Silvia Bellando-Randone1,2, Marco Matucci-Cerinic3,4. 1. Department of Experimental and Clinical Medicine, University of Florence, V. Pieraccini 18, 50139, Florence, Italy. s.bellandorandone@gmail.com. 2. Department of Geriatric Medicine, Division of Rheumatology, AOUC, Florence, Italy. s.bellandorandone@gmail.com. 3. Department of Experimental and Clinical Medicine, University of Florence, V. Pieraccini 18, 50139, Florence, Italy. 4. Department of Geriatric Medicine, Division of Rheumatology, AOUC, Florence, Italy.
Abstract
PURPOSE OF REVIEW: The approach to systemic sclerosis (SSc) has changed over the years with an increasing focus on the very early diagnosis of the disease. The terminology identifying patients in the early phase of SSc has been significantly confusing in the last three decades. The purpose of this article is to analyze how the concept of "very early SSc" has evolved over the years, which is the role of an early diagnosis and how early treat patients. RECENT FINDINGS: Several attempts have been made over time, to create more sensitive and specific classification criteria to include the largest number of SSc patients, also in the earliest phase. An algorythm for the very early diagnosis of SSc was identified, diagnostic preliminary criteria proposed, and new 2013 ACR/EULAR SSc classification criteria published, including new items and adding emphasis to the vasculopathic manifestations. True biomarkers that could predict the disease evolution are still missing. Treat or not to treat patients in the earliest phases still remain a dilemma. For the moment, the only feasible clinical strategy in very early SSc remains a tight follow up program to detect in "real time" the early internal organ involvement which may allow an aggressive therapeutic agenda.
PURPOSE OF REVIEW: The approach to systemic sclerosis (SSc) has changed over the years with an increasing focus on the very early diagnosis of the disease. The terminology identifying patients in the early phase of SSc has been significantly confusing in the last three decades. The purpose of this article is to analyze how the concept of "very early SSc" has evolved over the years, which is the role of an early diagnosis and how early treat patients. RECENT FINDINGS: Several attempts have been made over time, to create more sensitive and specific classification criteria to include the largest number of SSc patients, also in the earliest phase. An algorythm for the very early diagnosis of SSc was identified, diagnostic preliminary criteria proposed, and new 2013 ACR/EULAR SSc classification criteria published, including new items and adding emphasis to the vasculopathic manifestations. True biomarkers that could predict the disease evolution are still missing. Treat or not to treat patients in the earliest phases still remain a dilemma. For the moment, the only feasible clinical strategy in very early SSc remains a tight follow up program to detect in "real time" the early internal organ involvement which may allow an aggressive therapeutic agenda.
Authors: Ariane L Herrick; Xiaoyan Pan; Sébastien Peytrignet; Mark Lunt; Roger Hesselstrand; Luc Mouthon; Alan Silman; Edith Brown; László Czirják; Jörg H W Distler; Oliver Distler; Kim Fligelstone; William J Gregory; Rachel Ochiel; Madelon Vonk; Codrina Ancuţa; Voon H Ong; Dominique Farge; Marie Hudson; Marco Matucci-Cerinic; Alexandra Balbir-Gurman; Øyvind Midtvedt; Alison C Jordan; Paresh Jobanputra; Wendy Stevens; Pia Moinzadeh; Frances C Hall; Christian Agard; Marina E Anderson; Elisabeth Diot; Rajan Madhok; Mohammed Akil; Maya H Buch; Lorinda Chung; Nemanja Damjanov; Harsha Gunawardena; Peter Lanyon; Yasmeen Ahmad; Kuntal Chakravarty; Søren Jacobsen; Alexander J MacGregor; Neil McHugh; Ulf Müller-Ladner; Gabriela Riemekasten; Michael Becker; Janet Roddy; Patricia E Carreira; Anne Laure Fauchais; Eric Hachulla; Jennifer Hamilton; Murat İnanç; John S McLaren; Jacob M van Laar; Sanjay Pathare; Susannah Proudman; Anna Rudin; Joanne Sahhar; Brigitte Coppere; Christine Serratrice; Tom Sheeran; Douglas J Veale; Claire Grange; Georges-Selim Trad; Christopher P Denton Journal: Ann Rheum Dis Date: 2017-02-10 Impact factor: 19.103
Authors: Nina M van Leeuwen; Maaike Boonstra; Jaap A Bakker; Annette Grummels; Suzana Jordan; Sophie Liem; Oliver Distler; Anna-Maria Hoffmann-Vold; Karin Melsens; Vanessa Smith; Marie-Elise Truchetet; Hans U Scherer; René Toes; Tom W J Huizinga; Jeska K de Vries-Bouwstra Journal: Arthritis Rheumatol Date: 2021-11-02 Impact factor: 15.483