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

Luc Pijnenburg; Margherita Giannini; Maude Bouchard-Marmen; Laurent Arnaud; Simone Barsotti; Silvia Bellando-Randone; Livio Bernardi; Paola Bini; Jelena Blagojevic; Veronica Codullo; Marion Couderc; Claire de Moreuil; Emanuelle Dernis; Luca Diamanti; Jean Dubost; Fanny Duval; Giacomo Emmi; Jean-Marc Galempoix; Bernard Geny; Jacques-Eric Gottenberg; Monica Groza; Aurelien Guffroy; Isabelle Guichard; Philippe Guilpain; Baptiste Hervier; Marie Hudson; Luca Iaccarino; Florenzo Iannone; Delphine Lebrun; Enrico Marchioni; Kuberaka Mariampillai; Francois Maurier; Marta Mosca; Aleksandra Nadaj-Pakleza; Carlotta Nannini; Jean-Maxime Piot; Sergio Prieto-González; Nicolas Poursac; Eglantine Rouanet; Jérémie Sellam; Albert Selva-O'Callaghan; François Séverac; Jean Sibilia; Guilhem Sole; Antoine Soulages; Benjamin Terrier; Anne Tournadre; Yves Troyanov; Nathalie Vernier; Veronique Vesperini; Jean-François Viallard; Rahima Ziane; Lorenzo Cavagna; Alain Meyer

doi:10.1136/rmdopen-2023-003081

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

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

Luc Pijnenburg, Margherita Giannini, Maude Bouchard-Marmen, Laurent Arnaud, Simone Barsotti, Silvia Bellando-Randone, Livio Bernardi, Paola Bini, Jelena Blagojevic, Veronica Codullo, …

Rheumatic & musculoskeletal diseases open, Vol.9(3), p.e003081

04/09/2023

DOI: https://doi.org/10.1136/rmdopen-2023-003081

PMID: 37666644

Résumé

Aged

Cancer

Case-Control Studies

complications

diagnosis

Dropped Head Syndrome

Human health and pathology

Life Sciences

Myositis

Rheumatology

Scleroderma, Systemic

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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Titre: In inflammatory myopathies, dropped head/bent spine syndrome is associated with scleromyositis: an international case–control study
Créateurs - sans rôle: Luc Pijnenburg - Université de Strasbourg
Margherita Giannini - Université de Strasbourg
Maude Bouchard-Marmen - CHU de Québec–Université Laval
Laurent Arnaud - Université de Strasbourg
Simone Barsotti - University of Pisa [Italy] = Università di Pisa [Italia] = Université de Pise [Italie]
Silvia Bellando-Randone - Azienda Ospedaliero Universitaria Careggi [Firenze] = Careggi University Hospital [Florence, Italy]
Livio Bernardi - Università degli Studi di Padova = University of Padua
Paola Bini - Università degli Studi di Pavia [Italia] = University of Pavia [Italy] = Université de Pavie [Italie]
Jelena Blagojevic - Azienda Ospedaliero Universitaria Careggi [Firenze] = Careggi University Hospital [Florence, Italy]
Veronica Codullo - Fondazione IRCCS Policlinico San Matteo [Pavia]
Marion Couderc - Imagerie Moléculaire et Stratégies Théranostiques
Claire de Moreuil - Centre Hospitalier Régional Universitaire de Brest
Emanuelle Dernis - Centre Hospitalier Le Mans (CH Le Mans)
Luca Diamanti - Università degli Studi di Pavia [Italia] = University of Pavia [Italy] = Université de Pavie [Italie]
Jean Dubost - Service Médecine Interne - site Gabriel-Montpied [CHU Clermont-Ferrand]
Fanny Duval - Service de neurologie [Bordeaux]
Giacomo Emmi - Azienda Ospedaliero Universitaria Careggi [Firenze] = Careggi University Hospital [Florence, Italy]
Jean-Marc Galempoix - Centre Hospitalier de Charleville-Mezières
Bernard Geny - Hôpitaux Universitaires de Strasbourg
Jacques-Eric Gottenberg - Université de Strasbourg
Monica Groza - CH Colmar
Aurelien Guffroy - Hôpitaux Universitaires de Strasbourg
Isabelle Guichard - Centre Hospitalier Universitaire de Saint-Étienne
Philippe Guilpain - CHU Montpellier = Montpellier University Hospital
Baptiste Hervier - Assistance publique - Hôpitaux de Paris (AP-HP)
Marie Hudson - Jewish General Hospital
Luca Iaccarino - Università degli Studi di Padova = University of Padua
Florenzo Iannone - University of Bari Aldo Moro
Delphine Lebrun - Centre Hospitalier de Charleville-Mezières
Enrico Marchioni - Università degli Studi di Pavia [Italia] = University of Pavia [Italy] = Université de Pavie [Italie]
Kuberaka Mariampillai - Assistance publique - Hôpitaux de Paris (AP-HP)
Francois Maurier - Hôpitaux Privés de Metz
Marta Mosca - University of Pisa
Aleksandra Nadaj-Pakleza - Hôpitaux Universitaires de Strasbourg
Carlotta Nannini - Hospital of Prato
Jean-Maxime Piot - Centre Hospitalier Le Mans (CH Le Mans)
Sergio Prieto-González - Clinic Barcelona Hospital Universitari
Nicolas Poursac - Centre Hospitalier Universitaire de Bordeaux
Eglantine Rouanet - Centre Hospitalier Le Mans (CH Le Mans)
Jérémie Sellam - Assistance Publique – Hôpitaux de Paris
Albert Selva-O'Callaghan - Vall d'Hebron University Hospital [Barcelona]
François Séverac - Hôpitaux Universitaires de Strasbourg
Jean Sibilia - Hôpitaux Universitaires de Strasbourg
Guilhem Sole - Service de neurologie [Bordeaux]
Antoine Soulages - Service de neurologie [Bordeaux]
Benjamin Terrier - Hôpital Cochin [AP-HP]
Anne Tournadre - Service Médecine Interne - site Gabriel-Montpied [CHU Clermont-Ferrand]
Yves Troyanov - Hôpital du Sacré-Coeur de Montréal
Nathalie Vernier - Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand
Veronique Vesperini - Centre Hospitalier de Narbonne
Jean-François Viallard - Centre Hospitalier Universitaire de Bordeaux
Rahima Ziane - CHU Montpellier = Montpellier University Hospital
Lorenzo Cavagna - Fondazione IRCCS Policlinico San Matteo [Pavia]
Alain Meyer - Université de Strasbourg
Détails de publication: Rheumatic & musculoskeletal diseases open, Vol.9(3), p.e003081
Éditeur: BMJ Publishing Group
Identifiants: 99141187909311
Unité académique: Cell souches plasticité cellul régénérat tissulaire immuno des maladies inflamm - IRMB
Langue: English
Type de ressource: Journal article