SRS-X, The SRS Educational Resource for Radiology, CREST syndrome

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Case 81

CREST syndrome

A 63 year old lady with painful hands

This 63 year old caucasian lady presented with painful hands for some years, particularly during the winter months.

Findings

There are multiple areas of dense soft tissue calcification affecting several digits. There is an erosive arthropathy of the DIP joints. There is mild degenerative change in the right first CMC joint.

There is no soft tissue swelling or thinning and the terminal tufts are preserved.

Clinically the patient also had telangiectasia and her symptoms were typical of Raynaud's phenomenon.

Diagnosis

CRST syndrome

Discussion

CRST syndrome; also known as CREST syndrome when there are (o)esophageal abnormalities.

Calcinosis
Raynaud's
(o)Esophageal abnormalities
Sclerodactyly (not a prominent feature in this case)
Telangiectasia

CREST is one of the connective tissue disorders, closely allied to scleroderma.

Resnick gives some useful definitions:

Raynaud's Phenomenon or Disease: Paroxysmal occlusion of the digital arteries precipitated by cold or stress, (disease when it is idiopathic, phenomenon when it is secondary).
Acrosclerosis: Sclerosis of facial structures and fingers associated with Raynaud's.
Diffuse systemic sclerosis: Trunk involvement, commonly associated with systemic abnormalities. There may be peripheral involvement.
CRST syndrome: see above
CREST syndrome: see above
Thibierge-Weissenbach syndrome: The association of calcinosis and ischaemia.
Shulman syndrome: Scleroderma like syndrome with eosinophilia and hypergamaglobulinaemia, but without systemic involvement (aka eosinophilic fasciitis).

Arthropathy is a common feature of scleroderma (10-65%) and is often erosive.

See Resnick for a fuller description of these conditions.

References

(Resnick, D. Bone & Joint Imaging, WB Saunders)

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