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193Rd ENMC International Workshop Pathology Diagnosis of Idiopathic Inflammatory Myopathies 30 November – 2 December 2012, Naarden, the Netherlands

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193Rd ENMC International Workshop Pathology Diagnosis of Idiopathic Inflammatory Myopathies 30 November – 2 December 2012, Naarden, the Netherlands Available online at www.sciencedirect.com Neuromuscular Disorders 23 (2013) 945–951 www.elsevier.com/locate/nmd Workshop report 193rd ENMC International workshop Pathology diagnosis of idiopathic inflammatory myopathies 30 November – 2 December 2012, Naarden, The Netherlands Jan L. De Bleecker a,⇑, Ingrid E. Lundberg b, Marianne de Visser c, for the ENMC Myositis Muscle Biopsy Study Group1 a Department of Neurology, Ghent University Hospital, De Pintelaan 185, 9000 Ghent, Belgium b Rheumatology Unit, Department of Medicine, Karolinska University Hospital in Solna, Karolinska Institutet, SE-171 76 Stockholm, Sweden c Department of Neurology, Academic Medical Centre, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands Received 17 June 2013 1. Introduction inclusion body myositis (IBM) have been the main subsets of the IIM [1–3]. The definition of the PM entity The 193rd ENMC International Workshop brought has been variable between centers, more particularly together 21 researchers from nine countries: Belgium, between rheumatologic and neurological clinicians and Finland, France, Germany, The Netherlands, Spain, neuropathologists involved in the diagnosis and Sweden, United Kingdom, and United States of America. management of these patients. Recent studies have even The group was joined by a patient representative from the cast doubt on the very existence of the PM entity, Dutch working group of myositis patients. The suggesting that most patients diagnosed with PM either participants discussed the idiopathic inflammatory have IBM or an overlap disease between myositis and an myopathies (IIM), that comprise a number of different identifiable connective tissue disorder, often with an entities recognized on clinical, pathological and immunopathological profile more akin to DM [4,5]. immunological/serological grounds. Consensus documents Depending on the presenting symptoms and signs and on clinical and pathological classification criteria have local habits, patients suffering from IIM are cared for by been issued following previous ENMC and MRC rheumatologists or neurologists with special expertise in workshops, but there remains a strong need to revise and neuromuscular diseases. These two groups have based re-classify. The present workshop aimed, by combining their diagnosis and classification on partly different the input from people with diverse background, to draft a criteria. Rheumatologists have long been using the Bohan set of muscle biopsy diagnostic features and criteria that and Peter criteria published in the early seventies, mainly will gain wide acceptance in the clinical and scientific for research purposes [6]. Neurologists have based their communities involved in IIM diagnosis. The workshop diagnosis to a far larger extent on muscle biopsy findings. aimed at identifying crucial variables in muscle biopsies This divergent practice has lead some authors to suggest and how these should be evaluated qualitatively and that “neurologists are from Mars and rheumatologists quantitatively in routine histopathological studies, leading are from Venus” [7]. This different classification has to a consensus classification and scoring system to be hampered the comparison of clinical and basic research employed in diagnostic laboratories reading IIM publications and the set-up of international multicenter diagnostic muscle biopsies. studies including patients with different presentations of a Traditionally, adult dermatomyositis (DM), juvenile probable spectrum of disease manifestations. dermatomyositis (JDM), polymyositis (PM) and sporadic As research in the field has moved on in the past 10 years, it became clear that (1) the Bohan and Peter criteria, by not using strict muscle biopsy criteria, did not ⇑ Corresponding author. Tel.: +32 9 3324544; fax: +32 9 3324971. adequately deal with the rather common entity of IBM, E-mail address: [email protected] (J.L. De Bleecker). and that usage of these criteria led to indiscriminate 1 Members of the ENMC Myositis Muscle Biopsy Study Group are inclusion of probably different subsets of patients in listed at the end of the report. 0960-8966/$ - see front matter Ó 2013 Elsevier B.V. All rights reserved. http://dx.doi.org/10.1016/j.nmd.2013.07.007 946 J.L. De Bleecker et al. / Neuromuscular Disorders 23 (2013) 945–951 clinical trials [8]; (2) strictly pathologically defined PM adherence to pathological criteria (1) caused too many represents a rare entity, (3) many patients have mild patient biopsies to be classified as unspecific IIM and necrotizing myopathy as the sole biopsy manifestation of their exclusion from further basic research studies; (2) their illness; (4) some biopsies lack inflammatory cells but lead to delayed recognition of some entities such as exhibit other changes that indicate a pathology secondary NAM, and (3) probably resulted in publication bias. It to inflammation, e.g., major histocompatibility class I was suggested that this might be avoided by analysis of (MHC-I) expression on muscle fibers; (5) the increased individual muscle biopsy abnormalities (cellular recognition of myositis specific antibodies allows in at infiltrates, vascular changes, muscle fiber abnormalities, least some instances to define certain clinico-pathological etc.) instead of the pattern recognition that is mostly used subsets of IIM patients [9]. Conversely, the antibody in muscle pathology, and that these individual profile may identify a specific disease causing agent, such abnormalities could then be introduced in statistical as in statin induced necrotizing autoimmune myopathy analysis models confronting them with clinical and (NAM) [10]. serological parameters. Although consensus documents on classification criteria Marianne de Visser elaborated on the over-diagnosis of had been issued on previous ENMC [11] and MRC [12] PM. If the ENMC 2004 classification criteria for definite workshops, there remains a need to revise and re-define PM are applied, PM is rare and most IIM patients fall the pathological criteria and eventually confront them into the unspecific myositis group. A third of these with the evolving data from the other fields, e.g., patients developed an identifiable connective tissue serology. By bringing together researchers from different disorder in the following years. Also, retrospective backgrounds, i.e., neurologists, rheumatologists, observations showed that more than half the patients immunologists and pathologists, the workshop aimed to initially diagnosed as typical PM on clinical and combine this input in order to draft a set of diagnostic morphological grounds actually suffered from IBM, features and criteria that will gain wide acceptance across based on the evolving typical clinical picture of IBM, sub-specialties. Because a recent ENMC workshop dealt with or without occurrence of rimmed vacuoles on repeat with IBM, the focus was on the other IIM. State-of- muscle biopsy [4,15,16]. She also stressed that the focal the-art lectures covering recent developments, a nature of the inflammation in the IIM makes it pathological discussion session around the microscope important to choose the biopsy site wisely, for instance and round-table discussions on methodological aspects by MRI guidance. were held. Ingrid Lundberg discussed the International Myositis Classification Criteria Project (IMCCP), a retrospective, 2. Classification of the inflammatory myopathies multidisciplinary and multicenter project started in 2004 with the aim to develop new criteria based on patient Jan De Bleecker opened the workshop by presenting an data. Information has been collected from more than 900 overview of the classical pathological subdivision of patients, and 600 comparators (clinical and inflammatory myopathies and the basic immuno myopathological variables) via a web-based questionnaire. pathological features as they have been outlined in Disease controls, such as metabolic diseases and studies dating from the mid-eighties to just recent. DM is dysferlinopathies, have been included as reliably characterized by a complement-mediated endothe distinguishing IIM from other diseases is a top priority of liopathy. The immune effector mechanism in PM and the project. Further aims include the development IBM is different and is characterized by cell-mediated of classification criteria that separate major subgroups of cytotoxicity with infiltration of MHC-I expressing IIM with high specificity and sensitivity, and to validate nonnecrotic muscle fibers by CD8+ cytotoxic T cells and the reliability of the new classification criteria in a new set macrophages. Recent advances in IIM immunopathology, of patients. Two models have been developed, one which includes knowledge of the pro-inflammatory probability score and one classification tree. Preliminary factors [13] and their upstream regulators [14], have data were presented, indicating superior performance of further revealed the complexity of these autoimmune easy-to-access measurements and symptoms compared to diseases. In addition, the entity of NAM has been existing criteria. Further analysis and plans for external recognized. Unfortunately, many patients do not fit the validation are in progress. subcategories and are reported as non-specific or overlap Werner Stenzel discussed the more rare types of IIM. (1) myositis. Pitfalls such as inflammatory responses in Neuromuscular sarcoidosis may affect the skeletal muscle muscular dystrophy and the expression of MHC-I in producing granulomatous myositis and also the peripheral non-inflammatory myopathies was mentioned. It was nervous
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