Rare systemic autoimmune and autoinflammatory diseases

French national reference center for rare systemic autoimmune and autoinflammatory diseases in adults in Île-de-France, East and West

drapeau françaisThe coordinating site of the reference center for rare autoimmune and autoinflammatory systemic diseases in the Île-de-France, East and West of the Cochin hospital, headed by Pr Luc Mouthon since 2014, comprises the internal medicine, rheumatology and dermatology departments. It cares for patients with systemic scleroderma, systemic vasculitis, systemic lupus erythematosus, Sjögren syndrome, idiopathic inflammatory myopathy…

The reference center is located in the internal medicine department of Cochin hospital, headed by Pr Luc Mouthon, within the « rare diseases » functional unit. The center is housed in dedicated premises in the Saint Jacques building, and includes 2 examination chairs for taking samples and/or carrying out treatments within the framework of protocols, a reception/waiting room, a consultation cubicle, an office currently occupied by Pr Loïc Guillevin, who headed the reference center for 10 years, and two storage rooms with -80°C freezers (four freezers in one room and three in another, which house the reference center’s biotheque and protocols (PHRC)).
Patients with rare diseases are seen at the reference center in the Saint Jacques corridor or at the medical polyclinic in the Achard pavilion. They may be sampled during a consultation at the reference center. Depending on the context, they are cared for in day hospital, week hospital or conventional hospitalization.
The reference center also includes the rheumatology A department in the Hardy pavilion and the dermatology department in the Copernic pavilion at Port Royal.

Logo FAI2R

The Cochin reference center coordinates a network of 6 constituent sites: rheumatology department at Bicêtre hospital (Pr Xavier Mariette), internal medicine department at la Pitié Salpêtrière hospital (Pr Patrice Cacoub), internal medicine unit at Saint-Louis hospital (Pr Dominique Farge), nephrology department at Georges-Pompidou European hospital (Pr Alexandre Karras), internal medicine department at Dijon CHU (Pr Maxime Samson), and internal medicine department at Nantes CHU (Pr Christian Agard). There are also 13 competence centers : Annecy, Avicenne, Avignon, Henri Mondor, La Croix Saint Simon, La Réunion, Lariboisière, Orléans, Bichat, Perpignan, Saint Antoine, Saint Denis and Saint Etienne.

The Île-de-France reference center for rare autoimmune and autoinflammatory systemic diseases comprises a number of different activities. The systemic scleroderma activity is coordinated by Pr Yannick Allanore and Pr Luc Mouthon, while the vasculitis side is coordinated by Pr Benjamin Terrier at Cochin hospital (ANCA-positive vasculitis) and Pr Patrice Cacoub at La Pitié-Salpêtrière (cryoglobulinemia vasculitis). The systemic lupus and autoimmune diseases and pregnancy section is coordinated by Pr Nathalie Costedoat at Cochin, the Sjögren syndrome section is coordinated by Pr Xavier Mariette at Bicêtre and the cell therapy in autoimmune diseases section is coordinated by Pr Dominique Farge at Saint Louis hospital.

Logo RITAThis reference center is affiliated with the FAI2R rare diseases healthcare network and the RITA and ReCONNECT European reference networks (ERN).Logo Reconnet

> Learn more

Medical team

Pr Luc Mouthon

Pr Luc Mouthon
MD, PhD

Contact us

Monday to Friday 9 a.m. to 5 p.m.
Phone : +33 1 58 41 32 41

Outside these hours
Phone : +33 1 58 41 24 07

> Send an email

In case of emergency

The missions of the reference center for rare systemic autoimmune diseases in Île-de-France are:

  • to facilitate the diagnosis of rare systemic autoimmune diseases,
  • to improve the therapeutic management strategy for rare systemic autoimmune diseases through the implementation of therapeutic trials,
  • to optimize patient inclusion in existing databases,
  • to develop translational and epidemiological research,
  • to participate in training and information actions for healthcare professionals, patients and their families,
  • to lead and coordinate care networks for patients with rare autoimmune systemic diseases,
  • to develop therapeutic education

Systemic vasculitis

A heterogeneous group of diseases, systemic vasculitis include large vessel vasculitis, such as Takayasu’s arteritis or giant cell arteritis (Horton disease), vasculitis of the medium vessels such as polyarteritis nodosa, and finally vasculitis of the small vesselswhether associated with ANCA (anti-neutrophil cytoplasm antibodies) such as granulomatosis with polyangiitis (formerly Wegener’s disease), eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome) or microscopic polyangiitis, or to circulating immune complexes such as cryoglobulinemic vasculitis or IgA vasculitis. Other more polymorphic diseases such as Behçet’s disease are also classified as vasculitis.

These conditions are characterized by inflammatory involvement of arterial, capillary and/or venous blood vessels leading to damage to their walls, and ultimately to stenosis and occlusion of the vessels by thrombosis or thickening of the vessel wall.

Systemic vasculitis can be serious and life-threatening, depending on the different organs affected, but effective treatments do exist.


Site of the French group for the study of vasculitis (GFEV)

Website of the European Vasculitis Study Group, currently coordinated and directed by David Jayne, Cambridge, UK.

Systemic scleroderma

Systemic sclerosis is a generalized disorder of the connective tissue, in which dysfunctions of endothelial cells, fibroblasts, and cells of the immune system occur. Its prevalence is still poorly known, and is estimated in Europe at between 100 to 200 cases/million inhabitants. The number of cases in France is estimated at around 8000. There are two main forms, the diffuse cutaneous form characterized by extensive cutaneous sclerosis to the trunk, abdomen, and proximal parts of the limbs and the limited cutaneous form where the cutaneous sclerosis is more distal, not extending beyond the elbows and knees and touching neither the trunk nor the abdomen. There are rare « sine scleroderma » forms, without cutaneous sclerosis.

Diffuse cutaneous forms are more often associated with the presence of certain specific antibodies (anti-Scl70 antibodies, anti-RNA polymerase III antibodies) while limited cutaneous forms more often express anti-centromere antibodies.

The prognosis depends essentially on the presence of visceral involvement and in particular of diffuse infiltrating pneumonitis, pulmonary arterial hypertension (PAH), or scleroderma renal crisis. Cutaneous extension is also an important prognostic element.

Basic treatments can be proposed depending on the type of clinical presentation and any visceral damage.

The reference center for rare systemic autoimmune diseases in Île-de-France, coordinated by the internal medicine department of Cochin hospital, is a reference center for the diagnosis, management and follow-up of scleroderma patients. It has been organized since 2004 around several clinical and basic research and teaching activities.

The reference center initiates and collaborates in numerous observational and therapeutic clinical studies with, in particular, the French research group on systemic sclerosis (GFRS), the European research group on scleroderma (EUSTAR), and the support of the Association of French Scleroderma (ASF).


French Scleroderma Research Group website

National pulmonary arterial hypertension network website : PAH French network

European Scleroderma Research Group website : The EULAR Scleroderma Trials and Research group (EUSTAR)

European juvenile systemic sclerosis cohort website

Canadian Scleroderma Research Group (CSRG) website

Systemic lupus / antiphospholipid syndrome

A model of non-organ-specific autoimmune disease, systemic lupus is characterized by the association of widely varying clinical manifestations and almost constant biological abnormalities in the form of antibodies directed against various constituents of the cell nucleus (antinuclear antibodies ).

This disease, which is part of the national Rare Diseases plan, affects approximately one subject in 2000, 9 times out of 10 female.

It is potentially serious, especially when there is kidney damage or central neurological damage.

The diagnosis of systemic lupus justifies regular follow-up, both clinical and biological, by a specialist in this disease (rheumatologist, dermatologist, hematologist, nephrologist, specialist in internal medicine).

Anti-phospholipid syndrome, which is rarer, is marked by recurrent thrombotic events which may be venous (phlebitis, pulmonary embolism), arterial (cerebrovascular accidents, etc.) or obstetrical (miscarriages), linked to the presence of antibodies directed against membrane phospholipids. It is therefore also an autoimmune disease, which may or may not be associated with systemic lupus, and whose treatment is based above all, depending on the severity, on long-term anti-coagulant or anti-aggregant treatment.

Our reference center offers information to doctors treating patients and care for patients with systemic lupus or anti-phospholipid syndrome. We are also actively participating in various clinical and therapeutic studies in this area.

We also work in cooperation with patient associations :

Sjögren's syndrome

Primary Sjögren’s syndrome (SS) is a chronic autoimmune disease characterized by progressive destruction of the salivary and lacrimal glands leading to loss of secretory functions of the glands, with the main consequence being the onset of dryness of the glands. eyes and mouth. This disease can more rarely be complicated by damage to other organs, such as the peripheral nerves, the kidney, the lungs, the vessels, the joints.

It can be responsible, in addition to dryness, for pain and fatigue. The frequency of this disease is currently not fully known, but its management within the framework of the reference center for autoimmune diseases is largely justified because of its potential complications.

We offer, as part of the reference center, information for patients and doctors in charge of patients. We participate, as investigators, in several clinical and therapeutic trials, aimed at improving the understanding of this disease and the management of patients.

Finally, we work closely with the French association of Gougerot-Sjögren and Syndromes Secs.

Pregnancy and systemic diseases

Some autoimmune diseases are likely to worsen, sometimes to be revealed during pregnancy. These include, for example, systemic lupus, or anti-phospholipid syndrome, which are regularly associated.

Other autoimmune diseases, such as Sjögren’s syndrome, systemic sclerosis, inflammatory myopathies, necrotizing vasculitis, deserve special attention in the event of pregnancy. A pre-conception consultation is an essential step before any pregnancy in these patients. It makes it possible to check different aspects of the disease, before pregnancy, and to adapt the treatments so as to avoid any outbreak of the disease in the mother, without being toxic for the baby.

The follow-up of this particular pregnancy is fundamental, and must be carried out by both an obstetrician doctor and a doctor specializing in the underlying disease, who work in close collaboration, and throughout the duration of the pregnancy.

Within the reference center, we offer information oriented to patients and their families, to doctors in charge of patients.

Pre-conception consultations are provided at the reference center and/or at the Port-Royal maternity hospital by Dr Véronique Le Guern, specialist in internal medicine.

Obstetric follow-up is provided by obstetrician-gynecologists, and in particular by Dr Emmanuelle Pannier and Pr Vassili Tsatsaris.

> To make an appointment at the maternity ward

  • Pr Luc Mouthon – Internal medicine, head of the reference center
  • Dr Benjamin Chaigne – Internal medicine
  • Dr Pascal Cohen – Internal medicine
  • Pr Nathalie Costedoat-Chalumeau – Internal medicine
  • Dr Florence Delestre– Internal medicine
  • Dr Bertrand Dunogue – Internal medicine
  • Dr Anastasia Dupré– Internal medicine
  • Dr Gaëlle Guettrot-Imbert – Internal medicine
  • Pr Loïc Guillevin – Internal medicine
  • Dr Véronique Le Guern– Internal medicine
  • Dr Camille Mettler – Internal medicine
  • Pr Claire Le Jeunne– Internal medicine
  • Dr Nathalie Morel-Maillard – Internal medicine, TPE manager
  • Dr Xavier Puechal – Internal medicine
  • Pr Alexis Régent– Internal medicine
  • Dr Boris Sorin – Internal medicine
  • Pr Benjamin Terrier– Internal medicine, transition manager
  • Dr Benjamin Thoreau – Internal medicine
  • Pr Yannick Allanore – Rheumatologist
  • Pr Jérôme Avouac – Rheumatologist
  • Dr Roberto D’Alessandro – Rheumatologist
  • Pr Selim Aractingi – Dermatologist
  • Pr Nicolas Dupin – Dermatologist
  • Dr Nathalie Franck – Dermatologist
  • Pr Sarah Gueguan – Dermatologist

Sophie Hajjar – Health executive
Claude Parenti – Health executive
Valérie Almela – Research nurse
Sandrine Thiry – Medical secretary

RESEARCH

  • ETAP ClinicalTrials.gov. NCT01782235
  • CREHA ClinicalTrials.gov. NCT01808911
  • SPIRALE ClinicalTrials.gov. NCT02350491
  • ESBAM ClinicalTrials.gov. NCT02556866
  • WIN Lupus ClinicalTrials.gov. NCT01284725
  • TOCITAKA ClinicalTrials.gov. NCT02101333
  • ASSESS ClinicalTrials.gov. NCT03040583
  • DECOR ClinicalTrials.gov. NCT03187743
  • PERFOCTAPS ClinicalTrials.gov. NCT02719002
  • EGR2 ClinicalTrials.gov. NCT02450396
  • NOMADMUS ClinicalTrials.gov. NCT02850705
  • TIGRO ClinicalTrials.gov. NCT02892630
  • Base SAPL-LUPUS ClinicalTrials.gov. NCT02782039
  • CIRIS ClinicalTrials.gov. NCT03088293
  • SCLEROCYC ClinicalTrials.gov. NCT01570764
  • SPIN-HAND ClinicalTrials.gov. NCT03419208
  • SPIN-SSLED ClinicalTrials.gov. NCT03965780
  • CARESS 2 ClinicalTrials.gov. NCT01481662
  • MAINRITSEG ClinicalTrials.gov. NCT03164473
  • PIRFENIVAS ClinicalTrials.gov. NCT03385668
  • PNEUMOVAS ClinicalTrials.gov. NCT03069703
  • REOVAS ClinicalTrials.gov. NCT02807103
  • STATVAS ClinicalTrials.gov. NCT02117453
  • STELABEC ClinicalTrials.gov. NCT02648581
  • SPIN-SELF-F ClinicalTrials.gov. NCT03914781
  • Psy-LUP ClinicalTrials.gov. NCT03913754
  • MAINEPSAN ClinicalTrials.gov. NCT03290456
  • CINEVAS ClinicalTrials.gov. NCT03635385
  • METOGIA ClinicalTrials.gov. NCT03892785
  • COLCHIVAS ClinicalTrials.gov. NCT04008316
  • TINISS ClinicalTrials.gov. NCT03700138
  • RITUXGOPRO ClinicalTrials.gov. NCT03920722
  • TOCIAION ClinicalTrials.gov NCT04239196
  • INTORETAK ClinicalTrials.gov NCT04564001
  • FAMILYVASC ClinicalTrials.gov NCT04006535
  • VASCO ClinicalTrials.gov NCT04413331
  • ACOSTILL ClinicalTrials.gov non référencé
  • AFFEPI ClinicalTrials.gov NCT04155086
  • SURF ClinicalTrials.gov non référencé
  • CAPLAVIE ClinicalTrials.gov NCT04720261
  • MentCovid ClinicalTrials.gov NCT04510467

Our reference center handles large cohorts of patients for each of the pathologies covered by the FAI²R network, with the exception of autoinflammatory syndromes and pediatric forms. We plan to increase our patient recruitment in clinical studies and in our databases, and to expand our translational research.

  • UD in Systemic diseases
    University of Paris – Cochin site
  • UD in Immuno-hematology and internal medicine
    University of Paris – Cordeliers site
  • IUD in Targeted immunotherapies for inflammatory and autoimmune diseases
    University of Paris – Saint-Louis site
  • UD in Perinatal medicine
  • UD in Fetal Medicine

2022

Efficacy and safety of ustekinumab in Behçet disease: Results from the prospective phase 2 STELABEC trial.
Jonathan London, Alexis Régent, Jérémie Dion, Léa Jilet, Marie Jachiet, Olivier Lidove, Fleur Cohen-Aubart, Selim Aractingi, Sarah Guégan, Jean-Loup Pennaforte, Hendy Abdoul, Xavier Puéchal, Benjamin Terrier
J Am Acad Dermatol, 2022 Sep, PMID: 34864108 DOI: 10.1016/j.jaad.2021.11.045

Efficacy and safety of treatments in cutaneous polyarteritis nodosa: A French observational retrospective study.
Thomas Bettuzzi, Marie Jachiet, Emilie Sbidian, Laure Frumholtz, Florence Cordoliani, Luc Mouthon, François Chasset, Romain Paule, Jean-David Bouaziz, Loïc Guillevin, Benjamin Terrier, Selim Aractingi, Nicolas Dupin, Alexis Régent
J Am Acad Dermatol, 2022 May, PMID: 34224771 DOI: 10.1016/j.jaad.2021.06.872

 Presentation, Diagnosis, and Management of Subglottic and Tracheal Stenosis During Systemic Inflammatory Diseases.
Jennifer Catano, Yurdagul Uzunhan, Romain Paule, Jérémie Dion, Alexis Régent, Paul Legendre, François Gonin, Emmanuel Martinod, Pascal Cohen, Xavier Puéchal, Véronique Le Guern, Luc Mouthon, André Coste, Christine Lorut, Candice La Croix, Sophie Périé , Benjamin Terrier
Chest, 2022 Jan, PMID: 34324839 DOI: 10.1016/j.chest.2021.07.037

Do JAK inhibitors affect immune response to COVID-19 vaccination? Data from the MAJIK-SFR Registry.
Raphaèle Seror, Marine Camus, Jean-Hugues Salmon, Christian Roux, Emmanuelle Dernis, André Basch, Vincent Germain, Charles Leske, Steeve Brousseau, Marie-Elise Truchetet, André Ramon, Jacques-Eric Gottenberg, Renaud Felten, Fabienne Coury, Antoine Colombey, Clément Prati, Xavier Mariette, JérÔme Avouac
Lancet Rheumatol, 2022 Jan, PMID: 34642669 PMCID: PMC8494471 DOI: 10.1016/S2665-9913(21)00314-3

Independent Association Between Occupational Exposure and Decline of FVC in Systemic Sclerosis: A Multicenter Recruitment Retrospective Cohort Study.
Benjamin Thoreau, Marine Eustache, Adèle Fievet, Gérard Lasfargues, Laurent Plantier, Elisabeth Diot
Chest, 2022 Apr, PMID: 34793760 DOI: 10.1016/j.chest.2021.11.009

Severe COVID-19 is associated with hyperactivation of the alternative complement pathway.
Jeremy Boussier, Nader Yatim, Armance Marchal, Jérôme Hadjadj, Bruno Charbit, Carine El Sissy, Nicolas Carlier, Frédéric Pène, Luc Mouthon, Pierre-Louis Tharaux, Anne Bergeron, David M Smadja, Frédéric Rieux-Laucat, Darragh Duffy, Solen Kernéis, Véronique Frémeaux-Bacchi, Benjamin Terrier
J Allergy Clin Immunol, 2022 Feb, PMID: 34800432 PMCID: PMC8595971 DOI: 10.1016/j.jaci.2021.11.004

Immunogenicity of BNT162b2 vaccine against the Alpha and Delta variants in immunocompromised patients with systemic inflammatory diseases.
Jerome Hadjadj, Delphine Planas, Amani Ouedrani, Solene Buffier, Laure Delage, Yann Nguyen, Timothée Bruel, Marie-Claude Stolzenberg, Isabelle Staropoli, Natalia Ermak, Laure Macraigne, Caroline Morbieu, Soledad Henriquez, David Veyer, Hélène Péré, Marion Casadevall, Luc Mouthon, Frederic Rieux-Laucat, Lucienne Chatenoud, Olivier Schwartz, Benjamin Terrier
Ann Rheum Dis, 2022 May, PMID: 35022159 DOI: 10.1136/annrheumdis-2021-221508

Evaluation of Rituximab for Induction and Maintenance Therapy in Patients 75 Years and Older With Antineutrophil Cytoplasmic Antibody-Associated Vasculitis.
Sara Thietart, Alexandre Karras, Jean-François Augusto, Carole Philipponnet, Pierre-Louis Carron, Xavier Delbrel, Rafik Mesbah, Gilles Blaison, Pierre Duffau, Khalil El Karoui, Perrine Smets, Jonathan London, Luc Mouthon, Loïc Guillevin, Benjamin Terrier, Xavier Puéchal, French Vasculitis Study Group
JAMA Netw Open, 2022 Jul 1, PMID: 35802372 PMCID: PMC9270693 DOI: 10.1001/jamanetworkopen.2022.20925

Don »t mix apples and oranges. Reply to Azoulay et al.
Clara Vigneron, Luc Mouthon, Benjamin Chaigne
Autoimmun Rev, 2022 Nov, PMID: 36096469 DOI: 10.1016/j.autrev.2022.103189

Rituximab vs Cyclophosphamide Induction Therapy for Patients With Granulomatosis With Polyangiitis.
Xavier Puéchal, Michele Iudici, Elodie Perrodeau, Bernard Bonnotte, François Lifermann, Thomas Le Gallou, Alexandre Karras, Claire Blanchard-Delaunay, Thomas Quéméneur, Achille Aouba, Olivier Aumaître, Vincent Cottin, Mohamed Hamidou, Marc Ruivard, Pascal Cohen, Luc Mouthon, Loïc Guillevin, Philippe Ravaud, Raphaël Porcher, Benjamin Terrier; French Vasculitis Study Group
JAMA Netw Open, 2022 Nov 1, PMID: 36441554 PMCID: PMC9706346 DOI: 10.1001/jamanetworkopen.2022.43799

2021

Significance of PR3-ANCA positivity in eosinophilic granulomatosis with polyangiitis (Churg-Strauss).
Matthias Papo, Renato A. Sinico, Vítor Teixeira, Nils Venhoff, Maria-Letizia Urban, Michele Iudici, Juliane Mahrhold, Francesco Locatelli, Giulia Cassone, Franco Schiavon, Benjamin Seeliger, Thomas Neumann, Claus Kroegel, Matthieu Groh, Chiara Marvisi, Maxime Samson, Thomas Barba, David Jayne, Arianna Troilo, Jens Thiel, Bernhard Hellmich, Sara Monti, Carlomaurizio Montecucco, Carlo Salvarani, Jean-Emmanuel Kahn, Bernard Bonnotte, Cécile-Audrey Durel, Xavier Puéchal, Luc Mouthon, Loïc Guillevin, Giacomo Emmi, Augusto Vaglio, Benjamin Terrier, the French Vasculitis Study Group and the EGPA European Study Group
Rheumatology (Oxford), 2021 Sep 1, PMID: 33347592 DOI: 10.1093/rheumatology/keaa805

Severe COVID-19-associated pneumonia in 3 patients with systemic sclerosis treated with rituximab.
Jérôme Avouac, Paolo Airó, Nicolas Carlier, Marco Matucci-Cerinic, Yannick Allanore
Ann Rheum Dis, 2021 Mar, PMID: 32503849 DOI: 10.1136/annrheumdis-2020-217864

Temporal Arteritis Revealing Antineutrophil Cytoplasmic Antibody-Associated Vasculitides: A Case-Control Study.
Laure Delaval, Maxime Samson, Flora Schein, Christian Agard, Ludovic Tréfond, Alban Deroux, Henry Dupuy, Cyril Garrouste, Pascal Godmer, Cédric Landron et al.
Arthritis Rheumatol, 2021 Feb, PMID: 32951354 DOI: 10.1002/art.41527

Use of Biologics to Treat Relapsing and/or Refractory Eosinophilic Granulomatosis With Polyangiitis: Data From a European Collaborative Study.
Alice Canzian, Nils Venhoff, Maria Letizia Urban, Silvia Sartorelli, Anne-Marie Ruppert, Matthieu Groh, Nicolas Girszyn, Camille Taillé, François Maurier, Vincent Cottin, Claire de Moreuil, Vincent Germain et al.
Arthritis Rheumatol, 2021 Mar, PMID: 33001543 DOI: 10.1002/art.41534

Sustained Remission of Granulomatosis With Polyangiitis After Discontinuation of Glucocorticoids and Immunosuppressant Therapy: Data From the French Vasculitis Study Group Registry.
Xavier Puéchal, Michele Iudici, Christian Pagnoux, Alexandre Karras, Pascal Cohen, François Maurier, Thomas Quéméneur, François Lifermann, Mohamed Hamidou, Luc Mouthon, Benjamin Terrier, Loïc Guillevin, French Vasculitis Study Group
Arthritis Rheumatol, 2021 Apr, PMID: 33029946 DOI: 10.1002/art.41551

Different Control Populations May Lead to Different Understanding of Hydroxychloroquine Blood Levels as a Risk Factor for Retinopathy: Comment on the Article by Petri et al.
Tiphaine Lenfant, Gaëlle Leroux, Nathalie Costedoat-Chalumeau
Arthritis Rheumatol, 2021 Apr, PMID: 33191634 DOI: 10.1002/art.41588

Drug-induced IgA vasculitis in children and adults: Revisiting drug causality using a dual pharmacovigilance-based approach.
Camille Rasmussen, Mylène Tisseyre, Julie Garon-Czmil, Marina Atzenhoffer, Loic Guillevin, Joe-Elie Salem, Jean-Marc Treluyer, Benjamin Terrier, Laurent Chouchana
Autoimmun Rev, 2021 Jan, PMID: 33197572 DOI: 10.1016/j.autrev.2020.102707

Improving medication adherence in patients with lupus nephritis.
Nathalie Costedoat-Chalumeau, Frederic A Houssiau
Kidney Int, 2021 Feb, PMID: 33509345 DOI: 10.1016/j.kint.2020.10.037

Primary biliary cholangitis and systemic sclerosis (Reynolds syndrome): A case-control study.
Clémence David, Benjamin Chaigne, Clémence Hollande, Benoit Terris, Pascal Cohen, Bertrand Dunogue, Benjamin Terrier, Philippe Sogni, Claire Goulvestre, Luc Mouthon
Autoimmun Rev, 2021 Jul, PMID: 33971338 DOI: 10.1016/j.autrev.2021.102842

Ocular Safety Profile of BRAF and MEK Inhibitors: Data from the World Health Organization Pharmacovigilance Database.
Camille Mettler, Dominique Monnet, Nora Kramkimel, Jean-Marc Tréluyer, Luc Mouthon, Antoine Brézin, Nicolas Dupin, Marie-Blanche Valnet-Rabier, Laurent Chouchana, Benjamin Terrier
Ophthalmology, 2021 Dec, PMID: 34000304 DOI: 10.1016/j.ophtha.2021.05.008

Nonsystemic vasculitic neuropathy: Presentation and long-term outcome from a French cohort of 50 patients.
Marion Quirins, Marie Théaudin, Fleur Cohen-Aubart, Alain Créange, Luc Mouthon, Solène Genty, Jean-Emmanuel Kahn, Alice Bérezné, Aude Rigolet, David Adams, Clovis Adam, Zahir Amoura, Olivier Benveniste, François-Jérôme Authier, Loïc Guillevin, Thierry Maisonobe, Benjamin Terrier, French Vasculitis Study Group (FVSG)
Autoimmun Rev, 2021 Aug, PMID: 34118457 DOI: 10.1016/j.autrev.2021.102874

2020

Comment on the 2019 update of the EULAR recommendations for the management of systemic lupus erythematosus by Fanouriakis  et al.
Nathalie Costedoat-Chalumeau, David Isenberg, Michelle Petri
Ann Rheum Dis, 2020 Aug, PMID: 31048291 DOI: 10.1136/annrheumdis-2019-215573

Estrogens Counteract the Profibrotic Effects of TGF-β and their Inhibition Exacerbates Experimental Dermal Fibrosis.
Jérôme Avouac, Sonia Pezet, Virginie Gonzalez, Léa Baudoin, Anne Cauvet, Barbara Ruiz, Gonçalo Boleto, Marie Laure Brandely, Manon Elmerich, Yannick Allanore
J Invest Dermatol, 2020 Mar, PMID: 31476316 DOI: 10.1016/j.jid.2019.07.719

Risk of malignancy in patients treated for systemic necrotising vasculitis.
Antoine Lafarge, Adrien Joseph, Christian Pagnoux, Xavier Puechal, Pacal Cohen, Maxime Samson 6, Mohamed Hamidou, Alexandre Karras, Matthieu Groh, Thomas Quemeneur, Camillo Ribi, Luc Mouthon, Loic Guillevin, Benjamin Terrier, French Vasculitis Study Group (FVSG)
Ann Rheum Dis, 2020 Mar, PMID: 31767700 DOI: 10.1136/annrheumdis-2019-216452

Predictive factors of severe infections in patients with systemic necrotizing vasculitides: data from 733 patients enrolled in five randomized controlled trials of the French Vasculitis Study Group.
Antoine Lafarge, Adrien Joseph, Christian Pagnoux, Xavier Puéchal, Pascal Cohen, Maxime Samson, Mohamed Hamidou, Alexandre Karras, Thomas Quemeneur, Camillo Ribi, Matthieu Groh, Luc Mouthon, Loïc Guillevin, Benjamin Terrier, for the French Vasculitis Study Group (FVSG)
Rheumatology (Oxford), 2020 Sep 1, PMID: 31782786 DOI: 10.1093/rheumatology/kez575

Incidence and risk factors for gangrene in patients with systemic sclerosis from the EUSTAR cohort.
Carina Mihai, Oliver Distler, Ana Maria Gheorghiu, Paul I Constantin, Rucsandra Dobrota, Suzana Jordan, Vanessa Smith, Eric Hachulla, Jörg Henes, Elise Siegert, Serena Vettori, Ulf Müller-Ladner, Marco Matucci Cerinic, Yannick Allanore, EUSTAR collaborators
Rheumatology (Oxford), 2020 Aug 1, PMID: 31790136 DOI: 10.1093/rheumatology/kez558

Gastrointestinal involvement in adult IgA vasculitis (Henoch-Schönlein purpura): updated picture from a French multicentre and retrospective series of 260 cases.
Alexandra Audemard-Verger, Evangéline Pillebout, Zahir Amoura, Patrice Cacoub, Noémie Jourde-Chiche, Bertrand Lioger, Nihal Martis, Guillaume Moulis, Etienne Rivière, Aurélie Baldolli, Charlotte Girard, Julie Goutte, Noémie Le Gouellec, Loïc Raffray, Geoffrey Urbanski, Sébastien Sanges, Francois Maurier, Eric Thervet, Achille Aouba, Loïc Guillevin, Francois Maillot, Benjamin Terrier, French Vasculitis Study Group (FVSG)
Rheumatology (Oxford), 2020 Oct 1, PMID: 32211770 DOI: 10.1093/rheumatology/keaa104

Cardiac Intravoxel Incoherent Motion Diffusion-Weighted Magnetic Resonance Imaging With T1 Mapping to Assess Myocardial Perfusion and Fibrosis in Systemic Sclerosis: Association With Cardiac Events From a Prospective Cohort Study.
Benjamin Terrier,Agnès Dechartres,Hervé Gouya,Moncef Ben Arfi,Alice Bérézne,Alexis Régent,Bertrand Dunogué,Jonathan London,Pascal Cohen,Loïc Guillevin,Claire Le Jeunne,Paul Legmann,Olivier Vignaux,Luc Mouthon
Arthritis Rheumatol, 2020 Sep, PMID: 32379399 DOI: 10.1002/art.41308

Risk factors for hydroxychloroquine retinopathy in systemic lupus erythematosus: a case-control study with hydroxychloroquine blood-level analysis.
Tiphaine Lenfant, Sawsen Salah, Gaëlle Leroux, Elodie Bousquet, Véronique Le Guern, François Chasset, Camille Francès, Nathalie Morel, Julie Chezel, Thomas Papo, Patrice Cacoub, Luc Mouthon, Gaëlle Guettrot-Imbert, Pascal Cohen, Alexis Régent, Martine Mauget-Faÿsse, Jean-Charles Piette, Moez Jallouli, Nathalie Costedoat-Chalumeau, PLUS Group
Rheumatology (Oxford), 2020 Dec 1, PMID: 32442312 PMCID: PMC8186841 DOI: 10.1093/rheumatology/keaa157

Handgrip strength is a comorbidity marker in systemic necrotizing vasculitides and predicts the risk of fracture and serious adverse events.
Soledad Henriquez, Bertrand Dunogué, Raphael Porcher, Alexis Régent, Pascal Cohen, Alice Berezne, Sami Kolta, Claire Le Jeunne, Luc Mouthon, Christian Roux, Loïc Guillevin, Karine Briot, Benjamin Terrier, for the French Vasculitis Study Group (FVSG)
Rheumatology (Oxford), 2020 Sep 1, PMID: 32449923 DOI: 10.1093/rheumatology/kez680

Long-Term Rituximab Use to Maintain Remission of Antineutrophil Cytoplasmic Antibody-Associated Vasculitis: A Randomized Trial.
Pierre Charles, MD, Élodie Perrodeau, MSc, Maxime Samson, MD, PhD, Bernard Bonnotte, MD, PhD, Antoine Néel, MD, PhD, Christian Agard, MD, PhD, Antoine Huart, MD, Alexandre Karras, MD, PhD, François Lifermann, MD, Pascal Godmer, MD, Pascal Cohen, MD, Catherine Hanrotel-Saliou, MD, Nicolas Martin-Silva, MD, Grégory Pugnet, MD, PhD, François Maurier, MD, Jean Sibilia, MD, PhD, Pierre-Louis Carron, MD, Pierre Gobert, MD, Nadine Meaux-Ruault, MD, Thomas Le Gallou, MD, Stéphane Vinzio, MD, Jean-François Viallard, MD, PhD, Eric Hachulla, MD, PhD, Christine Vinter, MD, Xavier Puéchal, MD, PhD, Benjamin Terrier, MD, PhD, Philippe Ravaud, MD, PhD, Luc Mouthon, MD, PhD, Loïc Guillevin, MD, for the French Vasculitis Study Group*
Ann Intern Med, 2020 Aug 4, PMID: 32479166 DOI: 10.7326/M19-3827

Severe COVID-19-associated pneumonia in 3 patients with systemic sclerosis treated with rituximab.
Jérôme Avouac, Paolo Airó, Nicolas Carlier, Marco Matucci-Cerinic, Yannick Allanore
Ann Rheum Dis, 2020 Jun 5, PMID: 32503849 DOI: 10.1136/annrheumdis-2020-217864

Comment on: Gastrointestinal involvement in adult IgA vasculitis (Henoch-Schönlein purpura): updated picture from a French multicentre and retrospective series of 260 cases: reply.
Alexandra Audemard-Verger, Benjamin Terrier
Rheumatology (Oxford), 2020 Oct 1, PMID: 32617560 DOI: 10.1093/rheumatology/keaa261

A randomised, double-blind, placebo-controlled, 24-week, phase II, proof-of-concept study of romilkimab (SAR156597) in early diffuse cutaneous systemic sclerosis.
Yannick Allanore, Peter Wung, Christina Soubrane, Corinne Esperet, Frederic Marrache, Raphael Bejuit, Amel Lahmar, Dinesh Khanna, Christopher P Denton, Investigators
Ann Rheum Dis, 2020 Dec, PMID: 32963047 PMCID: PMC7677494 DOI: 10.1136/annrheumdis-2020-218447

2019

Controlling the Diffusion of Multidrug-Resistant Organisms in Intensive Care Units.
Kernéis, S ; Lucet, JC
Semin Respir Crit Care Med, DOI 10.1055/s-0039-1696980, PMID 31585481

Long-term safety and vaccine-induced seropositivity in healthy volunteers from HIV vaccine trials.
Durier, C ; Desaint, C ; Lelièvre, JD ; Silbermann, B ; Pialoux, G ; Cuzin, L & al
AIDS, 2019 Nov 1, PMID: 31306171 DOI: 10.1097/QAD.0000000000002310

A generalized additive model to disentangle age and diagnosis-specific cohort effects in psychological and behavioral outcomes in people living with HIV: the French cross-sectional ANRS-VESPA2 survey.
Sagaon-Teyssier, L ; Vilotitch, A ; Mora, M ; Maradan, G ; Guagliardo, V ; Suzan-Monti, M & al
BMC Public Health, 2019 May 17, PMID: 31101129 PMCID: PMC6525437 DOI: 10.1186/s12889-019-6905-z

Women in internal medicine academic positions in France.
Cohen Aubart, F ; Roeser, A ; Costedoat-Chalumeau, N ; Papo, T ; Piette, JC
Eur J Intern Med, 2019 Jun, PMID: 30981614 DOI: 10.1016/j.ejim.2019.04.002

[Reliability and validity of a workbook for assessment of professional competencies of internal medicine residents].
Bonnard, G ; Cohen-Aubart, F ; Steichen, O ; Bourgarit, A ; Abad, S ; Ranque, B & al
Rev Med Interne, 2019 Jul, PMID: 30871866 DOI: 10.1016/j.revmed.2019.02.003

Epidemiology and Appropriateness of Antibiotic Prescribing in Severe Pneumonia After Lung Resection.
Kernéis, S ; Blanc, K ; Caliez, J ; Canouï, E ; Loubinoux, J ; Gauzit, R & al
Ann Thorac Surg, 2019 Jul, PMID: 30853591 DOI: 10.1016/j.athoracsur.2019.01.072

High-dose chemotherapy without transfusion for Philadelphia chromosome negative B-cell acute lymphoblastic leukemia in two Jehovah »s Witnesses patients: a feasible option in the age of hematopoietic growth factors.
Perol, L ; Grignano, E ; Contejean, A ; Gastaud, L ; Legoff, M ; Franchi, P & al
Leuk Lymphoma, 2019 Sep, PMID: 30773115 DOI: 10.1080/10428194.2019.1577414

Early access to health products in France: Major advances of the French \ »Conseil stratégique des industries de santé\ » (CSIS) to be implemented (modalities, regulations, funding).
Albin, N ; Chassagnol, F ; Bergmann, JF
Therapie, 2019 Feb, PMID: 30745156 DOI: 10.1016/j.therap.2018.12.002

Surgery is safe and effective when indicated in the acute phase of hematogenous pyogenic vertebral osteomyelitis.
Canouï, E ; Zarrouk, V ; Canouï-Poitrine, F ; Desmoulin, U ; Leflon, V ; Allaham, W & al
Infect Dis (Lond), 2019 Apr, PMID: 30729831 DOI: 10.1080/23744235.2018.1562206

A 5-day course of oral antibiotics followed by faecal transplantation to eradicate carriage of multidrug-resistant Enterobacteriaceae: a randomized clinical trial.
Huttner, BD ; de Lastours, V ; Wassenberg, M ; Maharshak, N ; Mauris, A ; Galperine, T & al
Clin Microbiol Infect, 2019 Jul, PMID: 30616014 DOI: 10.1016/j.cmi.2018.12.009

Acute paraplegia due to schistosomiasis: an uncommon cause in developed countries.
Chauvin, A ; Ghazali, A ; Le Jeunne, C ; Plaisance, P ; Szwebel, TA ; Costedoat-Chalumeau, N & al
J Neurovirol, 2019 Jun, PMID: 30610737 DOI: 10.1007/s13365-018-0713-6

[History and principles of vaccination].
Canouï, E ; Launay, O
Rev Mal Respir, 2019 Jan, PMID: 30579659 DOI: 10.1016/j.rmr.2018.02.015

Assessing the benefit of the 72-hour antibiotic therapy reassessment documentation.
Pérut, V ; Gauzit, R ; Kernéis, S ; Canouï, E ; Chedhomme, FX ; Batista, R & al
Med Mal Infect, 2019 May, PMID: 30420165 DOI: 10.1016/j.medmal.2018.10.002

ESCMID generic competencies in antimicrobial prescribing and stewardship: towards a European consensus.
Dyar, OJ ; Beović, B ; Pulcini, C ; Tacconelli, E ; Hulscher, M ; Cookson, B
Clin Microbiol Infect, 2019 Jan, PMID: 30414817 DOI: 10.1016/j.cmi.2018.09.022

Clinical Significance of Upper Airway Virus Detection in Critically Ill Hematology Patients.
Legoff, J ; Zucman, N ; Lemiale, V ; Mokart, D ; Pène, F ; Lambert, J & al
Clin Microbiol Infect, 2019 Jan, PMID: 30414817 DOI: 10.1016/j.cmi.2018.09.022

Reply to the comment of Fayet et al. Obstacles and motivations to influenza and pneumococcal vaccination in patients with rheumatoid arthritis. A qualitative study.
Assala, M ; Groh, M ; Blanche, P ; Vinter, C ; Cohen, P ; Le Guern, V & al
Joint Bone Spine, 2019 Jan, PMID: 30053610 DOI: 10.1016/j.jbspin.2018.06.015

Abrupt and unexpected stressful life events are followed with increased disease activity in spondyloarthritis: A two years web-based cohort study.
Luo, G ; Boelle, PY ; Turbelin, C ; Costantino, F ; Kerneis, S ; Said Nahal, R & al
Joint Bone Spine, 2019 Mar, PMID: 29883766 DOI: 10.1016/j.jbspin.2018.05.009

2022

  • Performance of models predicting the risk of relapse and severe infection at the end of 18-month rituximab maintenance therapy in ANCA vasculitis. Florence Delestre, P. Charles, M. Samson, A. Néel, S. Faguer, A. Karras, F. Lifermann, P. Godmer, C. Hanrotel-Saliou, N. Martin-Silva, G. Pugnet, F. Maurier, T. Le Gallou, T. Quéméneur, N. Méaux-Ruault, J.F. Viallard, X. Puéchal, L. Guillevin, R. Porcher, B. Terrier. SNFMI Nantes June 29, 2022.
  • Study of granulomatosis with polyangiitis refractory to induction therapy. Speaker : Boris Sorin, M. Iudici, M.J. Guerry, M. SAMSON, P. Bielefeld, T. Maillet, M. Nouvier, A. Karras, L. Christian, C.A. Durel, M. Fabre, P. Charles, A. Lanteri, G. Pugnet, F. Riviere, G. Le Gueno, L. Guillevin, X. Puéchal, B. Terrier. SNFMI Nantes June 29, 2022.
  • Safety and efficacy of dupilumab in refractory eosinophilic granulomatosis with polyangiitis (EGPA). B. Molina, M.L. Urban, R. Padoan, P. Novikov, L. Bouillet, M. Caminati, C. Taillé, M. Hamidou, P. Fraticeli, N. Schleinitz, C. Christides, L. Moi, B. Godeau, A. Knight, J.W. Schroder, S. Marchand-Adam, H. Gil, V. Cottin, C.A. Durel, B. Terrier. SNFMI Nantes June 29, 2022.
  • Systemic Scleroderma Reference Center Workshop – May 18-19, 2022 – Luc Mouthon – Paris Cochin – Physicians.

2021

  • Antimalarials are useful but rather weak medications  for SLE. Nathalie Costedoat Chalumeau. 6th International  Congress on Controversies in Rheumatology and Autoimmunity (CORA) and 14th International  Congress on SLE. Distanciel.
  • Managing patient compliance in Lupus. Nathalie Costedoat Chalumeau. New York University (NYU) Langone Seminar in  Advanced Rheumatology (Virtuel). 18/03/2021.
  • Systemic scleroderma reference center workshop – February 5-6, 2021 – Luc Mouthon – Lille – Physicians.
  • Systemic scleroderma reference center workshop – May 19-20, 2021 – Luc Mouthon – Paris Cochin – Physicians.
  • Annual meeting. French-speaking research group on systemic scleroderma – November 19, 2021 – Online – Luc Mouthon – Physicians – Researchers.

2020

  • RITA Meeting – 17 – 18 Janvier 2020. Utrecht – Luc Mouthon – ERN RITA – Physicians.

2019

  • Annual day of the France Vasculites association. March 23, 2019. Therapeutic perspectives in systemic vasculitis. Cochin Hospital. Professor Loic Guillevin. Patient audience.
  • Scleroderma day. Meeting organized by the association of scleroderma in France and the reference center for rare systemic autoimmune diseases in Ile-de-France. Monday, June 24, 2019. Cochin Hospital. Digestive disorders of systemic sclerosis. Professor Luc Mouthon. Patient audience.
  • Annual day of the reference center for rare systemic autoimmune diseases in Ile de France. October 11, 2019. News on clinical trials in Scleroderma (Yannick ALLANORE). 140 doctors / reference centers / competence center / expert center / ARCS.
  • Annual day of the reference center for rare systemic autoimmune diseases in Ile de France. October 11, 2019. Recommendations and news in Antiphospholipid syndrome (Nathalie COSTEDOAT-CHALUMEAU)
  • Annual day of the reference center for rare systemic autoimmune diseases in Ile de France. October 11, 2019. Pathophysiological advances and therapeutic perspectives in ANCA vasculitis (Benjamin TERRIER)

Lupus
RFI | 09.20.2023
Lupus exists in two forms: cutaneous lupus, which mainly affects the skin, and systemic lupus, a chronic autoimmune disease that can affect any organ or tissue. This second form of lupus affects between 20,000 and 40,000 people in France, making it one of the most common rare diseases. Of every 10 people affected, 9 are women.
> Listen to the podcast

Therapeutic patient education
RFI | 07.19.2023
According to the WHO, therapeutic patient education (TPE) « aims to help patients acquire or maintain the skills they need to manage their lives as effectively as possible with a chronic disease ». Since 2009, this concept has been enshrined in law in France and is part of the care pathway for many diseases. It involves training provided by specific healthcare professionals, as well as other approved players, to the patients concerned.
> Listen to the podcast

Lupus: what are the symptoms ?
E-santé.fr | 12.14.2017
Lupus is a chronic autoimmune disease. It is estimated that about 30,000 people in France are affected by the disease. What are the symptoms? Explanations with Pr Nathalie Costedoat-Chalumeau, specialist in rare systemic autoimmune diseases at the Cochin hospital.
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Bandeau Vidéo Costedoat MAIS

Hydroxychloroquine (HCQ) and pregnancy: what are the practical implications? Let’s take stock with Pr Nathalie Costedoat-Chalumeau (Ile-de-France Reference center for rare systemic autoimmune and autoinflammatory  diseases, internal medicine department, Cochin hospital, Paris), interviewed by Pr Eric Hachulla (FAI²R coordinator).

How to diagnose and manage rare systemic autoimmune diseases ?
France Culture | 02.26.2024
At the Ile-de-France reference center for rare systemic autoimmune diseases at Cochin Hospital in Paris, Pr Luc Mouthon, head of the internal medicine department and coordinator of the center, and his team welcome and support patients throughout their treatment.
> Listen to the podcast

Contact information

Cochin hospital
> Internal medicine department

27 rue du faubourg Saint-Jacques
75014 Paris

> Welcome booklet

 

At Cochin hospital, the reference center for rare systemic autoimmune and autoinflammatory diseases in brief …

0
patients followed at least once a year*
0
medical consultations*
0
day hospitalizations*
0
fully hospitalized patients*
0
authorized therapeutic education programs*
0
patients trained in therapeutic education*
0
ongoing research projects*
0
university courses*
0
publications*
0
teleconsultation procedures*

* data valid for 2022