Disease subsets in clinical practice

Abstract

Systemic sclerosis is a heterogeneous disease with a varied clinical presentation and disease course. Classification is vital to grouping patients of similar phenotypes for both clinical care and research purposes. This chapter reviews the currently accepted cutaneous-based classification of patients with an emphasis on staging of disease. We propose that the serologic classification system be used to augment the cutaneous-based classification system. The concept of disease staging within the cutaneous-serologic classification systems is presented and illustrated by patient profile examples. Mixed connective tissue disease as a distinct clinical entity with SSc features is reviewed.