In inflammatory myopathies, dropped head/bent spine syndrome is associated with scleromyositis: an international case-control study.

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).

Association of tobacco exposure and reduction of radiographic progression in early rheumatoid arthritis: results from a French multicenter cohort.

OBJECTIVE: To investigate the initial response to treatment and risk of radiographic disease progression in current smokers (S), ex-smokers (EX), and nonsmokers (NS) in a prospective early arthritis cohort and to analyze the influence of smoking cessation on arthritis outcome. METHODS: The ESPOIR cohort is a prospective cohort study monitoring clinical, biologic, and radiographic data for patients with inflammatory arthritis lasting 6 weeks to 6 months. We examined the influence of smoking status on disease presentation (baseline characteristics) and therapeutic response at 1 year. Risk of structural progression at 12 months, defined as change in the modified Sharp/van der Heijde score ≥1, was analyzed by multivariate regression adjusted for potential confounders (age, sex, joint erosion at inclusion, educational level, positivity for rheumatoid factor or anti-cyclic citrullinated peptide 2 antibodies, and shared HLA-DRB1 epitope). RESULTS: A total of 813 patients were included; 641 (79%) fulfilled the 2010 American College of Rheumatology/European League Against Rheumatism (EULAR) criteria for rheumatoid arthritis (RA). At inclusion, 138 (21.5%) were S patients, 168 (26.2%) were EX patients, and 335 (52.3%) were NS patients. Baseline acute-phase indicator values were significantly lower for S patients than EX and NS patients (mean ± SD erythrocyte sedimentation rate was 24.2 ± 18.2 mm/hour versus 33.4 ± 28.0 and 31.4 ± 25.0 [P = 0.02], respectively, and mean ± SD C-reactive protein level was 17.7 ± 28.0 mg/dl versus 28.5 ± 42.5 and 21.4 ± 29.0 [P = 0.01], respectively). Smoking status had no influence on Disease Activity Score in 28 joints, Health Assessment Questionnaire score, EULAR response, or use of disease-modifying antirheumatic drugs and biologic therapy in the first 12 months of followup (P > 0.05). The adjusted risk for structural disease progression was associated with active smokers (odds ratio 0.50 [95% confidence interval 0.27-0.93], P = 0.028). Sixteen patients had stopped smoking at 12 months, with no significant difference in observed outcomes from other patients. CONCLUSION: In this large prospective cohort of patients with early arthritis, smoking status had no significant effect on disease activity and disability but did reduce 1-year radiographic disease progression. The antiinflammatory role of nicotine may explain the lower systemic inflammation and structural disease progression in current smokers with early RA.