French recommendations for the management of systemic sclerosis

Eric Hachulla; Christian Agard; Yannick Allanore; Jérôme 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; Géraldine Lescaille; Christophe Meune; Bruno Moulin; Christelle Nguyen; Nadine Omeish; Frédéric Pene; Marie-Aleth Richard; Juliette Rochefort; Alexandra Roren; Olivier Sitbon; Vincent Sobanski; Marie-Elise Truchetet; Luc Mouthon; Marc Bayen; Emmanuel Bergot; Sabine Berthier; Julia Bosco; Yoram Bouhnik; Benjamin Chaigne; Vincent Cottin; Bruno Crestani; Christophe Deligny; Vianney Descroix; Dominique Farge; Dominique Godard; Brigitte Granel; Philippe Guilpain; Bernard Imbert; Alain Le Quellec; Christophe Lega; Catherine Lok; Hélène Maillard; Thierry Martin; Grégory Pugnet; Viviane Queyrel; Loïc Raffray; Frédéric Rilliard; Mélanie Romier; Laurence Schuller; Amélie Servettaz

doi:10.1186/s13023-021-01844-y

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French recommendations for the management of systemic sclerosis

Journal article

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French recommendations for the management of systemic sclerosis

Eric Hachulla, Christian Agard, Yannick Allanore, Jérôme Avouac, Brigitte Bader-Meunier, Alexandre Belot, Alice Berezne, Anne-Sophie Bouthors, Geraldine Condette-Wojtasik, Joël Constans, …

Orphanet Journal of Rare Diseases, Vol.16(S2)

26/07/2021

DOI: https://doi.org/10.1186/s13023-021-01844-y

PMCID: PMC8310704

PMID: 34304732

Abstract

Recommendations

Systemic sclerosis

Treatment

Humans

Hypertension, Pulmonary

Lung Diseases, Interstitial

Scleroderma, Systemic

Skin

Skin Diseases

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.

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Title: French recommendations for the management of systemic sclerosis
Creators - without role: Eric Hachulla - Institute for Translational Research in Inflammation
Christian Agard - Centre Hospitalier Universitaire de Nantes
Yannick Allanore - Hôpital Cochin
Jérôme Avouac - Institut Cochin
Brigitte Bader-Meunier - Service d'immuno-hématologie pédiatrique [CHU Necker]
Alexandre Belot - Hôpital Femme Mère Enfant
Alice Berezne - Centre Hospitalier Annecy Genevois
Anne-Sophie Bouthors - Centre Hospitalier Universitaire de Lille
Geraldine Condette-Wojtasik - Centre Hospitalier Universitaire de Lille
Joël Constans - Centre Hospitalier Universitaire de Bordeaux
Pascal de Groote - Centre Hospitalier Universitaire de Lille
Elisabeth Diot - Centre Hospitalier Universitaire de Tours
Florence Dumas - Hôpital Cochin
Patrick Jego - Centre Hospitalier Universitaire de Rennes
Francisca Joly - Hôpital Beau-Séjour
David Launay - Institute for Translational Research in Inflammation
Veronique Le Guern - Laboratoire National de Référence
Janine-Sophie Le Quintrec - Hôpital Cochin
Géraldine Lescaille - Centre d'Immunologie et des Maladies Infectieuses
Christophe Meune - Hôpital Avicenne
Bruno Moulin - Hôpitaux Universitaires de Strasbourg
Christelle Nguyen - Hôpital Cochin
Nadine Omeish - Pitié-Salpêtrière Hospital
Frédéric Pene - Hôpital Cochin
Marie-Aleth Richard - Hôpital de la Timone
Juliette Rochefort - Pitié-Salpêtrière Hospital
Alexandra Roren - Centre de Recherche Épidémiologie et Statistique
Olivier Sitbon - Bicêtre Hospital
Vincent Sobanski - Centre Hospitalier Universitaire de Lille
Marie-Elise Truchetet - Centre Hospitalier Universitaire de Bordeaux
Luc Mouthon - Hôpital Cochin
Marc Bayen
Emmanuel Bergot
Sabine Berthier
Julia Bosco
Yoram Bouhnik
Benjamin Chaigne
Vincent Cottin - Centre Hospitalier Universitaire de Lille
Bruno Crestani - Hôpital Civil, Strasbourg
Christophe Deligny - Université Sorbonne Paris Nord
Vianney Descroix
Dominique Farge
Dominique Godard
Brigitte Granel - Hôpital Necker-Enfants Malades
Philippe Guilpain - Centre Hospitalier Régional Universitaire [Montpellier]
Bernard Imbert
Alain Le Quellec
Christophe Lega - Université Sorbonne Paris Nord
Catherine Lok
Hélène Maillard
Thierry Martin
Grégory Pugnet
Viviane Queyrel
Loïc Raffray
Frédéric Rilliard - Hôpital Cochin
Mélanie Romier
Laurence Schuller
Amélie Servettaz
Publication Details: Orphanet Journal of Rare Diseases, Vol.16(S2)
Identifiers: 9933634709311
Academic Unit: Cell souches plasticité cellul régénérat tissulaire immuno des maladies inflamm - IRMB
Language: English
Resource Type: Journal article
Local Fields: hal-03652133