In inflammatory myopathies, dropped head/bent spine syndrome is associated with scleromyositis: an international case-control study.
RMD Open
; 9(3)2023 09.
Article
en En
| MEDLINE
| ID: mdl-37666644
ABSTRACT
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).Palabras clave
Texto completo:
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Base de datos:
MEDLINE
Asunto principal:
Reumatología
/
Esclerodermia Sistémica
/
Miositis
Tipo de estudio:
Diagnostic_studies
/
Observational_studies
/
Risk_factors_studies
Idioma:
En
Revista:
RMD Open
Año:
2023
Tipo del documento:
Article