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

Alain Lescoat; Catherine Cavalin; Rodney Ehrlich; Claire Cazalets; Alice Ballerie; Nicolas Belhomme; Guillaume Coiffier; Marine de Saint Riquier; Paul-André Rosental; Eric Hachulla; Vincent Sobanski; Patrick Jégo

doi:10.1016/S2665-9913(19)30038-4

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

Lancet Rheumatol. 2019 Dec;1(4):e257-e264. doi: 10.1016/S2665-9913(19)30038-4.

Authors

Affiliations

¹ Department of Internal Medicine and Clinical Immunology, Rennes University Hospital, University of Rennes 1, Rennes, France; École des Hautes Études en Santé Publique, Research Institute for Environmental and Occupational Health, French National Institute of Health and Medical Research Unit 1085, Rennes University Hospital, University of Rennes 1, Rennes, France. Electronic address: alain.lescoat@chu-rennes.fr.
² Interdisciplinary Research Institute for the Social Sciences, Université Paris-Dauphine, Université Paris Sciences et Lettres, Paris, France; Centre for European Studies and Comparative Politics, Sciences Po, Paris, France; Laboratory for Interdisciplinary Evaluation of Public Policies, Sciences Po, Paris, France; The Employment and Labour Research Centre, Noisy-le-Grand, France.
³ Centre for Occupational and Environmental Health Research, School of Public Health and Family Medicine, University of Cape Town, Cape Town, South Africa.
⁴ Department of Internal Medicine and Clinical Immunology, Rennes University Hospital, University of Rennes 1, Rennes, France.
⁵ Department of Internal Medicine and Clinical Immunology, Rennes University Hospital, University of Rennes 1, Rennes, France; École des Hautes Études en Santé Publique, Research Institute for Environmental and Occupational Health, French National Institute of Health and Medical Research Unit 1085, Rennes University Hospital, University of Rennes 1, Rennes, France.
⁶ Department of Rheumatology, Rennes University Hospital, University of Rennes 1, Rennes, France; French National Institute of Health and Medical Research Unit 1241, University of Rennes 1, Rennes, France.
⁷ Department of Rheumatology, Rennes University Hospital, University of Rennes 1, Rennes, France.
⁸ Center for History, Sciences Po, Paris, France.
⁹ Lille Inflammation Research International Center, University of Lille, Lille, France; French National Institute of Health and Medical Research, University of Lille, Lille, France; Département de Médecine Interne et Immunologie Clinique, Lille University Hospital, Lille, France; Centre National de Référence Maladies Systémiques et Auto-Immunes Rares du Nord et Nord-Ouest de France, Lille, France.

PMID: 38229382
DOI: 10.1016/S2665-9913(19)30038-4

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.

Publication types

Review