BackgroundSeveral systemic autoimmune rheumatic diseases may affect both fetal and maternal outcomes during pregnancy. However, little information is available regarding pregnancy outcomes in women with idiopathic inflammatory myopathy. Previously published articles stated that the activity of maternal disease may worsen pregnancy outcomes. A former multicenter study suggested that anti-Jo1 antibody positivity and joint involvement could distinguish a more vulnerable group regarding pregnancy complications. Our aim was to identify prognostic factors among clinical symptoms at disease onset and auto-antibody profiles for identifying a high-risk group for poor pregnancy outcome.MethodsThe clinical data of the myositis cohort of the Division of Clinical Immunology, University of Debrecen, Hungary, were reviewed retrospectively. IIM diagnoses were made by Bohan and Peter's criteria or European Alliance of Associations for Rheumatology/American College of Rheumatology (EULAR/ACR) criteria. Disease activity was evaluated based on physician opinion. Gynecological definitions were used to evaluate fetal outcomes.ResultsReviewing clinical data of overall 763 patients (542 women and 221 men) revealed that 5.2% of female patients had pregnancies in the same time or after myositis onset. Among these, 71.4% of the mothers suffered from polymyositis (PM) and 28.6% suffered from dermatomyositis (DM). Their mean age at the time of myositis diagnosis was 25.28 years, and the average interval between myositis diagnosis and first pregnancy was 55.4 months. Maternal complications included preeclampsia in one case and pregnancy-induced myositis in 25% of cases. All cases of pregnancy-induced myositis improved after immunosuppressive treatment. Twenty-eight patients reported 60 pregnancies overall, with multiple pregnancies occurring in 57% of cases. Early or late fetal loss was detected in 41.7% of the pregnancies, and stillbirth occurred in 18.3% of deliveries. Although late fetal loss was observed mainly due to placental insufficiency in patients with anti-Jo1 positivity and complications seemed more frequent in PM cases, logistic regression analysis only confirmed that multiple pregnancies could be an independent risk factor for fetal (p = 0.0112) and interstitial lung disease of maternal complications (p = 0.02).ConclusionInternal organ involvement and the number of pregnancies could influence pregnancy outcomes in myositis patients. Patients’ family planning should be well organized and counseled by myositis experts. Prospective, multicenter collaborations are needed to precisely identify high-risk groups and state managing guidelines.

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.

BackgroundThe approved COVID-19 vaccines showed clear safety and efficacy in reduction of severe SARS-CoV-2 disease. Patients with idiopathic inflammatory myopathies (IIM) were not well represented in these vaccine trials and there are limited data in the literature about development of confirmed disease flare after COVID-19 vaccination.ObjectivesTo evaluate frequency, features and outcome of disease relapses in patients with IIM following SARS–CoV-2 vaccination.MethodsA cohort of 176 IIM patients (mean age:57.6 years, 117 females, 59 males, 106 PM, 70 DM) were interviewed after the 3rd wave of COVID-19 pandemic and prospectively followed. Relapses were determined using the IMACS disease state criteria, outcome of the flares with myositis response criteria, calculating the total improvement score (TIS).ResultsA total of 146 (82.9%) patients received vaccination and 17/146 (11.6%) patients had relapse within 3 months and 13/146 (8.9%) patients within one month. The relapse rate of unvaccinated patients (1/30; 3.3%) was not significantly different (p>0.1). No fatal flare has been observed. Three months after the post-vaccination relapses, 70.6% of the patients (12/17) achieved improvement of disease activity (average TIS score:30±15.81; 7 minor, 5 moderate and 0 major improvement). Six months after flares improvement was detected in 14/16 (87.5%) of relapsed patients (average TIS score: 43±20.17; 3 minimal, 7 moderate and 4 major). Forward stepwise logistic regression analysis revealed that the active state of myositis at time of injection (p<0.0001; OR: 31.2 CI: 9.0 – 108) and the application of BNT162b2 vaccination (p=0.026; OR: 4.06 CI: 1.1 – 14.7) were significantly associated with the occurrence of relapse.ConclusionMinority of the vaccinated IIM patients had confirmed disease flare after COVID-19 vaccination and majority of the relapses improved after individualized treatment. Active disease state at the time of vaccination probably contributes to the increased risk of post vaccination myositis flare.REFERENCES:NIL.Acknowledgements:NIL.Disclosure of InterestsNone Declared.

IntroductionPruritus is a common excruciating symptom in systemic autoimmune diseases such as dermatomyositis (DM) but the pathogenesis is not fully understood. We intended to investigate the targeted expression analysis of candidate molecules involved in the development of pruritus in lesional vs. non-lesional skin samples of patients affected with active DM. We looked for correlations between the investigated pruriceptive signaling molecules, disease activity, and itching sensation of DM patients.MethodsInterleukins (IL-33 and IL-6), tumor necrosis factor α (TNF-α), peroxisome proliferator-activated receptor γ (PPAR-γ), and ion channels belonging to the transient receptor potential (TRP) family were analyzed. The expression of TNF-α, PPAR-γ, IL-33, IL-6, and TRP channels in lesional DM skin was evaluated by RT-qPCR and immunohistochemistry and was compared with non-lesional DM skin samples. Pruritus, disease activity, and damage of DM were evaluated by the 5-D itch scale and Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI), respectively. Statistical analysis was performed with IBM SPSS 28 software.ResultsA total of 17 active DM patients participated in the study. We could show that the itching score was positively correlated with the CDASI activity score (Kendall's tau-b = 0.571; p = 0.003). TNF-α gene expression was significantly higher in lesional DM skin than in non-lesional DM skin (p = 0.009) and differed in the subgroups of patients with different itch intensities (p = 0.038). The mRNA expression of lesional IL-6 correlated positively with 5-D itch and CDASI activity score (Kendall's tau-b = 0.585; p = 0.008 and 0.45; p = 0.013, respectively). TRPV4 expressions were positively correlated with CDASI damage score (Kendall's tau-b = 0.626; p < 0.001), but the mRNA expressions of the TRP family, PPAR-γ, IL-6, and IL-33 were not different in lesional and non-lesional samples. Immunohistochemistry analysis did not find significant alterations in the expressions of TNF-α, PPAR-γ, IL-6, and IL-33 in lesional and non-lesional regions.DiscussionOur results argue that cutaneous disease activity, TNF-α, and IL-6 might play a central role in DM-associated itch, while TRPV4 plays a central role in tissue regeneration.

The association of neuromyelitis optica concurrently with two other autoimmune diseases is rare. Neuromyelitis optica should be taken into consideration when evaluating the symptoms of the patient as a differential diagnostic aspect.

OBJECTIVES:Pandemic caused by coronavirus disease (COVID-19) determines the life of clinicians and patients since 2 years. We have a lot of information about disease course, treatment and protection against virus, but less on the prognosis of infection in patients with idiopathic inflammatory myopathies (IIM). Also few data are available on triggered humoral response and side effects after vaccination.METHODS:Our goal was to assess by a retrospective cross-sectional study the above data in our cohort (176 IIM patients) by identifying COVID-19 positive patients and follow disease course. Incidence and complications of vaccination were determined by questionnaires. 101 patients volunteered for complex blood test.RESULTS:By June 1st, 2021 significantly higher incidence of COVID 19 infections (34.7%) were identified comparing to the national prevalence (8.2%). A third of these infections occurred asymptomatically or mild. Patients requiring hospitalisation had a significantly longer disease duration and a higher incidence of anti-Jo-1 antibody. All patients infected by COVID-19 became seropositive regardless the immunosuppressive therapy or symptoms severity. 54.3% of the patients received anti-COVID-19 vaccine. 72.3% of patients became seropositive after vaccination. Higher antibody titer against spike protein was detected after Pfizer-BioNTech vaccination compared to others. Patients receiving steroid therapy had decreased post-vaccination antibody response compared to those without steroid treatment. No major post-vaccination infection was observed.CONCLUSIONS:Based on our results, myositis may be associated with an increased risk of COVID-19 infection. Independent risk factor for hospitalisation are longer disease duration and anti-Jo1 positivity. Anti-SARS-CoV2 vaccines seem safe and tolerable and strongly recommended for that population.

BackgroundPandemic caused by severe acute respiratory syndrome coronavirus 2 (SARS-Cov2/COVID19) determines the life of clinicians and patients since 2 years. Limited information is available on the nature, prognosis, and complications of SARS-Cov2 virus infection in patients with idiopathic inflammatory myopathies (IIM). There are also few data on triggered humoral response, side effects and disease course after COVID19 infection or vaccination in IIM.ObjectivesThe primary goals of the current research were to assess frequency and outcome of COVID-19 disease and to determine the vaccination rate and effect in our IIM cohort. Secondary objectives were to search for risk factors of infection, predictive factors of hospitalization and to assess incidence of vaccination adverse events, complications and post vaccination breakthrough infections.MethodsWe retrospectively identified the confirmed COVID19 positive patients and assessed the symptoms, disease course and outcome on 01/06/2021 then patients were prospectively followed. Incidence and complications of vaccination were determined by questionnaires. Anti-SARS-CoV-2 S enzyme electrochemiluminescent immunoassay has been used to assess seroconversion, which measures total antibody (IgM and IgG) to the SARS-CoV2 S protein and SARS-CoV2 N protein. Disease activity was determined by physician global activity.ResultsA total of 176 patients were screened and 101 participated in the study. By 01/06/2021, the COVID infection rate was 34,7% (mean age: 49.54 years, 72.72% women), which was significantly higher than the national prevalence at that time (8.2%). A third of these infections occurred asymptomatically or mild but 20% of the infected patients were hospitalized, one patient died. Longer disease duration (8.67 vs. 17.87 years; p=0.003) and higher incidence of anti-Jo-1 antibody (57% vs. 10% p=0.018) were significantly associated with hospitalization. All of COVID infected patients became seropositive regardless of immunosuppressive therapy or symptoms severity. 53,4 % of the patients received anti-COVID19 vaccine, 75,9 % choose the mRNA type. The titer of antibodies against the spike protein induced by vaccines showed high variance, but 72,3% of patients became seropositive after vaccination. Higher antibody titer against spike protein was detected after Pfizer-BioNTech vaccination compared to others (177,1 U / ml vs. 81.1 U / ml; p <001). Patients receiving steroid therapy had decreased post-vaccination antibody response compared to those without steroid treatment (94,03 U/ml vs. 165.6 U/ml; p = 0.008). With the follow-up of vaccinated patients, we did not found short term vaccine related major adverse events, but long term data revealed 7,4 % post vaccination disease relapse. Breakthrough infection was detected in 9.25 % of the vaccinated patients, one cancer associated patient without post vaccination seroconversion died due to COVID pneumonia. All the fatal COVID infections occurred in patients with seronegativity to anti- SARS-CoV2 S protein.ConclusionBased on our results, myositis may be associated with an increased risk of infection with SARS-CoV-2. Independent risk factor for hospitalization is anti-Jo1 positivity and longer disease duration. Anti-SARS-CoV2 vaccines are safe, tolerable and strongly recommended for IIM population, but further investigation is required to assess clinical significance of post-vaccination disease flare.Disclosure of InterestsNone declared

Overlap myositis is a distinct subgroup of idiopathic inflammatory myositis (IIM) with various clinical phenotypes. The aim of this study was to determine the clinical, serological, and genetic features of systemic sclerosis (SSc)-IIM overlap patients. It was a retrospective study using clinical database of 39 patients, fulfilling both the criteria of SSc and IIM. 56.4% of the patients had limited cutaneous, 43.6% had diffuse cutaneous SSc, whereas 7.7% of the patients had dermatomyositis and 92.3% polymyositis. The two diseases occurred simultaneously in 58.97%, while 10.26% in myositis and 30.77% in scleroderma were initially diagnosed. The frequencies of organ involvement were interstitial lung disease 71.8%, dysphagia 66.7%, cardiac involvement 41%, pulmonary arterial hypertension (PAH) 30.8%, and renal involvement 12.8%, respectively. The presence of human leukocyte antigen (HLA) − DRB1∗03 and DQA1∗051∗01 alleles were significantly higher in the overlap patients than in healthy controls (82.35% vs. 27.54%; p < 0.0001 and 88.24% vs. 30.16; p < 0.0001). Certain clinical parameters, such as fever at diagnosis (41.67% vs. 7.41%, p = 0.0046), cardiac involvement (83.33% vs. 22.22%, p = 0.0008), subcutaneous calcinosis (41.66 vs. 11.11, p = 0.01146), and claw hand deformity (25% vs. 11.11%, p = 0.00016) were significantly associated with the presence of PAH. Upon comparison, the overlap patients and anti-Jo-1 positive antisynthetase patients showed similarities in terms of genetic results and major clinical features; however, SSc-IIM overlap patients could be distinguished with higher erythrocyte sedimentation rate (ESR) level, more frequent presence of Raynaud's phenomenon (p < 0.0001; OR: 20.00), dysphagia (p < 0.0001; OR: 15.63), and infrequent livedo reticularis (p < 0.01; OR: 0.11). SSc-IIM overlap myositis is a unique group within IIM-s possessing characteristic clinical features.