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Article Dans Une Revue RMD Open Année : 2023

In inflammatory myopathies, dropped head/bent spine syndrome is associated with scleromyositis: an international case–control study

1 UNISTRA - Université de Strasbourg
2 HUS - Les Hôpitaux Universitaires de Strasbourg
3 CHU de Québec–Université Laval
4 University of Pisa - Università di Pisa
5 AOUC - Azienda Ospedaliero Universitaria Careggi [Firenze] = Careggi University Hospital [Florence, Italy]
6 Unipd - Università degli Studi di Padova = University of Padua
7 UNIPV - Università degli Studi di Pavia = University of Pavia
8 Fondazione IRCCS Policlinico San Matteo [Pavia]
9 IMoST - Imagerie Moléculaire et Stratégies Théranostiques
10 Service de Rhumatologie [CHU Clermont-Ferrand]
11 CHRU Brest - Centre Hospitalier Régional Universitaire de Brest
12 Centre Hospitalier Le Mans (CH Le Mans)
13 Service de Médecine Interne [CHU Clermont-Ferrand]
14 Service de neurologie [Bordeaux]
15 Centre Hospitalier de Charleville-Mezières
16 CH Colmar
17 CHU ST-E - Centre Hospitalier Universitaire de Saint-Etienne [CHU Saint-Etienne]
18 CHU Montpellier
19 AP-HP - Assistance publique - Hôpitaux de Paris (AP-HP)
20 Jewish General Hospital
21 McGill University = Université McGill [Montréal, Canada]
22 UNIBA - Università degli studi di Bari Aldo Moro = University of Bari Aldo Moro
23 HPMetz - Hôpitaux Privés de Metz
24 Hospital of Prato
25 Clinic Barcelona Hospital Universitari
26 CHU Bordeaux
27 Vall d'Hebron University Hospital [Barcelona]
28 Hôpital Cochin [AP-HP]
29 Hôpital du Sacré-Coeur de Montréal
30 UdeM - Université de Montréal
31 CHU Dijon - Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand
32 CH Narbonne - Centre Hospitalier de Narbonne
Monica Groza
  • Fonction : Auteur
Nicolas Poursac
  • Fonction : Auteur
Benjamin Terrier
Rahima Ziane
  • Fonction : Auteur

Résumé

Background: Some myopathies can lead to dropped head or bent spine syndrome (DH/BS). The significance of this symptom has not been studied in inflammatory myopathies (IM). Objectives: To assess the significance of DH/BS in patients with IM. Methods: Practitioners from five IM networks were invited to report patients with IM suffering from DH/BS (without other known cause than IM). IM patients without DH/BS, randomly selected in each participating centre, were included as controls at a ratio of 2 to 1. Results: 49 DH/BS-IM patients (DH: 57.1%, BS: 42.9%) were compared with 98 control-IM patients. DH/BS-IM patients were older (65 years vs 53 years, p<0.0001) and the diagnosis of IM was delayed (6 months vs 3 months, p=0.009). Weakness prevailing in the upper limbs (42.9% vs 15.3%), dysphagia (57.1% vs 25.5%), muscle atrophy (65.3% vs 34.7%), weight loss (61.2% vs 23.5%) and loss of the ability to walk (24.5% vs 5.1%) were hallmarks of DH/BS-IM (p≤0.0005), for which the patients more frequently received intravenous immunoglobulins (65.3% vs 34.7%, p=0.0004). Moreover, DH/BS-IM patients frequently featured signs and/or complications of systemic sclerosis (SSc), fulfilling the American College of Rheumatology/European Alliance of Associations for Rheumatology criteria for this disease in 40.8% of the cases (vs 5.1%, p<0.0001). Distribution of the myopathy, its severity and its association with SSc were independently associated with DH/BS (p<0.05). Mortality was higher in the DH/BS-IM patients and loss of walking ability was independently associated with survival (p<0.05). Conclusion: In IM patients, DH/BS is a marker of severity and is associated with SSc (scleromyositis).
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hal-04302994 , version 1 (23-11-2023)

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Luc Pijnenburg, Margherita Giannini, Maude Bouchard-Marmen, Laurent Arnaud, Simone Barsotti, et al.. In inflammatory myopathies, dropped head/bent spine syndrome is associated with scleromyositis: an international case–control study. RMD Open, 2023, 9 (3), pp.e003081. ⟨10.1136/rmdopen-2023-003081⟩. ⟨hal-04302994⟩
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