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September 22, 1989

Systemic Sclerosis: Scleroderma

JAMA. 1989;262(12):1701-1702. doi:10.1001/jama.1989.03430120155044

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Fourteen years ago, I had the pleasure of reviewing Prof Stefania Jablonska's monograph, Sclerodermaand Pseudoscleroderma, for this journal. The publication of the current volume in 1988 permits us to understand the major advances in diagnosis, understanding of the disease, and treatment that the interval provided. And yet, if we accept the five outcome measures of disease championed by Fries and carefully defined in our book Prognosis, namely, death, disease activity, disability, treatment, and costs (listed, for the convenience of having five D's, as drugs and dollars), there are grounds for optimism. While mortality figures after diagnosis may not be particularly favorable, the introduction of angiotensinconverting enzyme inhibitors and other contemporary treatments may well influence the equation favorably.

Systemic sclerosis is not rare in specialized practices and university centers, which concentrate such cases. A recently concluded cooperative study in the United States contributed hundreds of cases, and more than 5000