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Several autoimmune rheumatic diseases have been associated with accelerated atherosclerosis or other different types of vasculopathy depending on the underlying disease, leading to increased cardio- and cerebrovascular disease risk. Polymyositis (PM) and dermatomyositis (DM), members of idiopathic inflammatory myopathies (IIMs), a group of systemic autoimmune diseases are also associated with elevated risk of cardiovascular diseases (CVD). Up until now, no specific data is known on the mechanisms, risk factors, or possible vasculopathy leading to increased CVD risk. The aims of the present study were to assess the flow-mediated dilatation of the brachial artery by a TensioClinic arteriograph and to measure the thickness of carotid artery intima-media, the augmentation index, and the pulse wave velocity using high-resolution ultrasonography in a cohort of PM and DM patients. We also investigated the correlation of these parameters with the traditional risk factors of atherosclerosis and overall cardiovascular status within PM and DM patients. Twenty-seven patients (21 females, six males) with IIMs were enrolled in this study, and 38 healthy individuals matched for sex and age served as controls. We found a decreased flow-mediated dilatation in the brachial artery (6.36 vs. 8.39 %) with increased arterial stiffness and carotid artery thickness in our patients compared to healthy controls. We found significantly decreased flow-mediated dilatation of the brachial artery (5.57 vs. 8.39 %) in DM patients. We also detected a correlation between these parameters and the traditional cardiovascular risk factors, as well as hypertriglyceridemy, hypertension, and peripheral arterial disease. In DM, overall, more vascular abnormalities were found than in PM. Our findings suggest that flow-mediated dilatation of the brachial artery, arterial stiffness, and carotid artery thickness measurements could be beneficial for predicting the CVD risk in myositis patients. Further investigations need to find the potential differences and role of inflammation and immune mechanisms in atherosclerotic processes in DM and PM.

Inclusion body myositis is a slowly progressive myopathy affecting predominantly the middle-aged and older patient population. It is a major form of the idiopathic inflammatory myopathies which are chronic systemic autoimmune diseases characterized by symmetrical proximal muscle weakness. Unfortunately, there is no effective therapy yet; however, the early diagnosis is essential to provide treatment options which may significantly slow the progression of the disease. In our case-based clinicopathological study the importance of the close collaboration between the clinician and the neuropathologist is emphasised.

Idiopathic inflammatory myopathies are systemic autoimmune diseases characterized by symmetrical proximal muscle weakness. One of them is the subgroup of necrotizing autoimmune myopathy, which has recently been recognized as a separate entity. In addition to the typical symmetrical muscle weakness, it is characterized by very high creatine kinase levels, myopathic triad in the electromyography, and myocyte necrosis without significant inflammation. The paper aims to review this rare entity, which has to be diagnosed and treated quickly in every case. Orv. Hetil., 2012, 153, 1502–1507.

Inflammatory myopathies are chronic, immune-mediated diseases characterised by progressive proximal muscle weakness. They encompass a variety of syndromes with protean manifestations. The diagnosis is based on Bohan and Peter's classification criteria, which nowadays seem to be obsolete. Our increasing knowledge about the risk factors, genetic susceptibility and immunological pathways in the disease mechanism leads to the establishment of a new, immunogenetically and serologically validated diagnostic criteria system. The treatment of idiopathic inflammatory myopathy is also a complex task requiring much experience. The aims of therapy are to increase muscle strength, prevent the development of contractures and manage the systemic manifestations of the disease. The most important one is the early detection of diseases and patients' immunological control in special centres. Using the basis therapeutic drugs temporary or permanent remission can be achieved, which improves patientsG' quality of life and functional ability. Rehabilitation and physiotherapy in the remission period may significantly improve the outcome of patients with functional disorders. The introduction of new biological therapies further allows us to control the myositis patients' state more effectively. The aim of this review is to summarise our knowledge about clinical symptoms, pathomechanism, as well as genetic, serologic and environmental risk factors. We would also like to present the way to diagnosis and the latest research about diagnostic criteria system, proposed outcome measures and therapeutic possibilities.

Inflammatory myopathies are chronic, immune-mediated diseases characterized with progressive proximal muscle weakness. They encompass a variety of syndromes with protean manifestations. The aims of therapy are to increase muscle strength, prevent the development of contractures, and to manage the systemic manifestations of the disease. This is a complex treatment which requires routine and wide knowledge. The most important task is to recognize the disease and guide the patient to immunologic center. Although the first line of therapy continues to include corticosteroids, there are a multitude of agents available for treating patients with myositis. There are several different immunosuppressive agents which may be applied alone or in combination with each other, as well as an increasing number of novel and exciting biologic agents targeting molecules participating in the pathogenesis of inflammatory myopathy. Physiotherapy and rehabilitation in the remission period may significantly improve the functional outcome of patients with these disorders. Orv. Hetil., 2011, 152, 1552–1559.

The idiopathic inflammatory myopathies are systemic, chronic autoimmune diseases characterized by proximal symmetrical muscle weakness. One of the main diseases in this group is inclusion body myositis (IBM), an underdiagnosed, progressive muscle disease characteristically affecting the middle-aged and older population. It has a slow, relentlessly progressive course. The precise pathogenesis of the disease remains unknown. In most of the cases it is diagnosed a few years after the appearance of the first symptoms. The muscle biopsy typically shows endomysial inflammation, with invasion of mononuclear cells into the non-necrotic fibers, and also rimmed vacuoles. It appers, that both inflammation and degeneration are present at the onset of the disease. Our aim is to raise awareness about this disease which leads to severe disability, with clinicopathological case presentations and literature overview, emphasizing the importance of collaboration between the clinician and the neuropathologist. No effective therapy is currently available but the rapid diagnosis is essential to slow disease progression. Although this is a relatively rare disease, patients are presenting not only in immunology outpatient clinics; our reports aims to raise awareness and facilitate accurate early diagnosis of IBM.

Inclusion body myositis is a rare, late-onset myopathy. Both inflammatory and myodegenerative features play an important role in their pathogenesis. Overlapping clinicopathological entities are the familial inclusion body myopathies with or without dementia. These myopathies share several clinical and pathological features with the sporadic inflammatory disease. Therefore, better understanding of the genetic basis and pathomechanism of these rare familial cases may advance our knowledge and enable more effective treatment options in sporadic IBM, which is currently considered a relentlessly progressive incurable disease.

Resume Nous rapportons le cas d’une patiente ayant presente une polymyosite avec presence d’anticorps anti-Jo1 associee a un adenocarcinome pulmonaire. Le diagnostic de polymyosite a repose sur les symptomes, les resultats electromyographiques et l’elevation des enzymes musculaires dont la creatine kinase et la lactate dehydrogenase. La positivite des anticorps anti-Jo1, l’hyperleucocytose et l’elevation des marqueurs tumoraux CA 15-3 et CA 72-4 ont egalement permis le diagnostic positif. Le caractere malin de la tumeur a ete confirme par la biopsie transthoracique a l’aiguille, realisee deux ans apres l’apparition des premiers signes cliniques de polymyosite. Apres l’exerese tumorale, la polymyosite etait en remission. Etant donnes les symptomes et la survenue concomitante de la tumeur et de la polymyosite, une origine paraneoplasique a ete suspectee.

Idiopathic inflammatory myopathies are systemic, autoimmune diseases characterized by proximal symmetrical muscle weakness. We review the myositis-associated and myositis-specific autoantibodies, among them the anti-SRP autoantibody. Among those autoimmune myopathy cases, that are associated with autoantibodies, we can detect anti-SRP autoantibody positive myositis cases. We describe the role of signal recognition particle, its structure and role in protein biosynthesis. We review how necrotizing autoimmune myopathy is identified, and the differences from classical polymyositis. The anti-SRP titer correlates with disease activity. We present some cases to show how the disease appears in childhood and also some rare cases from the literature. Finally we present a case to draw attention to the importance of this disease.

Idiopathic inflammatory myopathies are chronic, systemic autoimmune diseases, characterized by symmetric and progressive weakness of proximal muscles in the upper and lower extremities. Treatment of the disease presents a complex challenge and it needs practical knowledge. In this review the authors summarize current treatment options, and discuss intravenous immunoglobulin treatment in therapy-unresponsive cases. Relevant data from the international literature is collected, too. Benefits and side effects of this treatment are also disclosed.Az idiopathiás gyulladásos myopathiák krónikus, szisztémás autoimmun betegségek, amelyek a végtagizmok szimmetrikus, progresszív gyengeségével járnak. A betegség kezelése nagy gyakorlatot igénylő, összetett feladat. A közleményben a szerzők áttekintik a jelenleg rendelkezésre álló kezelési lehetőségeket idiopathiás gyulladásos myopathiákban, majd részletesen foglalkozunk az intravénás immunglobulin alkalmazhatóságával a terápiarefrakter esetekben. A szerzők célja, hogy minél több irodalmi példával, nemzetközi tapasztalattal támasszák alá a módszer hatékonyságát, figyelve előnyeire és esetleges hátrányaira is. Orv. Hetil., 2013, 154, 723–728.

Autoantibodies against several aminoacyl-transfer-RNA synthetases have been described in patients with myositis; anti-threonyl-tRNA synthetase (anti-PL-7) is one of the rarest. We describe the clinical and laboratory characteristics of a cohort of European anti-PL-7 patients, and compare them with previously reported cases. This multicenter study of patients positive for anti-PL-7, identified between 1984 and 2011, derives from the EUMYONET cohort. Clinical and serologic data were obtained by retrospective laboratory and medical record review, and statistical analyses were performed with chi-squared and Fisher exact tests. Eighteen patients, 15 women, were anti-PL-7 antibody positive. Median follow-up was 5.25 years (interquartile range, 2.8-10.7 yr), and 4 patients died. All patients had myositis (12 polymyositis, 5 dermatomyositis, and 1 amyopathic dermatomyositis), 10 (55.6%) had interstitial lung disease, and 9 (50%) had pericardial effusion. Occupational exposure to organic/inorganic particles was more frequent in patients with interstitial lung disease than in the remaining patients (5 of 10 vs. 1 of 7; p = 0.152), although the difference was not significant. Concurrent autoantibodies against Ro60 and Ro52 were seen in 8 of 14 (57%) patients studied. In the literature review the most common manifestations of anti-PL-7 antisynthetase syndrome were interstitial lung disease (77%), myositis (75%), and arthritis (56%). As in other subsets of the antisynthetase syndrome, myositis and interstitial lung disease are common features of the anti-PL-7 antisynthetase syndrome. In addition, we can add pericarditis as a possible manifestation related to anti-PL-7 antibodies.

Objectives HLA-DRB1*03 is strongly associated with anti-Jo-1-positive idiopathic infl ammatory myopathies (IIM) and there is now increasing evidence that Jo-1 antigen is preferentially expressed in lung tissue. This study examined whether smoking was associated with the development of anti-Jo-1 antibodies in HLA-DRB1*03-positive IIM. Methods IIM cases were selected with concurrent information regarding HLA-DRB1 status, smoking history and anti-Jo-1 antibody status. DNA was genotyped at DRB1 using a commercial sequence-specifi c oligonucleotide kit. Anti-Jo-1 antibody status was established using a line blot assay or immunoprecipitation. Results 557 Caucasian IIM patients were recruited from Hungary (181), UK (99), Sweden (94) and Czech Republic (183). Smoking frequency was increased in anti-Jo-1positive IIM cases, and reached statistical signifi cance in Hungarian IIM (45% Jo-1-positive vs 17% Jo-1-negative, OR 3.94, 95% CI 1.53 to 9.89, p

The idiopathic inflammatory myopathies are systemic autoimmune diseases characterized by chronic inflammation, resulting in the progressive weakness of the proximal muscles. Myositis-specific or myositis-associated autoantibodies can often be found in serum of polymyositis and dermatomyositis patients. This autoantibody presence may play a significant role in patient diagnosis and classification. We present a female polymyositis patient characterized with serious muscle weakness and lung involvement. Anti-Jo1 antibodies were detected in the patient's serum at the time of diagnosis. After 5 years of treatment and surveillance, recent laboratory analysis showed the presence anti-SRP antibody in her serum.

Background The idiopathic inflammatory myopathies (IIM) are systemic autoimmune disease characterised with proximal muscle weakness. The epidemiology of these multifactorial diseases is partially known. These are rare diseases and Immunological or Rheumatological centres or international collaborations are required to carry the majority or all of these patients. Nowadays there are studies for establishing national or international registry of IIM patient. The Myositis study group of Division of Clinical Immunology, Institution of Internal Medicine, University of Debrecen Medical and Health Science Centre have been found in 1975. Most of the Hungarian patients are carried in this department since then. So we have preliminary epidemiological data of Hungarian IIM patients. The database is under continuous development. Objectives The aim of this report is to show our epidemiological findings. Sex and age were analysed in each IIM subgroup. Standardized incidence rates were calculated as new cases/million population/year with 95% confidence interval (CI). Results Between 1999 and 2010 1119 patients were hospitalized due to IIM in Hungary. We selected the data of these years because we have less information about the patients before 1999 (no computerized data before this date). 288 patients were carried in our department. 61,93% suffered from polymyositis (PM), 32,17 % had dermatomyositis (DM), 4,15% had juvenile dermatomyositis (JDM), 0,69% was diagnosed with amyopathic dermatomyositis (ADM) and we found other myositis (OTM) in 1,03% of the patients. The mean age of patients was 47,73 years (ys), respectively 48,46 ys (PM) – 52,19 ys (DM) -9,66 ys (JDM) – 34 ys (ADM) – 32 ys (OTM) in the subgroups. 67,82% of the patients were adult woman (mean age: 50,07 ys), we found 27,68 % of adult man (mean age:49,11 ys) and 4,5% children (9,6 ys). Cancer associated myositis was found in 31 cases (10,72%), the mean age of patients were 53,51 years. The most frequent tumor types were breast and lung cancer but gastrointestinal, reproductive and hematological malignancies were also presented. Overlap syndromes, preciously the association of IIM with other autoimmune diseases have been found in 14 cases (4,84%), the mean age of these patients were 44,64 years. The mainly presented overlap syndrome was Systhemic sclerosis and Sjogren’s syndrome. The average incidence rate was 0.95 cases per 100.000 persons per year. Conclusions Our results represented that the epidemiological data of Hungarian IIM population correlate with the former publicised findings. IIM commonly developed in middle-aged woman. The male/female ratio is 1/2. Dermatomyositis can occur in children and in adults as well, however PM is mainly the disease of adults. These are rare diseases with incidence of 0.1-1 /100.000/year. Our next goal is to develop a National Registry for IIM patients to have more information about epidemiological, clinical and serological characteristics of Hungarian patients. Disclosure of Interest None Declared

Background The most frequent myositis associated autoautoantibodies are directed to Ro52-kd protein (12-26%). Antibodies to the immunodominant epitope (p200) of Ro52 are associated with SLE and Sjogren’s syndrome (SjS) with suggestion for a pathogenic effect in development of neonatal heart block. The role of anti-Ro52 autoantibodies in myositis is not clear. Presence of anti-Ro52 in combination with anti-Jo-1 autoantibodies is associated with interstitial lung disease (ILD). Objectives The aim was to investigate the associations of reactivity to Ro52 epitopes with disease manifestations, autoantibody profile, smoking status and HLA-DRB1 alleles in myositis. In order to test the hypothesis of epitope spreading the association of disease duration with polyreactivity was evaluated. Methods Serum samples from 468 myositis patients were collected in three countries: Czech (n=133); Hungary (n=202), Sweden (n=133). The reactivity with recombinant full length (FL) Ro52 antigen, its four deletion fragments (Ro52-3,-4,-5 and -6) and 7 overlapping synthetic peptides (p136-200) covering the major immunodominant region (Ro52-3) was measured by ELISA. Autoantibodies to Jo-1, PL-7, PL-12, EJ, OJ, SRP, PM-Scl, Ku, Mi-2β, U1-RNP were detected by LIA. 417 patients were genotyped for HLA-DRB1. Smoking status was known in 434 patients. Results 184 patients with dermatomyositis (DM), 250 polymyositis (PM), 11 inclusion body myositis, 20 juvenile DM/PM and three with mixed connective tissue disease were included in the analysis. Median disease duration was 4.7 (0-41.5) years. ILD was present in 132 (29%) of patients. In total 175 (40%) patients were smokers. Reactivity to Ro52-FL was present in 97 (21%) samples; 87 (90%) of these showed specificity to Ro52-3 and anti-p176 dominated (66; 76%) whereas only 44 (51%) had reactivity to the p200 epitope. Anti-Ro52FL positivity was associated with presence of ILD (OR 3.8; p Conclusions Our results confirm the association between anti-Ro52 positivity with ILD, anti-Jo-1 autoantibodies and HLA-DRB1*03 allele in myositis. The dominance of reactivity to epitope p176 in myositis patients differs from dominant p200 in SLE and SjS, but its clinical significance is not clear. There was no effect of smoking or evidence for epitope spreading in anti-Ro52 response. Supported by EU 6.FP integrated project AutoCure LSHB CT-2006-018661 and Grant NT 12438 of IGA MZCR Disclosure of Interest O. Krystufkova Grant/Research support from: EU 6.FP integrated project AutoCure LSHB CT-2006-018661 and Grant NT 12438 of IGA MZCR, V. Dzikatite: None Declared, P. Charles: None Declared, H. Mann: None Declared, L. Ekholm: None Declared, M. Vincze: None Declared, I. Putova: None Declared, N. Kasprikova: None Declared, L. Padyukov: None Declared, K. Danko: None Declared, P. Novota: None Declared, J. Vencovsky: None Declared, M. Wahren-Herlenius: None Declared, I. Lundberg: None Declared

Background Idiopathic inflammatory myopathies (IIM) are frequently accompanied by presence of autoantibodies. A correlation of anti-Jo-1 and anti-SRP antibody levels with disease activity and creatine kinase levels has been described. Recently a new anti-PM1α quantitative ELISA test detecting antibodies to the main epitope of PM-Scl-100 has become available. Objectives To investigate the prevalence of anti-PM1α autoantibodies and association with clinical symptoms, clinical disease activity and serum CK in a multicenter myositis cohort. Methods Two hundred and thirty eight consecutive patients with established IIM (103 DM, 99 PM, 8 JDM, 3 IBM and 25 IIM/CTD-overlap syndrome) were tested for the presence of anti PM-Scl autoantibodies by immunoblot and by immunoprecipitation. Eight additional anti-PM-Scl positive sera were also obtained. Serum levels of anti-PM1α autoantibodies were detected by ELISA kit (Dr. FOOKE Laboratorien GmbH, Neuss, DE). Clinical data were derived from the Euromyositis database (www.euromyositis.eu). Global and organ clinical disease activities were assessed by Myositis Activity Assessment Tool (MYOACT) 10 cm visual analogue scales. Results 19/238 (8%) patients (11 DM, 2 PM and 6 IIM/CTD-overlap syndrome (3 DM + SSc, 2 PM + SSc, 1 PM + SjS)) were positive for anti-PM-Scl in immunoblot, immunoprecipitation and anti-PM1α ELISA. 19 PM-Scl+ were 16/181 women and 3/57 men, average age 50.5 years (22.0 – 74.2) and average disease duration 3.0 years (0 – 11.6 years). Good agreement between detection tests was found. The most common clinical features among anti-PM-Scl positive patients were muscle weakness (96%), interstitial lung disease (77%), Raynaud’s phenomenon (69%) and mechanic’s hands (65%). One patient had a cancer. Anti-PM1α levels correlated with serum CK activity in the DM group (p=0.041, r=0.533) and with constitutional disease activity and seriousness of dysphagia in the PM group (p=0.048, r=0.768 and p=0.007, r=0.927). Furthermore longitudinal change in anti-PM1α levels correlated with HAQ scores in the whole group (p=0.016, r=0.730) and with global disease activity score in DM group (p=0.023, r=0.685), but not with other MYOACT parameters. Conclusions Anti-PM-Scl autoantibodies are detected in 8% of patients with myositis. They are more frequent in those with DM and overlap syndromes. Presence of these autoantibodies is associated with muscle weakness, ILD, Raynaud’s phenomenon and mechanic’s hands. Anti-PM1α levels correlate with serum CK levels in DM and their change is correlated with change of HAQ scores in the whole group and with MYOACT scores in DM group. This suggests that the quantitative measurement of anti-PM-1α may be a useful tool in the assessment of patients with this syndrome. Disclosure of Interest None Declared

Background Immunological changes during pregnancy are in the centre of scientific researches. We already know that immunological pathways may go wrong during pregnancy in autoimmune diseases with some general complications like fetal loss and low birth weight but we have less informations about the outcome of pregnancy in idiopathic inflammatory myopathies, polymyositis (PM) and dermatomyositis (DM). Objectives Our aim was to assess the clinical features, organ complications, functional course, therapeutic response in patients with PM/DM according to the pregnancy status. Methods Medical records were retrospectively revised using a standardised protocol. 23 PM/DM patients with pregnancy were identified in four countries: Hungary (n=9), Czech Republic (n=8); Sweden (n=5) and Poland (n=1). Immunological and serological characteristics were measured by ELISA, immunoblot and immuneprecipitation. Autoantibodies to Jo-1, PL-7, PL-12, EJ, OJ, SRP, PM-Scl, Ku, Mi-2, U1-, SSA, SSB were detected by LIA. We used the Fisher’s exact test for statistical analysis. Results These 23 patients had 33 pregnancies after or at the time of disease onset. 10 of them suffered from PM and 13 had DM or JDM. The most frequent symptoms were ILD, mechanic’s hand, Raynaud phenomenon and arthritis. 19 pregnancies ended without complications giving life to healthy babies. In 13 cases complications, like intra-uterine growth retardation, intra-uterine death, abortion, extra-uterine gravidity occurred. These complications were most frequently seen in patients with PM than DM (p=0,0729). The relative risk of complicated pregnancy in PM was 1,923 (CI 95% 1,041-3,553). This “almost significant” finding could be caused by PM and by anti-Jo1 and anti-phospholipid autoantibodies as well, because we found these antibodies frequently associated to PM in these cases. 13 pregnancies started during the active phase of myositis and the majority of these patients didn’t reach remission after the delivery. The mean weight (2599g in active disease vs. 3342g in remission) and height (48 cm vs. 51 cm) of babies was lower in the active cases and the pregnancy ended earlier than in patients with remission. Most of the affected patients used only glucocorticoid treatment during pregnancy. Conclusions Complications during pregnancy were frequently reported in PM/DM although no myositis specific complications could be found. In patients with PM there was a tendency for a higher risk of fetal problems than in DM. Disclosure of Interest None Declared

Background Specific or associated autoantibodes are present in a majority of patients with idiopathic inflammatory myopathies (IIM). The most frequent myositis associated autoautoantibodies are directed to Ro52-kd protein (present in 12–26%). Antibodies towards the immunodominant epitope (p200) of Ro52 are associated with neonatal heart block (CHB) in SLE and Sjogren9s syndrome (SjS) with the increasing evidence for their pathogenic role. Less is known about possible pathogenic involvement of anti-Ro52 in IIM. Presence of anti-Ro52 in patients with anti-Jo-1 autoantibody is associated with interstitial lung disease (ILD) and suggested decreased survival rate. Objective To investigate possible associations of reactivity to Ro52 epitopes with disease manifestations, autoantibody profile and HLA-DRB1 alleles in myositis. In order to confirm the hypothesis of ongoing epitope spreading with time, the association of disease duration with polyreactivity was evaluated. Methods Serum samples from 468 myositis patients were collected in three countries: Czech (n=133); Hungary (n=202), Sweden (n=133). Antibody specificity against recombinant full length (FL) Ro52 antigen, its four deletion fragments (Ro52-3, -4, -5 and -6) and 7 overlapping synthetic peptides (p136–200) covering the major immunodominant region (Ro52-3) was measured by ELISA. Myositis specific and associated autoantibodies (Jo-1, PL-7, PL-12, EJ, OJ, SRP, PM-Scl, Ku, Mi-2β, U1-RNP) were detected by line-blot immunoassay. 417 patients were genotyped for HLA-DRB1. Results One hundred and eighty-four patients with dermatomyositis (DM), 250 polymyositis (PM), 11 inclusion body myositis, 20 juvenile DM/PM and three with mixed connective tissue disease were included in the analysis. Overlap with other systemic disease (OM) was present in 67 (14%) and ILD in 132 (29%) of patients. Median disease duration was 4.7 (0–41.5) years. Reactivity to Ro52-FL was present in 97 (21%) samples. Of these, 87 (90%) patients showed specificity to Ro52-3. Within these anti-p176 dominated (66; 76%) compared to anti-p200 in 44 (51%) patients. Anti-Ro52FL positivity was associated with presence of ILD (OR 3.8; p Conclusion Our results confirm the association between anti-Ro52 autoantibody positivity with lung involvement, anti-Jo-1 autoantibodies and HLA-DRB1*03 allele in myositis. The predominance of reactivity to epitope p176 in myositis patients is different from that which has been reported in patients with SLE and SjS, but the clinical significance is not clear. There was no evidence of epitope spreading in anti-Ro52 response. Acknowledgement Supported by European Union 6.FP integrated project AutoCure LSHB CT-2006-018661; by MSM 0021620812 from Ministry of Education, Youth and Sports in the Czech Republic and by Grant NT 12438 of IGA Ministry of Health in the Czech Republic.