Peter Criteria Research Articles

FRI0504 Reliability and Validity of Patients Reported Outcomes in Poly/Dermatomyositis: A Pilot Study Using an Italian Version of Myositis Activity Profile (MAP) in A Monocentric Cohort of Patients

Backgroundthe evaluation of disease activity in patients with polymyositis (PM) and dermatomyositis (DM) still represents a challenge. International Myositis Assessment & Clinical Studies Group (IMACS) has performed a consensus on...

AB0612 Efficacy of Subcutaneous Immunoglobulins (SCIG) in Refractory Inflammatory Myopathies

ObjectivesTo evaluate the efficacy of subcutaneous of immunoglobulins (SCIg) in 13 patients with refractory polymyositis (PM) and dermatomyositis (DM).MethodsWe described 13 patients (10 female and 3 male) with PM (5)...

FRI0503 Influence of Treatment on the Outcome of Polymyositis and Dermatomyositis

ObjectivesTo describe disease outcome and the influence of treatment modalities in a cohort of patients with polymyositis (PM) and dermatomyositis (DM) prospectively followed by our Internal Medicine Department, between 1985...

AB0009 Association of MHC Haplotypes with Idiopathic Inflammatory Myopathy in Cohort of Czech Patients

BackgroundIdiopathic inflammatory myopathies (IIM) belong to the group of systemic autoimmune diseases. IIM are characterized clinically by chronic muscle inflammation leading to a dysfunction and/or destruction of muscle cells. Like...

AB0623 Correlating Muscle Biopsy Features with Autoantibodies in Patients with Dermatomyositis and the Jo-1 Antisynthetase Syndrome

BackgroundAutoantibodies in the inflammatory myopathies identify unique subsets of patients with distinctive clinical features and provide useful diagnostic and prognostic information. However the association of individual autoantibodies with specific muscle...

SAT0302 Does MRI Have an Added Value over CK in Assessing Disease Activity in the Idiopathic Inflammatory Myopathies?

BackgroundMRI is the most widely used imaging procedure to assess disease activity of the idiopathic inflammatory Myopathies (IIM). Thigh muscle oedema, detected by T2-weighted and fat-suppressed (short tau inversion recovery,...

Prevalence and clinical characteristics of adult polymyositis and dermatomyositis; data from a large and unselected Norwegian cohort

ObjectivesThe occurrence of polymyositis (PM) and dermatomyositis (DM) in the general population is largely unknown and unbiased data on clinical and laboratory features in PM/DM are missing. Here, we aim...

A58: Demographics, Clinical Features and Therapies of Patients with Juvenile Dermatomyositis Participating in a National Myositis Patient Registry

Background/Purpose:The myositis syndromes are rare systemic autoimmune diseases with poorly understood etiologies. We present the demographics, illness features and treatments of patients with juvenile dermatomyositis (JDM) who enrolled in a newly created national registry.Methods:Using a patient database from The Myositis Association and supplemental advertisements, a national registry of myositis patients was established. Between December 2010 and July 2012 questionnaires were mailed to 8847 myositis patients in the US and Canada. The questionnaire queried demographics, clinical features, environmental exposures, and quality of life. Descriptive statistics and multivariable logistic regression analysis were computed using GraphPad Prysm and SAS.Results:1956 patients (22%) returned the questionnaire and consented to participate; 1806 who met probable or definite Bohan and Peter criteria for myositis were included (708 DM, 483 PM, 466 IBM, 139 JDM, 10 JPM); juvenile patients were diagnosed before age 18 years. Of the 139 JDM patients, the median age at diagnosis was 6.9 years and median disease duration at enrollment was 10.3 years. Most JDM patients were female (78%) and Non‐Hispanic Caucasian (88%), and the remainder were Hispanic (6.5%), Asian (2.9%), multiple races (2.2%), and Black (0.7%). Patients or their parents often completed a graduate degree (23%) or college degree (29%). JDM patients were primarily diagnosed by a pediatric rheumatologist (48%), with adult and pediatric dermatologists (22%), pediatric neurologists and primary care physicians (11% each) diagnosing most of the remaining patients; 67% were under the care of a pediatric rheumatologist. JDM patients frequently had skin rashes as a major manifestation (86%); arthritis (35%) and dysphagia (32%) were also common, whereas lung disease (12%) was less frequent. An additional autoimmune disease was present in 18% of JDM patients, with JIA (8%) and SLE and celiac disease (3% each) the most frequent. There were no recorded associated malignancies. 98% of JDM patients received prednisone therapy. Methotrexate was the most common steroid‐sparing agent (84%), followed by hydroxychloroquine (60%), IV pulse solumedrol (54%), IVIG (48%), cyclosporine (19%), rituximab and anti‐TNFs (10% each). Predictors of which agents were received varied among medications, but included age, year of JDM diagnosis, gender, and region of country. Pulse solumedrol and cyclosporine were more likely to be used in JDM patients with dysphagia, hydroxychloroquine in patients with skin rashes and less likely in those with fevers, and azathioprine was less likely in patients with arthritis. Of medications utilized, 45% of JDM patients reported responding best to IVIG, 39% responded best to prednisone, 33% to anti‐TNFs, and 29% to cyclophosphamide.Conclusion:A nationwide myositis registry has been established and includes a subsample of JDM patients that appears demographically and clinically representative of other US populations. This registry may help identify environmental exposures associated with myositis, elucidate factors associated with quality of life, and serve as an important resource for future clinical investigations.

Clinical Characteristics of Children With Juvenile Dermatomyositis: The Childhood Arthritis and Rheumatology Research Alliance Registry

To investigate aspects of juvenile dermatomyositis (DM), including disease characteristics and treatment, through a national multicenter registry. Subjects meeting the modified Bohan and Peter criteria for definite juvenile DM were analyzed from the cross-sectional Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry between 2010 and 2012 from 55 US pediatric rheumatology centers. Demographics, disease characteristics, diagnostic assessments, and medication exposure data were collected at enrollment. A total of 384 subjects met the criteria for analysis. At enrollment, the median Childhood Myositis Assessment Scale score was 51 (interquartile range [IQR] 46-52), the median Childhood Health Assessment Questionnaire score was 0 (IQR 0-0.5), and the median physician and subject global assessment scores were 1 (IQR 0-2) and 1 (IQR 0-3), respectively, out of a maximum of 10. Of the diagnostic assessments, magnetic resonance imaging was more likely than electromyography or muscle biopsy to show abnormalities. A total of 329 subjects had ≥2 diagnostic studies performed, and >34% of these subjects reported ≥1 negative study. Ninety-five percent had been treated with corticosteroids and 92% with methotrexate, suggesting that these medications were almost universally prescribed for juvenile DM in the US. In 2 years, the ongoing CARRA Registry has collected clinical data on 384 children with juvenile DM and has the potential to become one of the largest juvenile DM cohorts in the world. More research is needed about prognostic factors in juvenile DM, and differences in therapy based on manifestations of disease need to be explored by practitioners. This registry provides the infrastructure needed to advance clinical and translational research and represents a major step toward improving outcomes of children with juvenile DM.

The clinical features, diagnosis and classification of dermatomyositis

Dermatomyositis (DM) is an idiopathic inflammatory myopathy (IIM) characterized by an inflammatory infiltrate primarily affecting the skeletal muscle and skin. Most common and peculiar cutaneous lesions include Gottron's papules, Gottron's sign and heliotrope rash. Different DM subsets have been identified until now encompassing classic DM, amyopathic DM, hypomyopathic DM, post-myopathic DM, and DM sine dermatitis.Patients with DM have a higher incidence rate of malignancy than the normal population. In these patients cancer occurs in about 30% of cases with higher occurrence in men and in elderly people.Bohan and Peter's diagnostic criteria, proposed in 1975, have been widely accepted and used until now. In the last ten years muscle immunopathology, myositis specific autoantibodies testing, and the use of new techniques of muscle imaging such as contrast-enhanced ultrasound or Magnetic Resonance Imaging have been introduced in the diagnostic work-up of patients with DM leading to the development of new diagnostic criteria.

Open-label study on treatment with 20 % subcutaneous IgG administration in polymyositis and dermatomyositis

The objective of this study is to describe the benefit of 20 % subcutaneous infusions of immunoglobulin (SCIg) in patients with dermatomyositis (DM) or polymyositis (PM) after a switch from previous 16 % SCIg treatment. Eight patients with DM or PM, who met the Bohan and Peter's criteria, previously treated with 16 % SCIg, were switched to weekly 20 % SCIg infusions (Hizentra®; CSL Behring) at doses equivalent to their previous subcutaneous treatment. A standardised protocol was used to evaluate patients, disease activity and treatment response. The disease remained stable in three and improved in four other patients, as documented by increased Medical Research Council scores and normal serum CK levels. No relapse of disease occurred. Local reactions were mild and self-limiting. No serious adverse events were reported. The mean duration of infusion per week was significantly lower compared to the mean duration of the 16 % SCIg preparation. A specifically designed questionnaire documented the patients' satisfaction with treatment. The weekly administration of 20 % SCIg is effective in maintaining a quiescent disease in patients with DM or PM with no increased safety concerns. SCIg therapy is particularly attractive for patients because it does not require venous access and hospitalisation and requires less assistance from healthcare provider services. The administration of 20 % SCIg was beneficial and safe in maintaining a quiescent disease and in inducing a complete remission in moderately active disease in patients with DM or PM. The treatment with 20 % SCIg led to the possibility to discontinue and to reduce the use of glucocorticoids and/or the immunosuppressants. Patients reported their satisfaction in terms of contact with health professionals, quality of treatment-related information and administration convenience, with an improved quality of life.

Amyopathic Dermatomyositis with Interstitial Lung Disease: A Rare Type of Dermatomyositis with Accelerated Mortality Rate

Background: It was observed that there have been cases with pathognomonic skin lesions of Dermatomyositis, but without muscle weakness and abnormal skeletal muscle enzymes, hence they did not satisfy the Peter and Bohan criteria, and management became undirected. Until in 1993, Euwer and Sontheimer published the diagnostic criteria for Amyopathic Dermatomyositis, a type of Dermatomyositis, without proximal muscle weakness and elevated skeletal muscle enzymes. Setting: St. Luke’s Medical Center, Quezon City, Philippines. Case: We report here a case of a 59-year old female who was admitted for shortness of breath presenting with hyperpigmented papules over the metacarpophalangeal joints. One week before admission, fever, productive cough, and progressive shortness of breath developed prompting hospital admission. She arrived at the ER tachypneic with oxygen saturation of 55% at room air. She had intercostal retractions and crackles on both lung fields. There were violaceous macular rashes on the metacarpophalangeal, and proximal interphalangeal joints of both hands. Neurological examination was normal. Diagnostics: Chest CT scan revealed diffuse ground glass opacities with interstitial thickening consistent with Interstitial Lung Disease (ILD). The serum Creatine Kinase level and electromyography were normal. Skin biopsy showed perivascular lymphohistiocytic infiltrates, suggestive of dermatomyositis. Conclusion: Dermatomyositis is rare and the Amyopathic type is more uncommon (incidence of 2.08/1,000,000 population). Recognizing it can be a challenge. Amyopathic Dermatomyositis (AMD) patients have 80% survival in less than a year and drops to 60% if ILD develops. Hence, early diagnosis and aggressive management are imperative.

PReS-FINAL-1013: Magnetic resonance imaging as a non-invasive tool to assess muscle edema in juvenile dermatomyositis. Return to normal after treatment

Juvenile Dermatomyositis (JDM) is the most common idiopathic inflammatory myopathy in childhood, a systemic vasculopathy that usually affects skin and skeletal muscle but also can affect gastrointestinal tract and other organs. Diagnosis is based on Bohan and Peter's criteria and the goal of treatment includes control of skin and muscle symptoms and prevent disease complications. Stepwise aggressive treatment decreases JDM activity and improves long-term outcome. Role of magnetic resonance imaging (MRI) as a non invasive tool to asses muscle edema have increased last years with a diagnostic sensitivity being 76%, compared with 64% for creatine kinase and ability to distinguish between active JDM patients and inactive healthy children. Attempt to establish MRI scores have been done whether evaluating thigh muscle groups or whole body MRI.

PReS-FINAL-1014: Role of MHC class I overexpression on muscle biopsy of patients with juvenile dermatomyositis

Juvenile Dermatomyositis (JDM) is the most common idiopathic inflammatory myopathy in childhood, a systemic vasculopathy that usually affects skin and skeletal muscle but also can affect gastrointestinal tract and other organs. Diagnosis is based on Bohan and Peter's criteria and the goals of treatment include control of skin and muscle symptoms and prevention of disease complications. Stepwise aggressive treatment decreases JDM activity and improves long-term outcome. Muscle involvement in JDM can be assessed by electromyography (EMG), magnetic resonance imaging (MRI) and/or muscle biopsy. Muscle biopsy assesses the presence of lymphocytic inflammatory infiltrate, perifascicular atrophy, muscle fibers necrosis and it allows studying the overexpression of major histocompatibility complex (MHC) class I in the sarcolemma and sarcoplasm of the muscle cell. In healthy muscles, there is no MHC class I expression but in inflammatory myopathies there is a distinctive and generalized overexpression, not limited to the affected areas, it appears before the inflammatory infiltrate occurs and it is not modified by immunosuppressive treatment.

Autoantibodies in primary biliary cirrhosis: Antimitochondrial autoantibodies

In the context of clinical evaluations performed on our prospective myositis cohort, we noted a striking association of severe cardiac disease in myositis patients with anti-mitochondrial antibodies. We sought to review all cases of anti-mitochondrial antibody (AMA) associated myositis in our cohort to describe the clinical features of this disease subset.We identified 7 patients with confirmed anti-mitochondrial antibodies who presented as an inflammatory myopathy. A retrospective chart review was completed to assess their clinical presentation, laboratory, imaging, electrophysiologic, and histopathologic features.One patient presented with dermatomyositis and 6 were classified as polymyositis using Bohan and Peter criteria. In all but one patient, a chronic course of muscle involvement was appreciated with an average of 6.5 years of weakness prior to presentation. Muscle atrophy was often noted, as well as atypical findings of scapular winging in 2 of the patients. Muscle biopsies were consistent with immune-mediated necrotizing myopathy in 4 patients, dermatomyositis in 1, polymyositis in 1 and nonspecific or granulomatous myositis in 1 patient. Changes pointing to mitochondrial alterations were seen in 2 of the 7 patients. Cardiac involvement (including myocarditis, atrial and ventricular arrhythmias, and cardiomyopathy), was seen in 5 out of 7 (71%) of the patients, and usually preceded the muscle involvement. Coexisting autoimmune conditions were seen in 3/7of the patients and included primary biliary cirrhosis, autoimmune hepatitis, psoriasis, and Hashimoto’s thyroiditis.Anti-mitochondrial antibodies identify a distinct inflammatory myopathy phenotype that is frequently associated with chronic skeletal muscle disease and severe cardiac involvement. Early recognition of this rare entity as an immune-mediated process is important due to implications for treatment. We propose that anti-mitochondrial antibody status should be determined in patients with a compatible clinical picture.

Clinical features and disease course of patients with juvenile dermatomyositis

To describe the clinical features and course of a cohort of patients with juvenile dermatomyositis (JDM) at a tertiary referral pediatric centre in Australia and examine changes in diagnostic and therapeutic approach over time. Retrospective review of patients diagnosed with JDM at the Royal Children's Hospital, Melbourne, between 1989 and 2010. Fifty-seven patients were identified. The female : male ratio was 2 : 1 and median age at diagnosis was 7.1 years (2.2-15.3). At diagnosis, 95% had weakness, all had typical rash and 68% had nailfold capillary changes. Calcinosis was not present in any patients at diagnosis and occurred in 18% over time. Creatine kinase, lactate dehydrogenase, aspartate aminotransferase, alanine aminotransferase and aldolase levels were abnormal in 65%, 92%, 88%, 58% and 100%, respectively. Magnetic resonance imaging (MRI) was abnormal in 97% of patients, electomyograph (EMG) in 83% and muscle biopsy in all four patients in whom it was performed. MRI was used in 86% (24/28) of patients diagnosed after 2000. Muscle biopsy was used in four and EMG in no patients over the same period. Treatment used throughout the disease course included oral steroids (93%), high-dose pulse intravenous steroids (82%), methotrexate (63%), intravenous immunoglobulin (32%) and cyclosporin (18%). The disease was monophasic in 46.7% (21/45), polyphasic in 17.7% (8/45) and chronic in 35.5% (16/45). Australian patients with JDM have similar characteristics to previously described cohorts. In practice, MRI has replaced the invasive diagnostic tests included in the Bohan and Peter criteria for the diagnosis of JDM. The early use of disease-modifying anti-rheumatic drugs has become the most common treatment approach.

THU0293 Juvenile systemic lupus erythematosus (JSLE) and juvenile dermatomyositis (JDM) patients have a distinct profile of soluble apoptosis molecules

BackgroundConflicting results concerning the role of soluble apoptosis molecules in rheumatic diseases still remains. In adulthood, sFas, sFasL, sBcl-2 and sTRAIL levels in lupus patients have been described as normal,...

AB0511 The analysis of prognostic factors in patients with inflammatory myopathies and amyopathic dermatomiositis complicated with interstitial lung disease

BackgroundBecause interstitial lung disease (ILD) is one of the most critical manifestations and can be lethal in inflammatory myopathies (IM), especially amyopathic dermatomiositis (ADM), it is important to determine prognostic...

THU0192 Myositis in primary sjægren’s syndrome: Data from a multicenter cohort

BackgroundAlthough muscle pain is relatively frequent in primary Sjogren Syndrome (pSS), a frank myositis is rare and reported only in 2.5-11% of patients. The most frequent symptoms are muscular weakness...

AB0813 Increased FASCII thickness in inflammatory myopathies: An ultrasound study

BackgroundInflammatory myopathies (IM) are a multisystem diseases characterized by inflammation of skeletal muscles and skin. The skeletal muscle fascia forms a part of the muscle. In this study we investigated...