In inflammatory myopathies, dropped head/bent spine syndrome is associated with scleromyositis: an international case-control study.
dermatomyositis
polymyositis
systemic sclerosis
Journal
RMD open
ISSN: 2056-5933
Titre abrégé: RMD Open
Pays: England
ID NLM: 101662038
Informations de publication
Date de publication:
09 2023
09 2023
Historique:
received:
18
02
2023
accepted:
22
06
2023
medline:
6
9
2023
pubmed:
5
9
2023
entrez:
4
9
2023
Statut:
ppublish
Résumé
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). To assess the significance of DH/BS in patients with IM. 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. 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). In IM patients, DH/BS is a marker of severity and is associated with SSc (scleromyositis).
Sections du 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).
Identifiants
pubmed: 37666644
pii: rmdopen-2023-003081
doi: 10.1136/rmdopen-2023-003081
pmc: PMC10481849
pii:
doi:
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Commentaires et corrections
Type : ErratumIn
Informations de copyright
© Author(s) (or their employer(s)) 2023. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.
Déclaration de conflit d'intérêts
Competing interests: None declared.
Références
Neurology. 2014 Jul 29;83(5):426-33
pubmed: 24975859
Neurology. 2018 Jun 5;90(23):e2068-e2076
pubmed: 29728522
RMD Open. 2019 Feb 26;4(Suppl 1):e000784
pubmed: 30886730
Muscle Nerve. 2015 Dec;52(6):1008-12
pubmed: 25900737
Acta Neuropathol. 2021 Aug;142(2):395-397
pubmed: 34115197
Joint Bone Spine. 2018 Jan;85(1):23-33
pubmed: 28343013
Clin Exp Rheumatol. 2016 Sep-Oct;34 Suppl 100(5):106-109
pubmed: 27749244
Age Ageing. 2010 Jul;39(4):412-23
pubmed: 20392703
Neuromuscul Disord. 2020 Feb;30(2):128-136
pubmed: 32005492
Neuromuscul Disord. 2020 Jan;30(1):70-92
pubmed: 31791867
JAMA Neurol. 2018 Dec 1;75(12):1528-1537
pubmed: 30208379
Ann Rheum Dis. 2013 Nov;72(11):1747-55
pubmed: 24092682
Neuromuscul Disord. 2018 Jan;28(1):87-99
pubmed: 29221629
Front Immunol. 2023 Jan 26;13:974078
pubmed: 36776390
Arthritis Rheum. 2004 Jul;50(7):2281-90
pubmed: 15248228
Neurol Clin Pract. 2018 Jun;8(3):240-248
pubmed: 30105164
Ann Rheum Dis. 2017 Dec;76(12):1955-1964
pubmed: 29079590
Arthritis Rheumatol. 2019 Jun;71(6):964-971
pubmed: 30614663
Neuropathol Appl Neurobiol. 2022 Dec;48(7):e12840
pubmed: 35894636
J Autoimmun. 2019 Jul;101:48-55
pubmed: 30992170
Chest. 2010 Dec;138(6):1464-74
pubmed: 21138882