In inflammatory myopathies, dropped head/bent spine syndrome is associated with scleromyositis: an international case–control study
Luc Pijnenburg
(1, 2)
,
Margherita Giannini
(1, 2)
,
Maude Bouchard-Marmen
(3)
,
Laurent Arnaud
(1, 2)
,
Simone Barsotti
(4)
,
Silvia Bellando-Randone
(5)
,
Livio Bernardi
(6)
,
Paola Bini
(7)
,
Jelena Blagojevic
(5)
,
Veronica Codullo
(8, 7)
,
Marion Couderc
(9, 10)
,
Claire de Moreuil
(11)
,
Emanuelle Dernis
(12)
,
Luca Diamanti
(7)
,
Jean Jacques Dubost
(13)
,
Fanny Duval
(14)
,
Giacomo Emmi
(5)
,
Jean-Marc Galempoix
(15)
,
Bernard Geny
(2, 1)
,
Jacques-Eric Gottenberg
(1, 2)
,
Monica Groza
(16)
,
Aurelien Guffroy
(2, 1)
,
Isabelle Guichard
(17)
,
Philippe Guilpain
(18)
,
Baptiste Hervier
(19)
,
Marie Hudson
(20, 21)
,
Luca Iaccarino
(6)
,
Florenzo Iannone
(22)
,
Delphine Lebrun
(15)
,
Enrico Marchioni
(7)
,
Kuberaka Mariampillai
(19)
,
Francois Maurier
(23)
,
Marta Mosca
(4)
,
Aleksandra Nadaj-Pakleza
(2, 1)
,
Carlotta Nannini
(24)
,
Jean-Maxime Piot
(12)
,
Sergio Prieto-González
(25)
,
Nicolas Poursac
(26)
,
Eglantine Rouanet
(12)
,
Jérémie Sellam
(19)
,
Albert Selva-O'Callaghan
(27)
,
François Séverac
(2, 1)
,
Jean Sibilia
(2, 1)
,
Guilhem Sole
(14)
,
Antoine Soulages
(14)
,
Benjamin Terrier
(28)
,
Anne Tournadre
(13)
,
Yves Troyanov
(29, 30)
,
Nathalie Vernier
(31)
,
Veronique Vesperini
(32)
,
Jean-François Viallard
(26)
,
Rahima Ziane
(18)
,
Lorenzo Cavagna
(8)
,
Alain Meyer
(1, 2)
1
UNISTRA -
Université de Strasbourg
2 HUS - Les Hôpitaux Universitaires de Strasbourg
3 CHU de Québec–Université Laval
4 UniPi - University of Pisa [Italy] = Università di Pisa [Italia] = Université de Pise [Italie]
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 [Italia] = University of Pavia [Italy] = Université de Pavie [Italie]
8 Fondazione IRCCS Policlinico San Matteo [Pavia]
9 IMoST - Imagerie Moléculaire et Stratégies Théranostiques
10 Service Rhumatologie [CHU Clermont-Ferrand]
11 CHRU Brest - Centre Hospitalier Régional Universitaire de Brest
12 Centre Hospitalier Le Mans (CH Le Mans)
13 Service Médecine Interne - site Gabriel-Montpied [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 = Montpellier University Hospital
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 - Centre Hospitalier Universitaire de 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
2 HUS - Les Hôpitaux Universitaires de Strasbourg
3 CHU de Québec–Université Laval
4 UniPi - University of Pisa [Italy] = Università di Pisa [Italia] = Université de Pise [Italie]
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 [Italia] = University of Pavia [Italy] = Université de Pavie [Italie]
8 Fondazione IRCCS Policlinico San Matteo [Pavia]
9 IMoST - Imagerie Moléculaire et Stratégies Théranostiques
10 Service Rhumatologie [CHU Clermont-Ferrand]
11 CHRU Brest - Centre Hospitalier Régional Universitaire de Brest
12 Centre Hospitalier Le Mans (CH Le Mans)
13 Service Médecine Interne - site Gabriel-Montpied [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 = Montpellier University Hospital
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 - Centre Hospitalier Universitaire de 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
Laurent Arnaud
- Fonction : Auteur
- PersonId : 1182354
- ORCID : 0000-0002-8077-8394
Veronica Codullo
- Fonction : Auteur
- PersonId : 810008
- ORCID : 0000-0003-2557-8514
Marion Couderc
- Fonction : Auteur
- PersonId : 1163509
- ORCID : 0000-0002-2001-1132
Jean Jacques Dubost
- Fonction : Auteur
- PersonId : 955175
- ORCID : 0000-0001-7415-6962
Giacomo Emmi
- Fonction : Auteur
- PersonId : 1312715
- ORCID : 0000-0001-9575-8321
Jacques-Eric Gottenberg
- Fonction : Auteur
- PersonId : 756827
- ORCID : 0000-0002-9469-946X
Baptiste Hervier
- Fonction : Auteur
- PersonId : 1312716
- ORCID : 0000-0002-1388-1121
Albert Selva-O'Callaghan
- Fonction : Auteur
- PersonId : 1312717
- ORCID : 0000-0003-2823-9761
Jean Sibilia
- Fonction : Auteur
- PersonId : 948651
Benjamin Terrier
- Fonction : Auteur
- PersonId : 762205
- ORCID : 0000-0001-6612-7336
- IdRef : 13141142X
Anne Tournadre
- Fonction : Auteur
- PersonId : 766543
- IdHAL : anne-tournadre
- ORCID : 0000-0002-5025-0214
Alain Meyer
- Fonction : Auteur
- PersonId : 1312718
- ORCID : 0000-0003-0893-6041
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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