Rare forms of inflammatory myopathies - part I, generalized forms.

INTRODUCTION: the idiopathic inflammatory myopathies traditionally comprise dermatomyositis, polymyositis, immune-mediated necrotizing myopathy, anti-synthetase syndrome and inclusion body myositis. However, there are uncommon forms that are less well characterized. In this review, we aimed to cover the uncommon forms of generalized myositis. AREAS COVERED: we identified rare forms of widespread myositis on the basis of list provided by the homepage of the Neuromuscular disease center of Washington University, USA and on the basis of the authors' knowledge. We searched PubMed® and EMBASE® for relevant articles on these forms with the aim of providing as much as possible information on their clinical manifestations as well as guidance on their work-up and treatment. EXPERT OPINION: herein, we provide un updated description of various rare forms of generalized myositis. There is substantial heterogeneity among these forms in terms of their frequency and characterization. Some forms are reasonably well defined, while others may not represent truly well-defined diseases, but rather variants of other myopathies. The landscape of rare forms appears to have evolved over time, with some forms now being better characterized, while others, such as SARS-Cov-2- and immune checkpoint inhibitor-related myositis have come to the fore only in recent years. Knowledge about rare forms of myositis can aid in their recognition and treatment.

Acute-phase reactants during tocilizumab therapy for severe COVID-19 pneumonia.

OBJECTIVES: To identify predictors of clinical improvement and intubation/death in tocilizumab-treated severe COVID19, focusing on IL6 and CRP longitudinal monitoring. METHODS: 173 consecutive patients with severe COVID-19 pneumonia receiving tocilizumab in Reggio Emilia province Hospitals between 11 March and 3 June 2020 were enrolled in a prospective cohort study. Clinical improvement was defined as status improvement on a six-category ordinal scale or discharge from the hospital, whichever came first. A composite outcome of intubation/death was also evaluated. CRP and IL-6 levels were determined before TCZ administration (T0) and after 3 (T3), and 7 (T7) days. RESULTS: At multivariate analysis T0 and T3 CRP levels were negatively associated with clinical improvement (OR 0.13, CI 0.03-0.55 and OR 0.11, CI 0.0-0.46) (p=0.006 and p=0.003) and positively associated with intubation/death (OR 17.66, CI 2.47-126.14 and OR 5.34, CI: 1.49-19.12) (p=0.01 and p=0.004). No significant associations with IL-6 values were observed. General linear model analyses for repeated measures showed significantly different trends for CRP from day 3 to day 7 between patients who improved and those who did not, and between patients who were intubated or died and those who were not (p<0.0001 for both). ROC analysis identified a baseline CRP level of 15.8 mg/dl as the best cut-off to predict intubation/death (AUC = 0.711, sensitivity = 0.67, specificity = 0.71). CONCLUSIONS: CRP serial measurements in the first week of TCZ therapy are useful in identifying patients developing poor outcomes.

Susceptibility to COVID-19 in Patients Treated With Antimalarials: A Population-Based Study in Emilia-Romagna, Northern Italy.

OBJECTIVE: To evaluate the susceptibility to coronavirus disease 2019 (COVID-19) in patients with autoimmune conditions treated with antimalarials in a population-based study. METHODS: All residents treated with chloroquine (CQ)/hydroxychloroquine (HCQ) from July through December 2019 and living in 3 provinces of Regione Emilia-Romagna were identified by drug prescription registries and matched with the registry containing all residents living in the same areas who have had swabs and tested positive for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Odds ratios (ORs) and 95% confidence intervals (95% CIs) were calculated. RESULTS: A total of 4,408 patients were identified. The prevalence of patients receiving antimalarials was 0.85 per 1,000 men and 3.3 per 1,000 women. The cumulative incidence of testing during the study period was 2.7% in the general population and 3.8% among those receiving CQ or HCQ, while the cumulative incidence of testing positive was 0.55% in the general population and 0.70% among those receiving CQ/HCQ. Multivariate models showed that those receiving CQ/HCQ had a slightly higher probability of being tested compared to the general population (OR 1.09 [95% CI 0.94-1.28]), the same probability of being diagnosed as having COVID-19 (OR 0.94 [95% CI 0.66-1.34]), and a slightly lower probability of being positive once tested (OR 0.83 [95% CI 0.56-1.23]). None of the differences were significant. CONCLUSION: Our findings do not support the use of antimalarials as a prophylactic treatment of COVID-19.