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Review
. 2016 May 20:7:64.
doi: 10.3389/fneur.2016.00064. eCollection 2016.

Idiopathic Inflammatory Myopathies: Clinical Approach and Management

Asma Malik  1 , Ghazala Hayat  1 , Junaid S Kalia  2 , Miguel A Guzman  3
Affiliations
Review

Idiopathic Inflammatory Myopathies: Clinical Approach and Management

Asma Malik et al. Front Neurol. .

Abstract

Idiopathic inflammatory myopathies (IIM) are a group of chronic, autoimmune conditions affecting primarily the proximal muscles. The most common types are dermatomyositis (DM), polymyositis (PM), necrotizing autoimmune myopathy (NAM), and sporadic inclusion body myositis (sIBM). Patients typically present with sub-acute to chronic onset of proximal weakness manifested by difficulty with rising from a chair, climbing stairs, lifting objects, and combing hair. They are uniquely identified by their clinical presentation consisting of muscular and extramuscular manifestations. Laboratory investigations, including increased serum creatine kinase (CK) and myositis specific antibodies (MSA) may help in differentiating clinical phenotype and to confirm the diagnosis. However, muscle biopsy remains the gold standard for diagnosis. These disorders are potentially treatable with proper diagnosis and initiation of therapy. Goals of treatment are to eliminate inflammation, restore muscle performance, reduce morbidity, and improve quality of life. This review aims to provide a basic diagnostic approach to patients with suspected IIM, summarize current therapeutic strategies, and provide an insight into future prospective therapies.

Keywords: antiSRP; dermatomyositis; idiopathic inflammatory mypathies; inclusion body myositis; myositis specific antibodies; necrotizing myopathy; polymyositis.

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Figures

Figure 1
Figure 1
Shawl sign in DM.
Figure 2
Figure 2
Heliotrope rash of dermatomyositis.
Figure 3
Figure 3
Gottron’s papules in a case of dermatomyositis.
Figure 4
Figure 4
Gottron’s papules and periungal erythema in DM.
Figure 5
Figure 5
Wasting of the small muscles of the hands and forearm in sIBM.
Figure 6
Figure 6
Perifascicular atrophy (arrowheads) with increased endomysial connective tissue (asterisks) and inflammatory infiltrates (arrows) are characteristics of dermatomyositis (A, H&E, 40×).
Figure 7
Figure 7
Aggregates of B lymphocytes (arrows) positive for CD20 immunohistochemical stain are found in dermatomyositis (B, CD20 immunostatin, 40×).
Figure 8
Figure 8
Muscle biopsy from a patient with polymyositis showing endomysial inflammatory cells (arrows) and variations of fiber size without a specific pattern (C, H&E 40×).
Figure 9
Figure 9
The inflammatory cells (arrows) in polymyositis are mostly T cells that are highlighted by CD3 immunohistochemistry (D, CD3 immunostatin, 40×).
Figure 10
Figure 10
Atrophic and hypertrophic fibers (asterisk) with internal, rimmed vacuoles (arrows) are typical findings in inclusion body myositis.
Figure 11
Figure 11
Rimmed vacuoles (arrow) in sIBM can be highlighted by modified trichrome stain (F, Modified trichrome stain, 40×).
See this image and copyright information in PMC

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