The nosology of systemic sclerosis: how lessons from the past offer new challenges in reframing an idiopathic rheumatological disorder.

ABSTRACT

Systemic sclerosis is a rare connective tissue disease characterised by a wide range of clinical manifestations. Compared with previous sets of criteria, the 2013 American College of Rheumatology and European League Against Rheumatism (ACR/EULAR) classification of systemic sclerosis encompasses a broader and more relevant spectrum of the condition. Nonetheless, clinical and prognostic heterogeneity persists among patients fulfilling these criteria. The next task in the classification of systemic sclerosis is the development of new subset criteria that can successfully identify subgroups of patients with distinct prognostic or pathophysiological features. In this Viewpoint we describe the history of systemic sclerosis over the past century with the objective of highlighting the effect of previous nosological debates on efforts to understand and manage this disorder. Rather than seeking to present a systematic review of possible subgrouping for systemic sclerosis in relation to prognosis, we aim to clarify how nosological considerations have influenced our understanding of the cause and prognosis of this so-called idiopathic rheumatological disorder and how aetiological, prognostic, and pathophysiological hypotheses have helped to describe clusters within the disease. By reflecting on past nosological debates and endeavours, we identify challenges for the current initiative to develop a new subgrouping of systemic sclerosis.