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Rheumatology Advance Access originally published online on May 3, 2005
Rheumatology 2005 44(10):1212-1216; doi:10.1093/rheumatology/keh671
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© The Author 2005. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oupjournals.org

REVIEW

Classification of systemic sclerosis. Visions and reality*

F. A. Wollheim

Department of Rheumatology, Lund University hospital, S-21774 Lund, Sweden.

Correspondence to: F. A. Wollheim. E-mail: frank.wollheim{at}reum.lu.se

Systemic sclerosis, scleroderma (SSc) is a disabling condition that shortens life expectancy. Disease heterogeneity and difficulties separating SSc from SSc-like conditions make classification an important issue. Limited cutaneous and diffuse cutaneous SSc, with different severity and survival, have been recognized for several years as distinct subsets. Some authors have suggested an intermediate cutaneous form with intermediate survival. This issue remains unsettled, however. The technique of capillaroscopy is helpful as an adjunct diagnostic tool to separate idiopathic Raynaud's phenomenon from SSc. Digitized video-capillaroscopy is developing as a powerful new method to assess individual capillaries over time. Using the simpler techniques of video-capillaroscopy, different patterns have been described and named ‘early’, ‘active’, ‘late’ and ‘slow’. The value of nailfold video-capillaroscopy to distinguish different subsets or provide prognostic information for use in daily practice remains to be assessed. The features of CREST (calcinosis, Raynaud's, oesophagus dysmotility, sclerodactyly, telangiectasias) are not confined to single subsets of SSc. There is no convincing evidence of any advantage for distinguishing the limited, intermediate and diffuse forms of SSc rather than only the limited and diffuse forms.

*Dedicated to the memory of E. Carwile LeRoy, 1933–2002.


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