Patient Perspectives of the Manifestations and Treatment of Anti-MDA5 Antibody-Positive Dermatomyositis: An Observational Survey.

Idiopathic inflammatory myopathies (IIM) are a heterogeneous group of autoimmune disorders classically characterized by proximal skeletal muscle inflammation leading to weakness, but they often possess additional systemic manifestations such as cutaneous, pulmonary, and articular disease.1 Although originally dichotomized as either dermatomyositis (DM) or polymyositis, the discovery of new myositis-specific antibodies (MSA) and myositis-associated antibodies has led to the delineation of more refined IIM patient subgroups.

Pain profile and opioid medication use in patients with idiopathic inflammatory myopathies.

OBJECTIVES: Pain is commonly reported in people living with myositis. This study assesses the presence of pain in the subtypes of myositis as well as the frequency of opioid and non-opioid pain medication use. METHODS: A survey was developed and distributed by Myositis Support and Understanding, a patient-led advocacy organization, to members of its group. Multivariate logistic regression analysis and chi-squared tests were performed. RESULTS: A total of 468 participants completed the survey. A total of 423 participants (DM n = 183, PM n = 109 and IBM n = 131) were included, based on reported diagnosis, for final analysis. Some 91.5% of myositis participants reported current or past pain, with 99% attributing their pain to myositis. There was a lower likelihood of pain in participants aged >60 years [odds ratio (OR) 0.2, 95% confidence interval (CI) 0.1, 0.6, P = 0.003]. The percentage of participants reporting pain was statistically different based on myositis type (DM 97.2%, IBM 80.9% and PM 94.5%, P < 0.001), with a higher likelihood of pain in DM compared with IBM (OR 3.7, 95% CI 1.3, 10.2, P = 0.011). There was a lower likelihood of pain in participants aged >60 years (OR 0.2, 95% CI 0.1, 0.6, P = 0.003). Of the 387 participants reporting pain, 335 reported using pain medications (69% prescribed opioids). Male sex, age >60 years and myositis subtype were not associated with likelihood of non-opioid use. CONCLUSION: Pain is a commonly reported symptom in myositis with variable treatment strategies, including opioid medications. This study highlights the importance of addressing pain as part of myositis treatment as well as the need for future studies understanding treatment effectiveness.

The personal financial burden associated with idiopathic inflammatory myopathies.

The economic burden of idiopathic inflammatory myopathies (IIMs) within the US is underexplored. We hypothesized that IIMs patients experience considerable personal financial burden due to risks of multi-specialist visits, chronic long-term care, costs associated with disability, medical treatment, and overall high spending costs within the US healthcare system. We surveyed members of Myositis Support and Understanding (MSU) (response rate 4.7 %), and of the 470 survey participants that self-reported with diagnoses of IIMs, we assessed financial burden using two validated measures: (1) Financial Worry Score, and (2) Financial Burden Composite Score (FBCS). We determined factors associated with increased FBCS using logistic and Poisson regression respectively. High financial worry was endorsed by 202 participants (43 %) and the average FBCS ± SD was 1.8 ± 1.9. The odds of financial worry among participants with Medicaid is 3.016 times the odds of financial worry among participants without Medicaid (p = 0.011), and the odds of financial worry among participants with private high-deductible insurance is 3.216 times the odds of financial worry among participants who do not have private high-deductible insurance (p =< 0.001). Given the link between personal financial burden and potential effects on patient outcomes, it is essential for physicians to consider patient financial health when determining management or treatment courses. Identifying specific risk factors that can further exacerbate personal financial burden can help physicians identify vulnerable patients to reduce financial hardship.

Tendon Transfers to Improve Grip and Pinch in Patients with Sporadic Inclusion Body Myositis.

Background: Sporadic inclusion body myositis (sIBM) is a rare and slowly progressive skeletal muscle disease that can cause hand dysfunction, which is a major source of disability. Tendon transfers have been reliably used to improve function in other neuromuscular settings. Given that sIBM patients often present with flexion impairments and mostly functioning extensors, we investigated the potential opportunity for tendon transfer surgery to improve hand dysfunction in sIBM patients. Methods: We conducted a scoping review for studies of sIBM and tendon transfers, extracted descriptions of hand function and surgical technique, and recorded results in terms of hand function. We also conducted an institutional review board-approved survey with 470 participants to determine baseline patient-reported function and to determine participant perceptions and expectations for tendon transfer surgery to improve hand function in sIBM. Results: We identified three published case reports on tendon transfers in sIBM patients with subjectively improved grip and pinch strength, but standardized measures of hand function or quality-of-life were not reported. Within the surveyed cohort, half of participants reported that they would consider surgery, yet only 8% had been referred to a hand surgeon. Fifty four percent of participants reported that they would consider surgery if there would be 1-2 years of benefit after surgery. All participants who would consider surgery also had significant upper extremity disability. Discussion: Tendon transfer surgery has the potential to improve quality-of-life for sIBM patients, and there is significant patient interest in this approach. To objectively assess its efficacy, we propose conducting a surgical trial.