Description

The diagnosis of systemic sclerosis in clinical practice can be challenging. The 1980 ACR preliminary criteria may not be sensitive for limited or subtle disease. Hudson et al list a number of findings that may be clues to the diagnosis. The authors are from McGill University and multiple institutions in Canada with participants in the Canadian Scleroderma Research Group (CSRG).


 

The goal is to identify findings which may help a clinician to identify a patient who may need a more extensive evaluation or referral to a specialist.

 

Clues to the possible presence of systemic sclerosis:

(1) Raynaud's phenomenon

(2) visible mat-like telangiectasias

(3) sclerodactyly

(4) skin involvement proximal to the fingers

(5) nailfold capillary changes

(6) one or more systemic sclerosis-related autoantibodies

 

Systemic sclerosis-related autoantibodies include:

(1) anti-centromere antibody

(2) anti-topoisomerase I

(3) anti-RNA polymerase III

(4) anti-PM/Scl (polymyositis, scleroderma)

(5) anti-fibrillin

(6) anti-RNA polymerase I

(7) anti-fibrillarin

 

Additional findings may include:

(1) fingertip pitting scars (ulcers)

(2) loss of substance in digital finger pad

(3) finger contractures

(4) lung fibrosis

(5) pulmonary hypertension

(6) gastrointestinal involvement

(7) renal crisis

 

Performance:

• No single finding can make the diagnosis of systemic sclerosis.

• 10% of patients do not have a systemic sclerosis-related autoantibody.

• The ability to examine nails properly may not be available to all clinicians.

• Not all autoantibodies are readily available.

 


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