French recommendations for the management of systemic sclerosis
É. Hachulla, C. Agard, Yannick Allanore, Jérôme Avouac, Brigitte Bader‐Meunier, Alexandre Bélot, A. Bérezné, Anne‐Sophie Bouthors, G. Condette-Wojtasik, J. Constans, Pascal de Groote, Élisabeth Diot, Florence Dumas, P. Jégo, Francisca Joly, David Launay, Véronique Le Guern, Janine-Sophie Le Quintrec, Géraldine Lescaille, Christophe Meune, Bruno Moulin, Christelle Nguyen, Nadine Omeish, Frédéric Pène, M.‐A. Richard, Juliette Rochefort, Alexandra Rören, Olivier Sitbon, Vincent Sobanski, Marie‐Elise Truchetet, Luc Mouthon, Collaborators, Marc Bayen, Emmanuel Bergot, S. Berthier, Julia Bosco, Yoram Bouhnik, Benjamin Chaigne, Vincent Cottin, Bruno Crestani, C. Deligny, Vianney Descroix, Dominique Farge, Dominique Godard, B. Granel, Philippe Guilpain, B. Imbert, Alain Le Quellec, Christophe Lega, Catherine Lok, Hélène Maillard, Thierry Martin, G. Pugnet, V. Queyrel, Loïc Raffray, F. Rilliard, Mélanie Romier, Laurence Schuller, Amélie Servettaz
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.