Band 3, Heft 1

Purpose of review—To review autoantibodies associated with different subtypes of idiopathic inflammatory myopathy (IIM) and their clinical applications. IIM are a heterogenous group of autoimmune disorders characterized by muscle weakness, cutaneous features, and internal organ involvement. The diagnosis and classification, which is often challenging, is made using a combination of clinical features, muscle enzyme levels, imaging, and biopsy. The landmark discoveries of novel autoantibodies specific to IIM subtypes have been one of the greatest advancements in the field of myositis. The specificity of these autoantibodies has simplified the diagnostic algorithm of IIM with their heterogenous presentation and outdated the earlier diagnostic criteria. Myositis-specific antibodies (MSAs) have improved diagnostics, clinical phenotyping, and prognostic stratification of the subtypes of IIMs. Furthermore, the levels of certain MSAs correlate with disease activity and muscle enzyme levels such that titers may be able to be used to predict disease course and treatment response.

As a newly emerged infectious disease, the coronavirus disease 2019 (COVID-19) has caused millions of deaths, resulting in a global health challenge. Currently, several vaccines have been approved with significant benefits against disease transmission. However, effective therapies are still needed for the clinical management of infected COVID-19 patients. Available evidence has indicated elevated levels of proinflammatory cytokines, including interleukin-6 (IL-6), in COVID-19 patients, with cytokine storm involving excessive cytokine release being observed in some severe cases. Several clinical studies have shown the promising effects of IL-6-blocking strategy in treating severe COVID-19 patients, but some observational studies have reported that IL-6-blocking therapy has no effects in preventing disease progression or death among COVID-19 patients. Herein, we review recent findings on the immunopathogenesis of COVID-19, with specific emphasis on the proinflammatory function of IL-6 and discuss the therapeutic potential of IL-6-blocking therapy for the treatment of COVID-19 patients, especially those with rheumatic diseases.

Anti-DNA autoantibodies are pathogenic in systemic lupus erythematosus (SLE). Cell-free chromatin associated long DNA fragments are antigens for anti-DNA antibodies. In health state, released by cell death and actively secreted by live cells, these cell-free DNA are cleared by deoxyribonucleases (DNASES). In SLE, cell-free DNA are accumulated. The defective clearance of long fragments of cell-free DNA in SLE is largely attributed to impaired deoxyribonuclease 1 like 3 (DNASE1L3). DNASE1L3 null mutation results in monogenic SLE. The SLE risk single-nucleotide polymorphism (rs35677470) encodes R260C variant DNASE1L3, which is defective in secretion, leading to reduced levels of DNASE1L3. In addition, neutralizing autoantibodies to DNASE1L3 are produced in SLE to inhibit its enzymatic activity.

Objective To investigate the efficacy of conventional rehabilitation alone and conventional rehabilitation combined with aerobic training on muscle strength and function, health condition, and quality of life for patients with stable idiopathic inflammatory myopathy (IIM). Methods This is a historical retrospective cohort study, in which the medical records of patients with IIM, who received the combination of conventional rehabilitative therapy and aerobic training (combined training group [CTG]), from February 2015 to December 2017 were reviewed. Patients with IIM who received conventional therapy alone were matched based on their age, gender, and disease activity as the control group (CG). Scores obtained on manual muscle testing of eight designated muscles (MMT8) was the primary outcome measure, and scores on the myositis Functional Index-2 (FI-2), Health Assessment Questionnaire (HAQ), and 36-item Short Form Medical Outcomes Study Questionnaire (SF-36) at 12 weeks during training were the secondary outcomes. Results Fifty-six patients (28 in the CTG and 28 in the CG) were included in this analysis. Patients in both groups had improved MMT8, FI-2, HAQ, and SF-36 scores after 12 weeks of physical therapy. The CTG had a significantly higher score on the MMT8 and HAQ than the CG in the 12th week. The FI-2 scores were significantly higher in the CTG for the four items ( P < 0.05) of hip flexion, step test, heel lift, and toe lift. SF-36 scores of the CTG were also higher than those of the CG for the five items ( P < 0.05) of physical functioning, general health, vitality, social functioning, and mental health. Conclusions Physical exercise training including conventional rehabilitation and aerobic training improved muscle function, health condition, and quality of life. Conventional rehabilitative training combined with aerobic training achieved better improvement compared with conventional rehabilitation training alone.

Objectives The pan-European BENEFIT study of patients with stable rheumatoid arthritis (RA) or axial spondyloarthritis (axSpA) who transitioned from reference etanercept to SB4 found no clinically meaningful changes in disease control after transition. The analysis aims to illustrate the peculiarities of the Italian cohort of patients compared with the whole population to provide a more real-life approach to the data for the Italian rheumatologists, ruling out possible local confounding factors. Methods A prospective study for up to 6 months following transition was conducted. Outcome measures of interest include clinical characteristics at time of transition and disease activity scores (Disease Activity Score-28 [DAS28] for RA, Bath Ankylosing Spondylitis Disease Activity Index [BASDAI] for axSpA) over time and safety. Results One-hundred and eleven subjects (out of the 557 in total enrolled in the study) were derived from 8 Italian sites, including 79 with RA and 32 with axSpA. In both cohorts, the efficacy was maintained at 3 months and 6 months from the transition to the biosimilar with no significant change in mean DAS28 and BASDAI scores: at the end of the 6 months of observation the mean DAS28 and BASDAI was similar to baseline (confidence interval [CI] −0.22, 0.22), while the mean variation of the BASDAI was −0.14. Of note, 100.0% (95% CI 89.1, 100.0) in the axSpA and 90.8% (95% CI 81.5, 95.5) in the RA cohort of patients continued to receive SB4 at month 6 (binary variable with 95% Clopper-Pearson CI). Conclusions Italian patients with stable RA or axSpA who transitioned from originator Etanercept to SB4 maintained clinical response at 6 months post-transition. Both the cohorts are representative of typical patients with long-standing established diagnoses. Most of the patients transitioned to the same dose regimen of biosimilar as that received for the originator, and the regimen remained unchanged at 6 months, supporting the effectiveness of the transition.