Master the board step 3

Rheumatology
Diagnostic Testing
CREST syndrome has anti-Scl70 less often, but does have anti-centromere
antibodies more often.
CREST is characterized
by anticentromere
antibodies.
Eosinophilic Fasciitis
Eosinophilic fasciitis presents with thickened skin that looks like scleroderma.
However, the following are not present:
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Hand involvement
··
Raynaud’s
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Heart, lung, or kidney involvement
There is marked eosinophilia and the appearance of an “orange peel” (peau
d’orange). Symptoms are worse with exercise.
Treatment is with corticosteroids.
Polymyositis (PM) and Dermatomyositis (DM)
In both conditions, the patient cannot get up from a seated position without
using the arms. There can also be muscle pain and tenderness.
For polymyositis, look for the following:
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Proximal muscle weakness
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Signs of muscle inflammation on blood tests, electromyography, and
biopsy
For dermatomyositis, you find the same thing and various rashes.
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Gottron’s papules: Over metocarpophalangeal joint surfaces
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Heliotrope rash: Periorbital and purplish lesion around the eyes
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Shawl sign: Shoulder and neck erythema
Diagnostic Testing
Testing reveals elevated CPK and aldolase with an abnormal electromyogram
(EMG). For CCS, order all the liver function tests as well as ANA.
• Weakness + ↑CPK +
↑Aldolase + Biopsy =
Polymyositis
• Weakness + ↑CPK +
↑Aldolase + Biopsy
+ Skin rash =
Dermatomyositis
Biopsy is the single most
accurate test of PM/DM.
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