Title :

Clinical presentation, course, and prognosis of patients with mixed connective tissue disease: A multicenter retrospective cohort.

Author(s) :

Chevalier, Kevin [Auteur]
Hôpital Cochin [AP-HP]
Thoreau, Benjamin [Auteur]
Hôpital Cochin [AP-HP]
Michel, Marc [Auteur]
Hôpital Henri Mondor
Godeau, Bertrand [Auteur]
Hôpital Henri Mondor
Agard, Christian [Auteur]
Université de Nantes [UN]
Papo, Thomas [Auteur]
AP-HP - Hôpital Bichat - Claude Bernard [Paris]
Sacre, Karim [Auteur]
AP-HP - Hôpital Bichat - Claude Bernard [Paris]
Seror, Raphaèle [Auteur]

Mariette, Xavier [Auteur]

Cacoub, Patrice [Auteur]
CHU Pitié-Salpêtrière [AP-HP]
Benhamou, Ygal [Auteur]
CHU Rouen
Levesque, Hervé [Auteur]
CHU Rouen
Goujard, Cécile [Auteur]

Lambotte, Olivier [Auteur]

Bonnotte, Bernard [Auteur]
CHU Dijon
Samson, Maxime [Auteur]
CHU Dijon
Ackermann, Félix [Auteur]
Hôpital Foch [Suresnes]
Schmidt, Jean [Auteur]
CHU Amiens-Picardie
Duhaut, Pierre [Auteur]
CHU Amiens-Picardie
Kahn, Jean-Emmanuel [Auteur]
Université de Versailles Saint-Quentin-en-Yvelines [UVSQ]
Hanslik, Thomas [Auteur]
Université de Versailles Saint-Quentin-en-Yvelines [UVSQ]
Costedoat-Chalumeau, Nathalie [Auteur]
Hôpital Cochin [AP-HP]
Terrier, Benjamin [Auteur]
Hôpital Cochin [AP-HP]
Regent, Alexis [Auteur]
Hôpital Cochin [AP-HP]
Dunogue, Bertrand [Auteur]
Hôpital Cochin [AP-HP]
Cohen, Pascal [Auteur]
Hôpital Cochin [AP-HP]
Le Guern, Véronique [Auteur]
Hôpital Cochin [AP-HP]
Hachulla, Eric [Auteur]
Institut de Recherche Translationnelle sur l'Inflammation (INFINITE) - U1286
Chaigne, Benjamin [Auteur]
Hôpital Cochin [AP-HP]
Mouthon, Luc [Auteur]
Hôpital Cochin [AP-HP]

Journal title :

Journal of Internal Medicine

Abbreviated title :

J Intern Med

Publication date :

2023-11-29

ISSN :

1365-2796

HAL domain(s) :

Sciences du Vivant [q-bio]

English abstract : [en]

Objectives The objective of this study is to better characterize the features and outcomes of a large population of patients with mixed connective tissue disease (MCTD). Methods We performed an observational ...
Show more >Objectives The objective of this study is to better characterize the features and outcomes of a large population of patients with mixed connective tissue disease (MCTD). Methods We performed an observational retrospective multicenter cohort study in France. Patients who fulfilled at least one diagnostic criterion set for MCTD and none of the criteria for other differentiated CTD (dCTD) were included. Results Three hundred and thirty patients (88% females, median [interquartile range] age of 35 years [26–45]) were included. The diagnostic criteria of Sharp or Kasukawa were met by 97.3% and 93.3% of patients, respectively. None met other classification criteria without fulfilling Sharp or Kasukawa criteria. After a median follow-up of 8 (3–14) years, 149 (45.2%) patients achieved remission, 92 (27.9%) had interstitial lung disease, 25 (7.6%) had pulmonary hypertension, and 18 (5.6%) died. Eighty-five (25.8%) patients progressed to a dCTD, mainly systemic sclerosis (15.8%) or systemic lupus erythematosus (10.6%). Median duration between diagnosis and progression to a dCTD was 5 (2–11) years. The presence at MCTD diagnosis of an abnormal pattern on nailfold capillaroscopy (odds ratio [OR] = 2.44, 95% confidence interval [95%CI] [1.11–5.58]) and parotid swelling (OR = 3.86, 95%CI [1.31–11.4]) were statistically associated with progression to a dCTD. Patients who did not progress to a dCTD were more likely to achieve remission at the last follow-up (51.8% vs. 25.9%). Conclusions This study shows that MCTD is a distinct entity that can be classified using either Kasukawa or Sharp criteria, and that only 25.8% of patients progress to a dCTD during follow-up.Show less >

Language :

Anglais

Audience :

Internationale

Popular science :

Non

Administrative institution(s) :

Université de Lille
Inserm
CHU Lille

Collections :

• Institut de Recherche Translationnelle sur l'Inflammation (INFINITE) - U1286

Submission date :

2024-02-06T22:18:02Z
2024-03-19T09:30:04Z