Orphanet Journal of Rare Diseases (Jul 2021)
French recommendations for the management of systemic sclerosis
- 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
Affiliations
- Eric Hachulla
- Service de Médecine Interne et Immunologie Clinique, Centre de Référence Des Maladies Autoimmunes Systémiques Rares du Nord et Nord-Ouest de France (CeRAINO), Univ. Lille, Inserm, CHU Lille, U1286 - INFINITE - Institute for Translational Research in Inflammation
- Christian Agard
- Internal Medicine, Nantes University Hospital, University of Nantes
- Yannick Allanore
- Rheumatology Department, Hôpital Cochin, AP-HP, Université de Paris
- Jerome Avouac
- Rheumatology Department, Hôpital Cochin, AP-HP, Université de Paris
- Brigitte Bader-Meunier
- Department of Pediatric Immunology and Rheumatology; Hospital Necker, APHP
- Alexandre Belot
- Pediatric Nephrology, Rheumatology, Dermatology, HFME, Hospices Civils de Lyon
- Alice Berezne
- Department of Internal Medicine, CHR Annecy-Genevois
- Anne-Sophie Bouthors
- Anaesthesia Intensive Care Unit, Jeanne de Flandre Women Hospital, Academic Hospital, ULR 7365 - GRITA - Groupe de Recherche Sur Les Formes Injectables Et Les Technologies Associées, University Lille
- Geraldine Condette-Wojtasik
- Service de Médecine Interne et Immunologie Clinique, Centre de Référence Des Maladies Autoimmunes Systémiques Rares du Nord et Nord-Ouest de France (CeRAINO), Univ. Lille, Inserm, CHU Lille, U1286 - INFINITE - Institute for Translational Research in Inflammation
- Joël Constans
- Vascular Medicine Department, Bordeaux University Hospital Centre, Saint André Hospital, FCRIN INI-CRCT (Cardiovascular and Renal Clinical Trialists) PeripherAL Artery DIsease Network (PALADIN)
- Pascal De Groote
- Cardiology Department, Lung-Heart Institute, CHU de Lille
- Elisabeth Diot
- Service de Médecine Interne, CHU Tours
- Florence Dumas
- Emergency Department, Cochin Hospital, Paris University
- Patrick Jego
- Internal Medicine and Clinical Immunology Unit, CHU Rennes
- Francisca Joly
- Department of Gastroenterology, IBD and Nutrition Support, Beaujon Hospital, INSERM UMRS-1149, Assistance Publique-Hôpitaux de Paris, University of Paris
- David Launay
- Service de Médecine Interne et Immunologie Clinique, Centre de Référence Des Maladies Autoimmunes Systémiques Rares du Nord et Nord-Ouest de France (CeRAINO), Univ. Lille, Inserm, CHU Lille, U1286 - INFINITE - Institute for Translational Research in Inflammation
- Veronique Le Guern
- Service de Médecine Interne, Centre de Référence Maladies Autoimmunes Systémiques Rares D’Ile de France, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP), APHP-CUP, Hôpital Cochin, Université de Paris
- Janine-Sophie Le Quintrec
- Rheumatology Department, Hôpital Cochin, AP-HP, Université de Paris
- Geraldine Lescaille
- Centre d’Immunologie et Maladies Infectieuses (CIMI-Paris), Department of Odontology, Paris Diderot/Paris 07, Sorbonne Paris Cité, AP-HP, Groupe Hospitalier Pitié-Salpêtrière
- Christophe Meune
- Cardiology Department, Hôpital Avicenne, AP-HP, Université de Paris
- Bruno Moulin
- Department of Nephrology and Kidney Transplantation, Nouvel Hôpital Civil, University Hospitals of Strasbourg
- Christelle Nguyen
- Physical Medicine and Rehabilitation Department, Hôpital Cochin, AP-HP, Université de Paris
- Nadine Omeish
- Oral and Dental Medicine, Hôpital Pitié-Salpêtrière, APHP, Université de Paris
- Frederic Pene
- Medical Intensive Care Unit, Hôpital Cochin, AP-HP. Centre & Université de Paris
- Marie-Aleth Richard
- Department of Dermatology, Timone Hospital, University Hospital of Marseille
- Juliette Rochefort
- Oral and Dental Medicine, Hôpital Pitié-Salpêtrière, APHP, Université de Paris
- Alexandra Roren
- AP-HP Cochin Hospital, Université Paris Descartes Sorbonne Paris Cité, INSERM U1153
- Olivier Sitbon
- Assistance Publique-Hôpitaux de Paris, Service de Pneumologie, Hôpital Bicêtre, Laboratoire d’Excellence en Recherche Sur le Médicament et Innovation Thérapeutique, Université Paris-Sud
- Vincent Sobanski
- Service de Médecine Interne et Immunologie Clinique, Centre de Référence Des Maladies Autoimmunes Systémiques Rares du Nord et Nord-Ouest de France (CeRAINO), Univ. Lille, Inserm, CHU Lille, U1286 - INFINITE - Institute for Translational Research in Inflammation
- Marie-Elise Truchetet
- Rheumatology Department, Hôpital Pellegrin, CHU de Bordeaux
- Luc Mouthon
- Service de Médecine Interne, Centre de Référence Maladies Autoimmunes Systémiques Rares D’Ile de France, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP), APHP-CUP, Hôpital Cochin, Université de Paris
- Collaborators
- DOI
- https://doi.org/10.1186/s13023-021-01844-y
- Journal volume & issue
-
Vol. 16,
no. S2
pp. 1 – 61
Abstract
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.
Keywords
