Abstract
Myositis is a systemic autoimmune disease frequently affecting the muscle, skin, joints, lungs, and vascular, gastrointestinal, and cardiac organ systems. The evaluation of a patient with a suspected diagnosis of myositis begins with a comprehensive history and physical examination of all organ systems as well as the consideration of other possible diagnoses. Demographics as well as the onset, distribution, pattern, and progression of muscle symptoms can provide important diagnostic clues. Laboratory, imaging, and electromyography studies will narrow the differential diagnosis, but in most cases, a muscle biopsy is necessary to confirm the diagnosis. The need for a muscle biopsy may be unnecessary in classic presentations of the anti-synthetase syndrome and in patients with clinically amyopathic dermatomyositis, where skin biopsy may be required. Polymyositis (PM) is often misdiagnosed or overdiagnosed as many other muscular disorders including inclusion body myositis, muscular dystrophies, and metabolic myopathies can mimic PM. In this chapter, we discuss the art and science of making a diagnosis of myositis from the history to a confirmatory muscle biopsy, as well as important pitfalls in evaluating myositis.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Similar content being viewed by others
References
Dalakas MC. Toxic and drug-induced myopathies. J Neurol Neurosurg Psychiatry. 2009;80:832–8.
Bohan A, Peter JB. Polymyositis and dermatomyositis. N Engl J Med. 1975;292:344–7.
Alexanderson H, Lundberg IE. The role of exercise in the rehabilitation of idiopathic inflammatory myopathies. Curr Opin Rheumatol. 2005;17:164–71.
Needham M, Mastaglia FL. Inclusion body myositis: current pathogenetic concepts and diagnostic and therapeutic approaches. Lancet Neurol. 2007;6:620–31.
Dietz F, Longeman JA, Sahgal V, et al. Cricopharyngeal muscle dysfunction in the differential diagnosis of dysphagia in polymyositis. Arthritis Rheum. 1980;23:491–5.
Rider LG, Koziol D, Giannini EH, Jain MS, Smith MR, Whitney-Mahoney K, Feldman BM, Wright SJ, Lindsley CB, Pachman LM, Villalba ML, Lovell DJ, Bowyer SL, Plotz PH, Miller FW, Hicks JE. Validation of manual muscle testing and a subset of eight muscles for adult and juvenile idiopathic inflammatory myopathies. Arthritis Care Res (Hoboken). 2010;62(4):465–72. https://doi.org/10.1002/acr.20035. PubMed PMID: 20391500; PubMed Central PMCID: PMC2924143.
Volochayev R, Csako G, Wesley R, Rider LG, Miller FW. Laboratory test abnormalities are common in polymyositis and dermatomyositis and differ among Clinical and Demographic Groups. Open Rheumatol J. 2012;6:54–63.
Mathur T, Manadan AM, Thiagarajan S, Hota B, Block JA. Serum transaminases are frequently elevated at time of diagnosis of idiopathic inflammatory myopathy and normalize with creatine kinase. J Clin Rheumatol Pract Rep Rheum Musculoskelet Dis. 2014;20:130–2.
Hoshino K, Muro Y, Sugiura K, Tomita Y, Nakashima R, Mimori T. Anti-MDA5 and anti-TIF1-gamma antibodies have clinical significance for patients with dermatomyositis. Rheumatol Oxf. 2010;49:1726–33.
Benveniste O, Guiguet M, Freebody J, Dubourg O, Squier W, Maisonobe T, et al. Long-term observational study of sporadic inclusion body myositis. Brain. 2011;134:3176–84.
Moghadam-Kia S, Oddis CV, Sato S, Kuwana M, Aggarwal R. Antimelanoma differentiation-associated gene 5 antibody: expanding the clinical Spectrum in North American patients with dermatomyositis. J Rheumatol. 2017;44(3):319–25. https://doi.org/10.3899/jrheum.160682. Epub 2017 Jan 15.
Aggarwal R, Oddis CV, Goudeau D, Fertig N, Metes I, Stephens C, Qi Z, Koontz D, Levesque MC. Anti-signal recognition particle autoantibody ELISA validation and clinical associations. Rheumatology (Oxford). 2015;54(7):1194–9. https://doi.org/10.1093/rheumatology/keu436. Epub 2014 Dec 17. PubMed PMID: 25524922; PubMed Central PMCID: PMC4473763.
Aggarwal R, Cassidy E, Fertig N, Koontz DC, Lucas M, Ascherman DP, Oddis CV. Patients with non-Jo-1 anti-tRNA-synthetase autoantibodies have worse survival than Jo-1 positive patients. Ann Rheum Dis. 2014;73(1):227–32. https://doi.org/10.1136/annrheumdis-2012-201800. Epub 2013 Feb 19. PubMed PMID: 23422076; PubMedCentral PMCID: PMC4031026.
Aggarwal R, Dhillon N, Fertig N, Koontz D, Qi Z, Oddis CV. A negative antinuclear antibody does not indicate autoantibody negativity in myositis: role of anticytoplasmic antibody as a screening test for antisynthetase syndrome. J Rheumatol. 2017;44(2):223–9. https://doi.org/10.3899/jrheum.160618. Epub 2016 Dec 1.
Day J, Patel S, Limaye V. The role of magnetic resonance imaging techniques in evaluation and management of the idiopathic inflammatory myopathies. Semin Arthritis Rheum. 2017;46(5):642–9.
Rider LG, Aggarwal R, Machado PM, Hogrel JY, Reed AM, Christopher-Stine L, Ruperto N. Update on outcome assessment in myositis. Nat Rev Rheumatol. 2018;14(5):303–18. https://doi.org/10.1038/nrrheum.2018.33. Epub 2018 Apr 12. Review. PubMed.
Daube JR, Rubin DI. Needle electromyography. Muscle Nerve. 2009;39:244–70. https://doi.org/10.1002/mus.21180.
Pestronk A. Acquired immune and inflammatory myopathies: pathologic classification. Curr Opin Rheumatol. 2011;23(6):595–604. https://doi.org/10.1097/BOR.0b013e32834bab42.
Shaibani A, Jabari D, Jabbour M, Arif C, Lee M, Rahbar MH. Diagnostic outcome on muscle biopsy. Muscle Nerve. 2015;51:662–8. https://doi.org/10.1002/mus.24447.
van de Vlekkert J, Maas M, Hoogendijk J, et al. Combining MRI and muscle biopsy improves diagnostic accuracy in subacute-onset idiopathic inflammatory myopathy. Muscle Nerve. 2015;51:253–8.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2020 Springer Nature Switzerland AG
About this chapter
Cite this chapter
Aggarwal, R., Oddis, C.V. (2020). Evaluating the Patient with Suspected Myositis. In: Aggarwal, R., Oddis, C. (eds) Managing Myositis. Springer, Cham. https://doi.org/10.1007/978-3-030-15820-0_3
Download citation
DOI: https://doi.org/10.1007/978-3-030-15820-0_3
Published:
Publisher Name: Springer, Cham
Print ISBN: 978-3-030-15819-4
Online ISBN: 978-3-030-15820-0
eBook Packages: MedicineMedicine (R0)