French recommendations for the management of systemic sclerosis

• Main Authors: Eric Hachulla, Christian Agard, Yannick Allanore, Jerome Avouac, Brigitte Bader-Meunier, Alexandre Belot, Alice Berezne, Anne-Sophie Bouthors, Geraldine Condette-Wojtasik, Joël Constans, Pascal De Groote, Elisabeth Diot, Florence Dumas, Patrick Jego, Francisca Joly, David Launay, Veronique Le Guern, Janine-Sophie Le Quintrec, Geraldine Lescaille, Christophe Meune, Bruno Moulin, Christelle Nguyen, Nadine Omeish, Frederic Pene, Marie-Aleth Richard, Juliette Rochefort, Alexandra Roren, Olivier Sitbon, Vincent Sobanski, Marie-Elise Truchetet, Luc Mouthon, Collaborators
• Format: Article
• Language: English
• Published: BMC 2021-07-01
• Series: Orphanet Journal of Rare Diseases
• Subjects: Systemic sclerosis; Recommendations; Treatment
• Online Access: https://doi.org/10.1186/s13023-021-01844-y

Abstract Systemic sclerosis (SSc) is a generalized disease of the connective tissue, arterioles, and microvessels, characterized by the appearance of fibrosis and vascular obliteration. There are two main phenotypical forms of SSc: a diffuse cutaneous form that extends towards the proximal region of the limbs and/or torso, and a limited cutaneous form where the cutaneous sclerosis only affects the extremities of the limbs (without passing beyond the elbows and knees). There also exists in less than 10% of cases forms that never involve the skin. This is called SSc sine scleroderma. The prognosis depends essentially on the occurrence of visceral damage and more particularly interstitial lung disease (which is sometimes severe), pulmonary arterial hypertension, or primary cardiac damage, which represent the three commonest causes of mortality in SSc. Another type of involvement with poor prognosis, scleroderma renal crisis, is rare (less than 5% of cases). Cutaneous extension is also an important parameter, with the diffuse cutaneous forms having less favorable prognosis.