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Home arrow Environment arrow Inflammatory Disorders of the Nervous System: Pathogenesis, Immunology, and Clinical Management
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Idiopathic Inflammatory Myopathies

Robert N. Schwendimann

Introduction

In 1975, Bohan and Peter described clinical features of polymyositis and dermatomyo- sitis. They stated: “polymyositis is an inflammatory myopathy of unknown cause to which the term dermatomyositis is applied in the presence of the characteristic skin rash” [4]. Today we know that DM is not simply PM with a rash. While they share certain characteristics clinically, they differ greatly in their pathophysiology, histology, and immunology. More recently, sporadic inclusion body myopathy (IBM) and autoimmune necrotizing myopathy (NM) have been added to this group of idiopathic inflammatory myopathies (IIM). IBM and NM also share common characteristics with DM and PM but differ somewhat in their clinical presentations. IBM also differs greatly from the other IIMs in treatment. DM, PM, and NM are extremely important because they are potentially treatable. This review will recount the clinical features of these myopathies along with an approach to diagnosis and treatment. It will also amplify what is known about IBM and autoimmune necrotizing myopathy as far as clinical presentation, diagnostic features, immunological features, treatment, and prognosis.

The clinical feature common to all of these disorders includes weakness, primarily of proximal muscles. Patients with DM, PM, and NM typically complain of difficulty arising from a chair, going up or down stairs, or working with their arms above their heads. Neck weakness and difficulty in swallowing are quite common as well. Usually the extraocular muscles are spared, and respiratory difficulties are uncommon except in severe and/or acute cases. The pattern of weakness is somewhat different in IBM where distal finger and wrist weakness occurs along with proximal weakness in the quadriceps muscles. Early atrophy in these muscles often occurs with IBM. Myalgias may occur in all of these, but pain is more common in NM [2, 5, 6, 8].

R.N. Schwendimann, MD

Department of Neurology, LSU Health Sciences Center, 1501 King Highway, Shreveport, LA 71130, USA e-mail: This email address is being protected from spam bots, you need Javascript enabled to view it

© Springer International Publishing AG 2017

A. Minagar, J.S. Alexander (eds.), Inflammatory Disorders of the Nervous System, Current Clinical Neurology, DOI 10.1007/978-3-319-51220-4_12

 
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