IDIOPATHIC INFLAMMATORY MYOPATHIES – RECENT DEVELOPMENTS Edited by Jan Tore Gran Contents Preface IX Chapter 1 Idiopathic Inflammatory Myopathies: A Review of Immunopathological Features and Current Models of Pathogenesis 1 Cristina Cappelletti, Lucia Morandi, Marina Mora, Franco Salerno, Paolo Confalonieri, Renato Mantegazza and Pia Bernasconi Chapter 2 Serological Aspects of Myositis 25 Johannes Schulte-Pelkum Chapter 3 Arthrogenic Alphaviruses and Inflammatory Myopathies 43 Lara Herrero and Suresh Mahalingam Chapter 4 The Antisynthetase Syndrome 65 Jan Tore Gran and Øyvind Molberg Chapter 5 Aminoacyl-tRNA Synthetases in Idiopathic Inflammatory Myopathies: An Update on Immunopathogenic Significance, Clinical and Therapeutic Implications 77 Codrina Mihaela Ancuta, Eugen Ancuta and Rodica Marieta Chirieac Chapter 6 Interstitial Lung Disease Associated with Clinically Amyopathic Dermatomyositis 91 Toshinori Takada, Eiichi Suzuki and Ichiei Narita Chapter 7 Myositis and Cancer 111 Sergio Prieto-González, Vladimir Lazo, Ernesto Trallero-Araguás, Albert Selva-O’Callaghan and Josep M Grau Chapter 8 Orbital Myositis 123 Toshinobu Kubota Chapter 9 Myouclear Breakdown in Sporadic Inclusion Body Myositis 143 Satoshi Nakano and Hirofumi Kusaka Chapter 10 Immunoglobulin Treatment in Polymyositis and Dermatomyositis 163 Maria Giovanna Danieli, Lucia Pettinari, Romina Moretti, Francesco Logullo and Armando Gabrielli Chapter 11 Pregnancy in Myositis: Challenges and Pitfalls 197 Codrina Mihaela Ancuta, Eugen Ancuta and Rodica Marieta Chirieac Preface The idiopathic inflammatory myopathies present some of the most challenging clinical problems in medicine. For many years, these myopathies were simply classified into polymyositis, dermatomyositis and myositis associated with other connective tissue diseases. The treatment relied solely on administration of glucocorticosteroids. Fortunately, our understanding of the immunology, pathology, genetics and clinical aspects of the idiopathic inflammatory myopathies has increased enormously in recent years. The discovery of the myositis specific autoantibodies is of particular imporance, linking specific immunological reactions to distinct clinical subsets of myositis. These advances will most likely contribute to improved definitions and classification of the myopathies, and in due time better treatment and care for the patients. The clinicians involved will be equipped with better tools for predicting disease outcome and final prognosis. As new important findings are continously published, reliable and up-to-date reviews are strongly warranted. The emphasis of this book is to provide the reader with recent developments in etiopathogenesis, diagnostics and treatment of the idiopathic inflammatory myopathies. The purpose has not been to present comprehensive descriptions of all myopathic disorders, which can be found in most traditional textbooks. Our goal was to have chapters written by authors with the most expertice. The contributors to this book represent some of the most competent and dedicated workers in the field of myopathies today. I am confident that the book will provide physicians of varied medical specialities with new information, which will be of great value to their research and patient care. Jan Tore Gran Consultant, Professor dr. med. Departement of Rheumatology Oslo University Hospital, Rikshospitalet Sognsvannsveien 200026, Oslo Norway