The aim of this study was to determine the clinical, serological, and genetic features of anti-Jo-1 positive antisynthetase patients followed by a Hungarian single centre to identify prognostic markers, which can predict disease phenotypes and disease progression. It was a retrospective study using clinical database of 49 anti-Jo-1 positive patients. 100% of patients exhibited myositis, 73% interstitial lung disease, 88% arthritis, 65% Raynaud's phenomenon, 43% fever, 33% mechanic's hand, and 12% dysphagia. We could detect significant correlation between anti-Jo-1 titer and the CK and CRP levels at disease onset and during disease course. HLA DRB1⁎03 positivity was present in 68.96% of patients, where the CK level at diagnosis was significantly lower compared to the HLA DRB1⁎03 negative patients. HLA DQA1⁎0501-DQB1⁎0201 haplotype was found in 58.62% of patients, but no significant correlation was found regarding any clinical or laboratory features. Higher CRP, ESR level, RF positivity, and the presence of fever or vasculitic skin lesions at the time of diagnosis indicated a higher steroid demand and the administration of higher number of immunosuppressants during the follow-up within anti-Jo-1 positive patients. The organ involvement of the disease was not different in HLA-DRB1⁎0301 positive or negative patients who were positive to the anti-Jo-1 antibody; however, initial CK level was lower in HLA-DRB1⁎0301 positive patients. Distinct laboratory and clinical parameters at diagnosis could be considered as prognostic markers.

Background: The prevalence of osteoporosis and risk of fractures is elevated in rheumatoid arthritis (RA), but we have limited information about the bone mineral density (BMD) and fracture risk in patients with inflammatory myopathies. We intended to ascertain and compare fracture risk, bone mineral density and the prevalence of vertebral fractures in patients with inflammatory myositis and rheumatoid arthritis and to assess the effect of prevalent fractures on the quality of life and functional capacity. Methods: Fifty-two patients with myositis and 43 patients with rheumatoid arthritis were included in the study. Fracture Risk was determined using FRAX® Calculation Tool developed by the University of Sheffield. Dual energy Xray absorptiometry and bidirectional thoracolumbar radiographs were performed to assess BMD and vertebral fractures. Quality of life was measured with Short Form-36 (SF-36) and physical function assessment was performed using Health Assessment Questionnaire (HAQ). Results: We found a significantly elevated fracture risk in RA as compared to myositis patients if the risk assessment was performed without the inclusion of the BMD results. If BMD results and glucocorticoid dose adjustment were taken into account, the differences in fracture risk were no longer significant. The prevalence of osteoporosis was found to be significantly higher in the myositis group (7% vs. 13.5%, p: 0.045), but the fracture prevalence was similar in the two groups (75% vs. 68%). The fracture rates were independently associated with age in the myositis group, and with lower BMD results in the RA patients. The number of prevalent fractures was significantly correlated to poorer physical function in both groups, and poorer health status in the myositis group, but not in the RA group.

BackgroundPruritus is a common symptom in systemic autoimmune diseases like dermatomyositis (DM). Recent researches have indicated that interleukin-31 (IL-31), IL-33, IL-6, or inflammatory cytokines, such as tumor necrosis factor (TNFα), peroxisome proliferator-activated receptor γ (PPARγ) and ion channels belonging to the transient receptor potential (TRP) family are involved in pruriception.ObjectivesWe examined targeted gene expression analysis of lesional versus non-lesional skin samples of patients affected with active DM. We looked for correlations between the examined pruriceptive signaling molecules, disease activity and itching sensation of DM patients.MethodsGene expression of TNFα, PPARγ, IL-33, IL-6 and TRPV channels in lesional DM skin was evaluated by RT-qPCR and was compared with non-lesional DM skin samples. Pruritus and disease activity of DM was evaluated by the 5-d itch scale and Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI), respectively. Statistical analysis was performed with IBM SPSS 20.0 software.ResultsSkin samples of 17 active DM patients were analyzed. We could show, that itching index in DM was positively correlated with CDASI score with a correlation coefficient of 0.82 (p<0.001). TNFα gene expression was significantly higher (34.87%) in lesional DM skin than non-lesional DM skin (p=0.03). The normalized TNFα mRNA expression was positively correlated with itch scale (R= 0.605, p=0.022) and its level was significantly higher in skin samples of patients with severe itch (itching score: 15-20) versus mild itch (itching score: 5-10) (2.02±0.38 vs. 1.12±0.20; p<0.01). The level of PPARγ was decreased in lesional DM skin, but this was statistically not significant. The mRNA expression of normalized PPARγ was negatively correlated with itch scale (R= -0.618, p=0.019), and its level was significantly lower in skin samples of patients with severe itch versus mild itch (0.28±0.36 vs. 1.53±0.98; p=0.038). Lesional IL-6 mRNA levels were associated with CDASI activity score (R=0.619, p=0.018). The mRNA levels of TRPV1-4 channels were not associated with 5-D itch score, but normalized TRPV1 and TRPV4 mRNA expressions were positively correlated with CDASI damage score (R=0.699, p=0.008; R=0.789, p=0.001). Interestingly, itching sensation of DM patients was not correlated with IL-33 mRNA levels measured in skin samples.ConclusionOur results argue for that higher cutaneous disease activity generate pruritus. TNFα and PPARγ might play a determining, but opposite role in DM-associated itch. Furthermore IL-6, TRPV1 and TRPV4 channels might participate in pathomechanism of cutaneous manifestation of the disease.Disclosure of Interests:

Purpose of Review This article provides an update on the most recent advances in epidemiology, pathogenesis, diagnostic procedures, and therapeutic approaches for myositis-associated bone diseases, such as osteoporosis and bone fractures. Recent Findings In the recent years, several studies showed that osteoporosis and consequent fractures are a common and frequently underestimated complication in patients with idiopathic inflammatory myopathies (IIM). In younger patients, asymptomatic fractures might present in the early phase of the disease which could increase the risk of development of further fractures. High-risk patients could be selected with early application of combined diagnostic procedures, such as fracture risk scores with steroid dose adjustments and imaging. Summary Recent advances might help clinicians from different fields of medicine in the early recognition and management of myositis-associated osteoporosis, which will potentially improve the quality of life of patients with IIM.

Introduction/Aims Vaccination against coronavirus disease 2019 (COVID‐19) is relatively safe in patients with idiopathic inflammatory myopathies (IIM); however, myositis flares following vaccination have been poorly studied. We aimed to evaluate the frequency, features, and outcomes of disease relapses in patients with IIM following COVID‐19 vaccination. Methods A cohort of 176 IIM patients were interviewed after the third wave of the COVID‐19 pandemic and followed prospectively. Relapses were determined using the disease state criteria and the outcome of the flares with myositis response criteria, calculating the total improvement score (TIS). Results A total of 146 (82.9%) patients received a vaccination, 17/146 (11.6%) patients had a relapse within 3 mo, and 13/146 (8.9%) patients within 1 mo. The relapse rate of unvaccinated patients was 3.3%. Three months after the post‐vaccination relapses, 70.6% of the patients (12/17) achieved an improvement of disease activity (average TIS score: 30 ± 15.81; seven minor, five moderate, and zero major improvements). Six months after flares improvement was detected in 15/17(88.2%) of relapsed patients (average TIS score: 43.1 ± 19.53; 3 minimal, 8 moderate, and 4 major). Forward stepwise logistic regression analysis revealed that the active state of myositis at the time of injection (p < .0001; odds ratio, 33; confidence interval, 9–120) was significantly associated with the occurrence of a relapse. Discussion A minority of the vaccinated IIM patients had a confirmed disease flare after COVID‐19 vaccination and the majority of the relapses improved after individualized treatment. An active disease state at the time of vaccination probably contributes to the increased risk of a post vaccination myositis flare.