ArticleRMD open2023
In inflammatory myopathies, dropped head/bent spine syndrome is associated with scleromyositis: an international case-control study.
Article in RMD open, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. An erratum has been issued. Cited by 6 papers.
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Who cites it
6 citing papers in PubMed, 9 citations in OpenAlex.
- Review
- Dropped head syndrome leading to the diagnosis of antisynthetase syndrome: a motor neuron mimicker.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2026Article
- Revisiting the link between oropharyngeal dysphagia and cancer in autoimmune myositis: a descriptive study.Arthritis research & therapy · 2025Article
- Nailfold capillary abnormalities as useful clues in an atypical case suggestive of cancer-associated dermatomyositis sine dermatitis.Rheumatology advances in practice · 2025Article
- Case report: A patient with brachio-cervical inflammatory myopathy was misdiagnosed as flail arm syndrome.Frontiers in immunology · 2024Article
- Systemic sclerosis associated myopathy: how to treat.Current treatment options in rheumatology · 2023Article
Corrections and comments
- Erratum issued
Authors and funding
54 authors at 20 institutions in 4 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundSome 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).
objectivesTo assess the significance of DH/BS in patients with IM.
methodsPractitioners 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.
results49 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).
conclusionIn IM patients, DH/BS is a marker of severity and is associated with SSc (scleromyositis).
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