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COVID-19; Registries; Vaccination COVID-19; Registros; Vacunación COVID-19; Registres; Vacunació Vaccine hesitancy is considered a major barrier to achieving herd immunity against COVID-19. While multiple alternative and synergistic approaches including heterologous vaccination, booster doses, and antiviral drugs have been developed, equitable vaccine uptake remains the foremost strategy to manage pandemic. Although none of the currently approved vaccines are live-attenuated, several reports of disease flares, waning protection, and acute-onset syndromes have emerged as short-term adverse events after vaccination. Hence, scientific literature falls short when discussing potential long-term effects in vulnerable cohorts. The COVAD-2 survey follows on from the baseline COVAD-1 survey with the aim to collect patient-reported data on the long-term safety and tolerability of COVID-19 vaccines in immune modulation. The e-survey has been extensively pilot-tested and validated with translations into multiple languages. Anticipated results will help improve vaccination efforts and reduce the imminent risks of COVID-19 infection, especially in understudied vulnerable groups. HC is supported by the National Institution for Health Research Manchester Biomedical Research Centre Funding Scheme. The views expressed in this publication are those of the authors and not necessarily those of the NHS, the National Institute for Health Research or the Department of Health.

Objectives The COVID-19 vaccination in autoimmune diseases (COVAD) study aimed to assess short-term COVID-19 vaccination-related adverse events (AEs) in RA patients. Methods An online self-reported questionnaire (March–December 2021) was used to capture data related to COVID-19 vaccination-related AEs in RA, other autoimmune rheumatic diseases (AIRDs) (excluding RA and inflammatory myositis), non-rheumatic autoimmune diseases (nrAIDs) and healthy controls (HCs). Descriptive and multivariable regression analyses were performed. Results Of the 9462 complete respondents, 14.2% (n = 1347) had been diagnosed with RA; they had a mean (s.d.) age of 50.7 (13.7) years, 74.2% were women and 49.3% were Caucasian. In total, 76.9% and 4.2% of patients with RA reported minor and major AEs, respectively. Patients with active and inactive RA had similar AE and hospitalization frequencies. Overall, AEs were reported more frequently by BNT162b2 and mRNA-1273 recipients and less frequently by BBV152 recipients compared with the rest. Major AE and hospitalization frequencies were similar across recipients of different vaccines. Patients receiving methotrexate and hydroxychloroquine reported fewer minor AEs than those patients not on them. Compared with HCs and patients with other AIRDs, patients with RA reported similar total AEs, overall minor AEs, and hospitalizations. Compared with nrAIDs, patients with RA reported lower frequencies of overall AEs, minor AEs (both odds ratio [OR] = 0.7; 95% CI: 0.5, 0.9), and injection site pain (OR = 0.6; 95% CI: 0.5, 0.8) with similar major AE and hospitalization frequencies. Conclusion Despite the differences in AE frequency across different COVID-19 vaccines, all were well tolerated in patients with RA and were comparable to HCs, providing reassurance as to the safety of COVID-19 vaccination.

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%

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.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.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.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.

Background: The prevalence of osteoporosis and risk of fractures is elevated in rheumatoid arthritis, but we have little information about the bone mineral density and fracture risk in patients with inflammatory myopathies. We intended to ascertain and compare fracture risk, bone mineral density (BMD) and the prevalence of vertebral fractures in patients with inflammatory myositis and rheumatoid arthritis (RA) 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 X-ray 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 compared to myositis patients if the risk assessment was performed without the application 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 fractures rates were associated with age in both groups, but not with cumulative dose of steroid and BMD results correlated with fracture prevalence only 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. Conclusions: Our findings suggest that inflammatory myopathies carry significantly elevated risk for osteoporosis and fractures. This higher risk is comparable to one detected with RA in studies and strongly affects the physical function and quality of life of patients. Therefore further efforts are required to make the fracture risk assessment reliable and to facilitate the use early preventive treatments.

Several systemic autoimmune rheumatic diseases (SARDs) may affect both fetal and maternal outcomes in pregnancy. Little information is available regarding pregnancy outcomes in women with idiopathic inflammatory myopathy (IIM; or myositis) where both adaptive and innate immune mechanisms are likely to be involved in the pathogenesis. The immunological pathways leading to complications are unexplored.

Dermatomyositis is a rare heterogeneous systemic autoimmune disease with multiple organ involvement which can result in significant disability and mortality. Despite the lack of placebo-controlled trials, glucocorticoids are considered to be the mainstay of initial management. Treatment strategies are mainly based on uncontrolled studies, evidence based guidelines for treatments do not exist. Areas covered: This review provides an overview of the currently available pharmacological treatments in the field of dermatomyositis including conventional immunosuppressants, biologics and topical agents. The role of antibodies in different treatment responses of dermatomyositis related clinicoserological syndromes is also discussed. A PubMed search was performed in order to find relevant literature for this review. Expert commentary: Early recognition and intervention is essential to ameliorate disease outcome. Determination of antibodies provide a useful key in diagnosis, clinical manifestations, malignancy, prognosis, and treatment response and may lead to wider acceptance of personalized medicine. Corticosteroids with adjunctive steroid-sparing immunosuppressive therapies are recommended to treat disease activity, prevent mortality, and reduce long-term disability. Combinations of second-line therapies or newer third-line therapies are used in severe, refractory, or corticosteroid-dependent diseases. Further research is required to assess the role of new therapies.

MicroRNA (miRNA) research has intensively developed over the past decade. Characterization of dysregulated miRNA expression profiles could give a better understanding of the development of pathological conditions and clinical disorders, such as autoimmune diseases with polygenic etiology, including idiopathic inflammatory myopathies (IIMs). IIMs are a group of rare autoimmune disorders characterized by skeletal weakness and inflammation. Polymyositis (PM) is one of the conditions of autoimmune myopathies with proximal skeletal muscle weakness. A novel group of miRNAs, known as myomiRs are described as striated muscle-specific or muscle-enriched miRNAs. They are involved in myoblast proliferation/differentiation as well as muscle regeneration. To determine the role of myomiRs in the development and progression of PM, we performed an initial skeletal muscle miRNA profiling using microarray technique at diagnosis. The aim of the study was to examine myomiRs expression profile in patients with PM in order to remark the association between the dysregulated myomiRs’ expression and the development of the disease. As a results of microarray investigation, most of the myomiRs showed altered expression patterns in the muscle samples of PM patients compared to controls. These results suggest that myomiRs, especially miR-1, miR-133a, miR-208b, miR-486, and miR-499 function in a network, and are associated with the development of PM.

Objectives To determine prevalence and co-existence of myositis specific autoantibodies (MSAs) and myositis associated autoantibodies (MAAs) and associated clinical characteristics in a large cohort of idiopathic inflammatory myopathy (IIM) patients. Methods Adult patients with confirmed IIM recruited to the EuroMyositis registry (n = 1637) from four centres were investigated for the presence of MSAs/MAAs by radiolabelled-immunoprecipitation, with confirmation of anti-MDA5 and anti-NXP2 by ELISA. Clinical associations for each autoantibody were calculated for 1483 patients with a single or no known autoantibody by global linear regression modelling. Results MSAs/MAAs were found in 61.5% of patients, with 84.7% of autoantibody positive patients having a sole specificity, and only three cases (0.2%) having more than one MSA. The most frequently detected autoantibody was anti-Jo-1 (18.7%), with a further 21 specificities each found in 0.2–7.9% of patients. Autoantibodies to Mi-2, SAE, TIF1, NXP2, MDA5, PMScl and the non-Jo-1 tRNA-synthetases were strongly associated (p, Highlights • Myositis specific autoantibodies very rarely coexist in the one individual allowing endotypes to be more precisely defined. • The association of anti-TIF1 and cancer-associated myositis is confirmed with a cut-off age of over 58 years. • In a large combined European myositis cohort associations of anti-SRP with carditis and anti-Mi-2 with cancer have emerged. • Myositis associated autoantibodies are strongly associated with having myositis in association with another connective tissue disease.

Introduction: In idiopathic inflammatory myopathies, the presence of anti-Jo-1 antibody defines a distinct clinical phenotype (myositis, arthritis, interstitial lung disease, Raynaud’s phenomenon fever, mechanic’s hands), called antisynthetase syndrome. Aim: To determine the demographic data as well as clinical, laboratory and terapeutical features of anti-Jo1 positive patients, followed by the department of the authors. Method: The medical records of 49 consecutive anti-Jo1 patients were reviewed. Results: Demographic and clinical results were very similar to those published by other centers. Significant correlation was found between the anti-Jo-1 titer and the creatine kinase and C-reactive protein levels. Distinct laboratory results measured at the time of diagnosis of the disease (C-reactive protein, antigen A associated with Sjogren’s syndrome, positive rheumatoid factor), and the presence of certain clinical symptoms (fever, vasculitic skin) may indicate a worse prognosis within the antisyntetase positive patient group. Conclusion: In the cases above more agressive immunosuppressive therapy may be required. Orv. Hetil., 2016, 157(15), 575–583.

Background:Overlap syndromes are autoimmune disorders in which classification criteria of at least two connective tissue diseases (CTDs) are fulfilled. The Division of Clinical Immunology in Debrecen, Hungary oversees patients with idiopathic inflammatory myopathies (IIMs) since the 1980’s.Objectives:In a work called The Myositis Antibody Research Project, which is coordinated together with the Bath Institute for Rheumatic Diseases (UK), authors investigated 330 patients with myositis, all treated in this Hungarian center.Methods:The aim of this retrospective study was to investigate the frequency, clinical and serological parameters and therapeutic options in myositis – rheumatoid arthritis overlap cases in this group of 330 patients.Results:The frequency of overlap cases, as seen in literature, was 13.64%. The importance of myositis-associated antibodies (MAAs) can be seen on this results: 44.44% of overlap patients, while only 7.02% of not overlap patients were MAA positive. Out of our 45 overlap patients twenty-seven myositis - RA overlap patients could be identified. Among these 27 patients the women:men ratio was 12.5:1, PM:DM ratio was 2.375:1. Muscle weakness and sceletal involvement was present in every patient, myalgia in 77.78% and Raynaud’s phenomenon in 48.15% of the patients; general symptoms during disease relapse (fatigue, weight loss, fever) were present in 59.26%, 18.52% and 14.82%, respectively. Ten cases had seropositive rheumatoid arthritis, with high titer of rheumatoid factor. Authors underline the importance of lung involvement as internal organ manifestation: 8 patients had anti-synthetase antibodies (six anti-Jo-1, one anti-PL-7 and one anti-PL-12). In anti-PL-7 and anti-PL-12 positive patients interstitial lung disease (ILD) appeared some years before myositis and early agressive management of ILD resulted in fast remission of muscle weakness. Anti-Mi-2 and anti-SRP could be detected in two patients. Most frequent used disease modifying antirheumatic drugs were cyclophosphamide, methotrexate and cyclosporine, 19 patients received DMARD combination therapy. Biological therapy was used in 3 patients, intravenous or subcutaneous immuneglobulin in 4 patients and one patient with RA – myositis – antiphospholipid syndrome died because of asipartion pneumonia.Conclusion:Authors conclude that it is really challenging to treat these overlap cases: they form a very heterogenous group of patients and management has to be personalized.Disclosure of Interests:None declared

Background Assessing disease activity in patients with myositis is an important goal. A method called Disease Activity Core Set Measures was established by International Myositis Assessment and Clinical Studies Group (IMACS) for evaluating activity of myositis. This assesses the manifestations of myositis which are thought to be reversible that result directly from the inflammatory process. Objectives In this prospective study authors aimed to monitoring disease activity in Hungarian myositis patients treated at the Autoimmune Outpatient Clinic, Department of Clinical Immunology, University of Debrecen. First, disease activity data of poly (PM) – and dermatomyositis (DM) patients were compared. Secondly, by patients with active disease, the correlations between the components of Disease Activity Core Set Measures were studied. Methods During the three-year follow-up period, at every single visit, authors evaluated disease activity using factors of Disease Activity Core Set Measures and patients filled Health Assessment Questionnaire (HAQ). Only those patients who appeared at least three times during the follow-up period were selected. Statistical analysis was made using SPSS 17.0 statistical software. Results Collection of data from 219 patients (age: 44.93 years, female: male ratio 2.98:1) happened in a total of 1101 outpatient visits. The 107 PM patients had statistically significant higher physician and patient visual analogue (VAS) and myositis disease activity assessment tools scores than the 61 DM patients, with the exception of cardiovascular disease activity. Activity of skin lesions was significant higher in DM patients. By the 44 patients, who had an active disease, the MMT scores gave significant negative correlations with HAQ scores (R=−0.536 and p Conclusions As far as we know, this is the first study which compares the activities of PM and DM patients based on the IMACS system. According to our data, we can conclude that PM patients had more severe muscle symptoms and extramuscular manifestations. Secondly, as seen in other previous studies, calculations in patients with active myositis showed a clear correlation between the most important components of Disease Activity Core Set Measures. Disclosure of Interest None declared

Antiphospholipid syndrome is characterized by multiple arterial and/or venous thrombotic events, recurrent fetal losses in the presence of antiphospholipid antibodies (aPL). Catastrophic antiphospholipid syndrome is a life-threatening, rare subset of antiphospholipid syndrome when the thrombotic events affect at least three organs, and clinical manifestations develop simultaneously or within a week. Diagnostically, small vessel occlusions can be detected by histopathology in the presence of aPL. Our case report describes an 18-year-old man who has been treated for antiphospholipid syndrome associated with systemic lupus erythematosus (SLE) since 2011. The clinical findings were dominated by recurrent deep vein thrombosis, and severe proteinuria caused by lupus nephritis, accompanied by mild serological and laboratory findings. The patient was hospitalized in March 2014 because of severe thrombocytopenia and infective diarrhoea. At this time the renal functions deteriorated rapidly. Simultaneously, left upper extremity paresis was observed; computed tomography showed ischaemic lesions in the territory of the middle cerebral artery. Abdominal discomfort and pain occurred. On computed tomography scan ischaemic lesions were seen in the spleen, the right kidney and the coeliac trunk. Laboratory and serological findings verified the presence of aPL and anti-DNA antibodies, anaemia and thrombocytopenia. Based on the above-mentioned clinical and laboratory findings, the diagnosis of catastrophic antiphospholipid syndrome was established. Anticoagulation, corticosteroids and plasma exchange treatment, as well as haemodiafiltration were initiated. Although the thrombotic cascade decelerated following these interventions, we could not see an improvement in the renal function. Rituximab treatment was started, leading to a significant improvement in renal function. After 5 weeks of treatment the patient was discharged from hospital.

Hintergrund Die idiopathischen inflammatorischen Myositiden sind chronische, systemische Autoimmunkrankheiten, die durch symmetrische, proximale Muskelschwache charakterisiert sind. Die klinisch-pathologische Einteilung scheint heutzutage obsolet zu sein, deshalb ist eine immunserologische Klassifikation erstellt worden.

Idiopathic inflammatory myopathies are a group of clinically heterogeneous diseases, which have been classified by myositis specific antibodies recently. The anti-SRP positive subset of this group is characterized by more severe clinical prognosis than other myositis specific antibody positive types.Our goal was to compare 16 anti-SRP positive patients in the Division of Clinical Immunology, Department of Internal Medicine, University of Debrecen with 16 antibody negative ones.Muscle strength validated in both groups by the manual muscle test proved to be significantly decreased both before and after therapy (χMuscle-specific inflammatory laboratory parameters showed significant difference only in case of LDH-levels after therapy. Both groups showed good clinical response to first line steroid treatment, yet the significantly higher rate of second line administration suggests worse therapeutic response of the antibody positive group.Based on these facts we determined poor clinical prognosis and therapeutic response of the anti-SRP positive group. Orv Hetil. 2017; 158(35): 1382-1389.

Abstract: Introduction: Idiopathic inflammatory myopathies are a group of clinically heterogeneous diseases, which have been classified by myositis specific antibodies recently. The anti-SRP positive subset of this group is characterized by more severe clinical prognosis than other myositis specific antibody positive types. Aim: Our goal was to compare 16 anti-SRP positive patients in the Division of Clinical Immunology, Department of Internal Medicine, University of Debrecen with 16 antibody negative ones. Method: Muscle strength validated in both groups by the manual muscle test proved to be significantly decreased both before and after therapy (χ2 = 0.006 and 0.019) in the anti-SRP positive group. Results: Muscle-specific inflammatory laboratory parameters showed significant difference only in case of LDH-levels after therapy. Both groups showed good clinical response to first line steroid treatment, yet the significantly higher rate of second line administration suggests worse therapeutic response of the antibody positive group. Conclusion: Based on these facts we determined poor clinical prognosis and therapeutic response of the anti-SRP positive group. Orv Hetil. 2017; 158(35): 1382–1389.

Revue du rhumatisme - In Press.Proof corrected by the author Available online since vendredi 3 fevrier 2017

The authors discuss a rare case of a 25-year-old female patient having dermatomyositis associated with celiac disease and ulcerative colitis. The idiopathic inflammatory myopathies are systemic, chronic, immune-mediated diseases characterized by proximal, symmetrical muscle weakness. Many examples from the literature refer that celiac disease occurs more often in patients with myositis than in the general population, but its association with ulcerative colitis is a real rarity in the international literature. Orv. Hetil., 2014, 155(26), 1033–1038.

Idiopathic inflammatory myopathies are systemic, immune-mediated diseases characterized by proximal, symmetrical, progressive muscle weakness. The aim of this work is to give an overview of the biological therapy used in the treatment of idiopathic inflammatory myopathies. The authors also focus on novel results in the therapy directed against the B- and T-cells. They emphasize the importance of new trials in these diseases which may lead to the introduction of novel therapeutic options in these disorders.Az idiopathiás inflammatorikus myopathiák szisztémás, a proximalis végtagizmok progresszív, szimmetrikus gyengeségével jellemezhető immunmediált betegségek. A szerzők áttekintést adnak a gyulladásos izombetegségek esetén alkalmazott biológiai terápiáról. Külön figyelmet fordítanak a B- és T-sejt-gátló terápia legújabb eredményeire. Rávilágítanak az új tanulmányok fontosságára ezen betegségekben. Mindez elvezethet új terápiás lehetőségek bevezetéséhez is. Orv. Hetil., 2014, 155(1), 3–10.

In idiopathic inflammatory myopathies, the presence of anti-Jo-1 antibody defines a distinct clinical phenotype (myositis, arthritis, interstitial lung disease, Raynaud's phenomenon fever, mechanic's hands), called antisynthetase syndrome.To determine the demographic data as well as clinical, laboratory and terapeutical features of anti-Jo1 positive patients, followed by the department of the authors.The medical records of 49 consecutive anti-Jo1 patients were reviewed.Demographic and clinical results were very similar to those published by other centers. Significant correlation was found between the anti-Jo-1 titer and the creatine kinase and C-reactive protein levels. Distinct laboratory results measured at the time of diagnosis of the disease (C-reactive protein, antigen A associated with Sjogren's syndrome, positive rheumatoid factor), and the presence of certain clinical symptoms (fever, vasculitic skin) may indicate a worse prognosis within the antisyntetase positive patient group.In the cases above more agressive immunosuppressive therapy may be required.

Joint Bone Spine - In Press.Proof corrected by the author Available online since mardi 13 octobre 2015

Myositis is an autoimmune disease characterised by proximal muscle weakness.The aim of the authors was to determine the frequency of dermatomyositis-specific autoantibodies (anti-Mi-2, anti-transcriptional intermediary factor 1 gamma, anti-nuclear matrix protein 2, anti-small ubiquitin-like modifier activating enzyme, anti-melanoma differentiation-associated gene) in a Hungarian myositis population and to compare the clinical features with the characteristics of patients without myositis-specific antibodies.Antibodies were detected using immunoblot and immunoprecipitation.Of the 330 patients with myositis, 48 patients showed dermatomyositis-specific antibody positivity. The frequency of antibodies in these patients was lower than those published in literature Retrospective analysis of clinical findings and medical history revealed that patients with dermatomyositis-specific autoantibody had more severe muscle weakness and severe skin lesions at the beginning of the disease.Antibodies seem to be useful markers for distinct clinical subsets, for predicting the prognosis of myositis and the effectiveness of the therapy.

Introduction: Myositis is an autoimmune disease characterised by proximal muscle weakness. Aim: The aim of the authors was to determine the frequency of dermatomyositis-specific autoantibodies (anti-Mi-2, anti-transcriptional intermediary factor 1 gamma, anti-nuclear matrix protein 2, anti-small ubiquitin-like modifier activating enzyme, anti-melanoma differentiation-associated gene) in a Hungarian myositis population and to compare the clinical features with the characteristics of patients without myositis-specific antibodies. Method: Antibodies were detected using immunoblot and immunoprecipitation. Results: Of the 330 patients with nyositis, 48 patients showed dermatomyositis-specific antibody positivity. The frequency of antibodies in these patients was lower than those published in literature Retrospective analysis of clinical findings and medical history revealed that patients with dermatomyositis-specific autoantibody had more severe muscle weakness and severe skin lesions at the beginning of the disease. Conclusions: Antibodies seem to be useful markers for distinct clinical subsets, for predicting the prognosis of myositis and the effectiveness of the therapy. Orv. Hetil., 2015, 156(36), 1451–1459.

Idiopathic inflammatory myopathies are autoimmune diseases characterized by symmetrical proximal muscle weakness. Our aim was to identify a correlation between VDR polymorphisms or haplotypes and myositis. We studiedVDR-BsmI,VDR-ApaI,VDR-TaqI, andVDR-FokIpolymorphisms and haplotypes in 89 Hungarian poly-/dermatomyositis patients (69 females) and 93 controls (52 females). We did not obtain any significant differences forVDR-FokI,BsmI,ApaI, andTaqIgenotypes and allele frequencies between patients with myositis and healthy individuals. There was no association of VDR polymorphisms with clinical manifestations and laboratory profiles in myositis patients. Men with myositis had a significantly different distribution ofBB,Bb, andbbgenotypes than female patients, control male individuals, and the entire control group. Distribution ofTT,Tt, andttgenotypes was significantly different in males than in females in patient group. According to four-marker haplotype prevalence, frequencies of sixteen possible haplotypes showed significant differences between patient and control groups. The three most frequent haplotypes in patients were thefbAt,FBaT, andfbAT. Our findings may reveal that there is a significant association:BbandTtgenotypes can be associated with myositis in the Hungarian population we studied. We underline the importance of our result in the estimated prevalence of four-marker haplotypes.

Objective.To identify early echocardiographic abnormalities at the time of diagnosis of polymyositis (PM) and dermatomyositis (DM) and follow the echocardiographic findings during the first 3 months of therapy.Methods.We included 30 PM/DM patients (23/7) with a mean age of 42.3 ± 1.6 years and without cardiovascular symptoms. Age-matched healthy patients served as controls. Clinical characteristics were recorded. Traditional echocardiography and tissue Doppler imaging (TDI) were performed to measure systolic [ejection fraction, right ventricular fractional area change (RV FAC), lateral and tricuspid annulus s velocities] and diastolic echocardiographic variables (mitral inflow velocities: E, A; deceleration time: DT; lateral and tricuspid annulus e′, a′ velocities, lateral E/e′).Results.The left and right ventricular systolic dysfunction detected by TDI at the time of the PM/DM diagnosis improved, and characteristic values at the end of the followup period were comparable to those of the controls (lateral s: 10.6 ± 0.2, 8.7 ± 0.4, 9.6 ± 0.3, 11.3 ± 0.2 cm/s; RV FAC: 45.2 ± 2.3, 36.9 ± 1.5, 42.2 ± 1.3, 46.9 ± 1.2%; tricuspid s: 13.3 ± 0.2, 9.5 ± 0.4, 10.3 ± 0.3, 11.6 ± 0.5 cm/s; control, 0, 1, and 3 mos, respectively). Measurements indicated the development of diastolic dysfunction at 3 mos (E/A: 1.4 ± 0.1, 1.29 ± 0.05, 1.03 ± 0.05, 0.92 ± 0.05; DT: 148.6 ± 3.6, 157.3 ± 5.7, 168.3 ± 6.0, 184.3 ± 6.2 ms; lateral e′: 12.8 ± 0.3, 12.1 ± 0.5, 10.2 ± 0.6, 10.8 ± 0.8 cm/s; E/e′: 5.6 ± 0.1, 5.0 ± 0.22, 6.92 ± 0.46, 7.64 ± 0.47; control, 0, 1, and 3 mos, respectively).Conclusion.TDI is a useful method to detect early cardiac abnormalities complementing the conventional echocardiographic measurements. LV and RV systolic dysfunction found in the acute phase significantly improved during the first 3 months of therapy; however, deterioration of diastolic dysfunction was also observed.

Idiopathic inflammatory myopathy (called also myositis) is a systemic autoimmune disease mainly characterised with proximal muscle weakness. The most frequent subsets are polymyositis and dermatomyositis. The epidemiology of these diseases is not entirely explored. There is a need to build national and international registries which may help to obtain more data. The Myositis Team at the Department of Clinical Immunology, University of Debrecen, has been established in 1975.The aim of the authors was to obtain epidemiological data on this disease.The authors analysed the database of the National Health Insurance Fund Administration of Hungary which included 1119 patients with myositis, of which 289 patients were followed up by the authors.The average incidence of the disease was found to be 0.95/100.000/year. The male/female ratio was 1/2. Dermatomyositis occurred both in children and adult, but polymyositis was found mainly in adults. These epidemiological data partly correlate with those published in the international literature.The authors propose to establish a National Myositis Registry in the frame of multicentric collaboration in order to have more information about the disease.Bevezetés: Az idiopathiás inflammatoricus myopathiák (más néven myositisek) szisztémás autoimmun betegségek, amelyek közös jellemzője az immunmediált izomgyulladás és a proximalis izomgyengeség. Két leggyakoribb kórformája a polymyositis és dermatomyositis. A betegség etiológiája részben ismert, epidemiológiai adatok csak néhány országban állnak rendelkezésre. Folyamatban van egy nemzetközi és egy magyar myositisregiszter kialakítása, amely adatbázisok elősegítenék a betegségek pontosabb megismerését. A Debreceni Egyetem Klinikai Immunológia Tanszék Myositis Munkacsoportja 1975 óta gondoz myositises betegeket. Célkitűzés: A szerzők a magyar myositises betegek epidemiológiai adatainak felmérését tűzték ki célul. Módszer: Az Országos Egészségbiztosítási Pénztár adatbázisa szerint 1999 és 2010 között myositis miatt kezelt 1119 beteg, köztük a szerzők tanszékén gondozott 289 beteg adatait elemezték. Eredmények: Megállapították, hogy a myositis átlagos incidenciája 0,95/100000 fő évente. A férfi:nő arány 1:2. Dermatomyositis gyermekeknél és felnőtteknél egyaránt előfordul, de a polymyositis elsősorban a felnőttek betegsége. A magyar epidemiológiai adatok részben korrelálnak a korábbi irodalmi adatokkal. Következtetések: A szerzők célja multicentrikus együttműködés keretein belül egy részletes nemzeti myositisregiszter létrehozása az adatok további elemzése céljából. Orv. Hetil., 2014, 155(41), 1643–1646.

Idiopathic inflammatory myopathies are systemic autoimmune diseases characterized by progressive proximal muscle weakness. Cancer-associated myositis represents the worst prognostic group within this heterogeneous disease.The aim of this study was to reveal factors which increase the risk factors for association of cancerous disease in patients with myositis. Furthermore, the authors explored the most common types of associated malignancies in their patients with myositis and characterize the clinical findings in a sub-group of anti-p155/140 positive patients.In this retrospective study, myositis patients with and without associated cancer were analysed (32 and 64 patients, respectively). In addition, anti-p155/140 positive and negative groups were compared, irrespective to the presence of associated malignancies.The risk for associated malignancy was higher in patients with severe muscle and skin symptoms and those with dermatomyositis. Furthermore, increased risk for malignancy was noted in the presence of particular skin symptoms and the absence of systemic symptoms. The anti-p155/140 antibody was proved to be a feasible marker of an independent clinical sub-group which overlapped clinical characteristics with cancer-associated myositis.These results may help the identification of patients with myositis with a higher risk for associated malignancy.Bevezetés: Az idiopathiás inflammatoricus myopathiák szisztémás autoimmun betegségek, jellegzetességük a proximalis végtagövi izmok progresszív gyengesége. A heterogén betegségcsoport legrosszabb prognózisú alcsoportja a tumorral társult myositis. Célkitűzés: Jelen tanulmány célja, hogy felderítse azokat a tényezőket, amelyek társuló daganatos megbetegedés kialakulásának kockázatát fokozzák myositises betegek esetében. További célja, hogy meghatározza a myositissel gyakran társuló daganatok típusait; az anti-p155/140 antitest jelenlétével összefüggő klinikai kép jellegzetességeit, különös tekintettel az antitest és a tumorral társult myositis kapcsolatára. Módszer: A szerzők a vizsgálatban részt vevő betegek klinikai dokumentációinak retrospektív elemzésével összehasonlították a kialakított primer, tumorral nem társult (n = 64) és tumorral társult (n = 32) myositises betegcsoportokat; valamint anti-p155/140 antitest pozitív és negatív alcsoportokat a legfontosabb klinikai, laboratóriumi és immunológiai tulajdonságok szempontjából. Eredmények: Daganat kialakulásának kockázatát növelő tényezők a súlyos izom- és bőrtünetek, dermatomyositis diagnózisa, egyes bőrtünetek jelenléte és a szisztémás tünetek hiánya. Az anti-p155/140 antitest önálló szerológiai alcsoportot jelöl ki, amely tulajdonságaiban átfedést mutat a tumorral társult myositissel. Következtetések: A vizsgálat eredményei segíthetik a daganat kialakulásának nagyobb kockázatával bíró myositises betegek azonosítását. Orv. Hetil., 2014, 155(36), 1437–1444.

The authors discuss a rare case of a 25-year-old female patient having dermatomyositis associated with celiac disease and ulcerative colitis. The idiopathic inflammatory myopathies are systemic, chronic, immune-mediated diseases characterized by proximal, symmetrical muscle weakness. Many examples from the literature refer that celiac disease occurs more often in patients with myositis than in the general population, but its association with ulcerative colitis is a real rarity in the international literature.A szerzők az idiopathiás inflammatorikus myopathia ritka, colitis ulcerosával és coeliakiával társult esetét mutatják be egy általuk kezelt, 25 éves dermatomyositises nőbeteg kórtörténetén keresztül. Az idiopathiás inflammatorikus myopathiák szisztémás, krónikus, immunmediált megbetegedések, amelyeket a proximalis végtagizmok szimmetrikus gyengesége jellemez. Irodalmi példák utalnak rá, hogy a coeliakia gyakrabban jelentkezik myositisben, mint az átlagpopulációban. Colitis ulcerosával való asszociációja azonban irodalmi ritkaságnak számít. Orv. Hetil., 2014, 155(26), 1033–1038.

To the Editor: Pregnancy in autoimmune diseases may be complicated for both the fetus and the mother. Polymyositis (PM) and dermatomyositis (DM) are autoimmune diseases that affect women more often than men, and can occur before childbearing years1,2. Complications during pregnancy were frequently reported in patients with PM/DM with active disease, whereas there seems to be a low risk for the fetus and mother in women with a well-controlled disease at time of conception2,3,4,5,6,7,8,9,10. Our aims were to assess the pregnancy outcome in myositis. Based on international collaboration, records for women with PM/DM who had been pregnant after the diagnosis of myositis were searched in 4 countries using the clinical databases of contributing hospitals. There were 23 women identified: Czech Republic (n = 8), Hungary (n = 9), Sweden (n = 5), and Poland (n = 1). All 23 women were white. Medical records were retrospectively reviewed using a standardized protocol. Normal delivery was defined when a healthy newborn weighing > 2500 g was delivered after 37 weeks of pregnancy. Premature delivery was defined when the pregnancy … Address correspondence to Dr. M. Nagy-Vincze, Division of Clinical Immunology, Department of Internal Medicine, University of Debrecen, Moricz Zs. u. 22, H-4032 Debrecen, Hungary. E-mail: melinda.nagyvincze{at}gmail.com

Background The most frequent myositis specific antibody (MSA) in the serum of patients with idiopathic inflammatory myopathies is anti-Jo-1. The presence of anti-Jo-1 define a distinct clinical phenotype, antisynthetase syndrome (ASS), which is characterized by poor prognosis and multiple organ involvement, such as myositis, interstitial lung disease (ILD), non-erosive arthritis, Raynaud9s phenomen, mechanic9s hand, skin rashes, and fever. Objectives The aim of this study was to determine the clinical, serological, laboratory and genetic features of anti-Jo1 positive patients followed by our department. Methods Retrospective analysis of medical records of 49 patients (42 female, 7 male) were reviewed. Anti-Jo-1 titer was detected with enzyme-linked immunosorbent assay. HLA DRB1, DQA1 and DQB1 genotype was determined using commercial sequence-specific oligonucleotide kit. Statistical analysis was performed using Pearson Chi 2 , Fisher exact test, or Spearman correlation. Results The median age at diagnosis was 43±13,28 years (range: 18-70), 48 patients exhibited myositis (98%), 35 ILD (73%), 43 arthritis (88%), 32 Raynaud9s phenomen (65%), 21 fever (43%), 16 mechanic9s hand (33%), 27 skin rash (55%) and 6 dysphagia (12%). We could detect significant correlation between the initial anti-Jo-1 titer and the first CK (R=0,328; p=0,003) and CRP (R=0,374; p=0,016) level. Furthermore anti-Jo1 levels during disease course had significant correlation to the corresponding CK (R=0,497; p Conclusions Our results confirm previously reported data from other centers considering the clinical features of anti-Jo1 positive patients. HLA DRB1*03 positivity was associated with lower CK level but has no influence on clinical or serological features. It seems that anti-Jo1 level might reflect disease activity; ESR, CRP levels, associated fever and anti-SSA positivity at the diagnosis could be considered as prognostic markers. Disclosure of Interest None declared