1

scleroderma (limited) - 80% of cases

Question 2

widespread thickening of skin, including truncal involvement, with areas of increased pigmentation & depigmentation

Answer 2

scleroderma (diffuse) - 20% of cases

Question 3

these 2 things are present in virtually all scleroderma patients

Answer 3

Raynaud phenomenon

positive ANA test

Question 4

another name for limited scleroderma

Answer 4

CREST syndrome

Question 5

CREST syndrome is:

Answer 5

Calcinosis cutis
Raynaud phenomenon
Esophageal motility disorder
Sclerodactyly
Telangiectasia

Question 6

dryness of the eyes and dry mouth (sicca components) are the most common features of this disease

Answer 6

Sjögren syndrome

Question 7

Sjögren syndrome is usually associated with ____ but could occur with SLE, scleroderma, etc.

Answer 7

Rheumatoid arthritis

Question 8

These two lab tests are probably positive in Sjögren syndrome

Answer 8

RF

ANA

Question 9

This test measures the quantity of tears secreted, and therefore could be useful in diagnosing Sjögren syndrome

Answer 9

Schirmer test

Question 10

bilateral gradual, progressive muscle weakness of proximal upper and lower extremities as well as neck

Answer 10

polymyositis, dermatomyositis

Question 11

How can polymyositis/dermatomyositis be differentiated from myasthenia gravis?

Answer 11

facial/ocular weakness!

polymyositis/dermatomyositis: NO; myasthenia gravis yes

Question 12

What differentiates the dysphagias of scleroderma and polymyositis/dermatomyositis?

Answer 12

~scleroderma: affects smooth muscle of lower esophagus, causing a “sticking” sensation below sternum
~poly/dermato: affects striated muscles of upper pharynx, making initiation of swallowing difficult

Question 13

What are THREE characteristic rashes of dermatomyositis?

Answer 13

1. dusky red, malar distribution
2. heliotrope (violaceous periorbital edema)
3. shawl sign (erythema over neck, shoulders, upper chest/back)

Question 14

Describe the hands of someone with dermatomyositis.

Answer 14

Gottron sign! (scaly red patches over the dorsum of the PIP and MCP joints)

–also periungual erythema and dilations of nailbed capillaries

Question 15

The only specific diagnostic test for polymyositis/dermatomyositis:

Answer 15

muscle biopsy

Question 16

The 2 different muscle biopsy findings for poly and dermato myositis

Answer 16

polymyositis: endomysial infiltration
dermatomyositis: perifascicular atrophy

Question 17

treatment for polymyositis/dermatomyositis

Answer 17

corticosteroids

Question 18

hypercoagulability with recurrent thromboses

Answer 18

antiphospholipid syndrome

Question 19

With antiphospholipid syndrome, thromboses occur in (venous, arterial) circulation

Answer 19

EITHER

Question 20

If your patient has three or more unexplained miscarriages during the first trimester, you may suspect:

Answer 20

antiphospholipid syndrome

Question 21

treatment for antiphospholipid syndrome

Answer 21

lifelong anticoagulation with warfarin

heparin + aspirin if pregnant

Question 22

Patients with ____ or _____ have an increased risk of malignancy.

Answer 22

Dermatomyositis

SLE

Question 23

Many of the clinical manifestations of this disease are due to the trapping of antigen-antibody complexes in capillaries of viscera OR due to autoimmune destruction of host cells

Answer 23

Systemic lupus erythematosus (SLE)

Question 24

This demographic group tends to get SLE.

Answer 24

Young black women

Question 25

Before making a diagnosis of SLE, it is important to ascertain that the condition is not due to _____.

Answer 25

DRUGS

Question 26

This test is sensitive but not specific for SLE.

Answer 26

ANA | positive in virtually all patients with SLE but also positive in other conditions (e.g. scleroderma, Sjögren syndrome)

Question 27

What is the rash usually thought of as characteristic of SLE?

Answer 27

malar (butterfly) rash on face

Question 28

Over 90% of patients with SLE have this clinical manifestation, which is often the earliest manifestation.

Answer 28

joint symptoms

Question 29

What are 3 major sources of disease morbidity in SLE?

Answer 29

1. glomerulonephritis 2. CNS disease 3. antiphospholipid antibodies

Question 30

SLE patient education: patients with SLE must be cautioned against exposure to what?

Answer 30

SUN

Question 31

Treatments for SLE:

Answer 31

~NSAIDs (for joint pain) ~corticosteroids (for skin lesions, glomerulonephritis, CNS problems, etc.) ~antimalarials (for lupus rashes, joint symptoms) ~immunosuppressive agents

Question 32

Symmetric polyarthritis in small joints of hands and feet

Answer 32

Rheumatoid arthritis

Question 33

What is the most specific blood test for rheumatoid arthritis?

Answer 33

Anti-CCP antibodies

Question 34

Radiographic changes seen in rheumatoid arthritis are:

Answer 34

1. joint space narrowing | 2. joint erosion

Question 35

Compare and contrast rheumatoid arthritis and osteoarthritis. (3)

Answer 35

Osteoarthritis spares the wrist and MCP joints. Osteoarthritis joint pain is relieved by rest. Osteoarthritis has minimal articular inflammation.

Question 36

As soon as you confirm your diagnosis of RA, you should start what treatment?

Answer 36

DMARDs | disease-modifying anti-rheumatic drugs

Question 37

Name one synthetic DMARD and one biologic DMARD.

Answer 37

synthetic: methotrexate biologic: TNF inhibitors

Question 38

What is the role of corticosteroids in the treatment of RA?

Answer 38

Corticosteroids have a PROMPT anti-inflammatory effect, and are often used as a "bridge" to reduce disease activity until the slower-acting DMARDs take effect (or as adjunctive therapy for disease that persists despite treatment with DMARDs).

Question 39

Why would you give ACE inhibitors for SLE?

Answer 39

renal disease/BP control

Question 40

Smith autoantibody

Answer 40

SLE