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).

Anti-synthetase syndrome (ASS) is characterized by the presence of anti-aminoacyl-tRNA synthetase antibodies, most commonly anti-Jo-1, and the triad of myositis, arthritis, and interstitial lung di...

The prevalence of long-term antimalarial muscle and cardiac toxicity has been estimated at 6.5%.¹ Antimalarial-induced cardiomyopathy is reversible in 45% of cases after treatment discontinuation and is associated with a 20% mortality rate.²

Objective: Little is known as to whether the American College of Rheumatology/European Alliance of Associations for Rheumatology Total Improvement Score (TIS) reflects how patients with autoimmune inflammatory myopathies (AIM) feel and function. We assessed the associations between TIS and changes in the scores of a wide range of patient-reported outcomes (PROs). Methods: Adult patients with AIM and active disease at baseline who completed a 1-year follow-up visit were identified from a research cohort. PROs included a numerical rating scale for pain, the Functional Assessment of Chronic Illness Therapy – Fatigue (FACIT-Fatigue) scale, the Patient-Reported Outcomes Measurement Information System (PROMIS) Short Form v1.0 Fatigue 8a, the 9-item Patient Health Questionnaire (PHQ-9), and the 36-Item Short Form Health Survey (SF-36) physical and mental component summary scores (PCS and MCS, respectively). Multivariable linear regression models were generated to determine the absolute change in PRO scores for each 20-unit increase in absolute TIS. Results: We identified 65 patients with active disease for whom TIS could be calculated. At 1 year, 40% showed no improvement, 34% achieved minimal improvement, and 26% achieved moderate improvement. In adjusted models, increase in absolute TIS was associated with statistically significant improvements in pain, PROMIS-Fatigue, FACIT-Fatigue, and SF-36 PCS scores. For FACIT-Fatigue, a significant interaction was identified between age and TIS. In stratified analyses, only patients with shorter disease duration significantly improved in pain and FACIT-Fatigue scores. Conclusion: The findings of this cohort study support the use of the TIS in AIM research but also highlight potential limitations in its ability to capture outcomes across the broader AIM spectrum, particularly among older individuals and those with longer disease duration.