eMedicine Specialties > Emergency Medicine > Allergy & Immunology

Polymyositis: Differential Diagnoses & Workup

Author: Henry Rosenkranz, MD, Assistant Professor, Department of Emergency Medicine, Tufts University, New England Medical Center
Contributor Information and Disclosures

Updated: Aug 25, 2009

Differential Diagnoses

Alcohol and Substance Abuse Evaluation
Myasthenia Gravis
Amyotrophic Lateral Sclerosis
Myopathies
Arthritis, Rheumatoid
Polymyalgia Rheumatica
Cushing Syndrome
Sarcoidosis
Hyperthyroidism, Thyroid Storm, and Graves Disease
Systemic Lupus Erythematosus
Hypokalemia
Trichinosis
Hypophosphatemia

Other Problems to Be Considered

Vasculitis
Progressive systemic sclerosis
Infectious myositis
Muscular dystrophy
Eaton-Lambert syndrome
Drug-induced myopathies - Corticosteroids, statins, zidovudine
Electrolyte disorders
Inherited myopathies

Workup

Laboratory Studies

  • Creatine kinase, aldolase, myoglobin, lactate dehydrogenase, aspartate aminotransferase, and alanine aminotransferase levels may be elevated.
  • In practice, usually only the creatine kinase and aldolase levels are determined. The creatine kinase level is the most sensitive and specific; it usually is 5-50 times above the reference level. A level greater than 100 times the reference level is rare and is a signal of other diagnoses.
  • The erythrocyte sedimentation rate usually is elevated.
  • Myoglobinuria may be present.
  • Positive rheumatoid factor results are found in more than 50% of patients.
  • Positive antinuclear antibody results are found in fewer than 50% of patients.
  • Leukocytosis is present in more than 50% of patients.

Imaging Studies

  • MRI examination
    • MRIs show signal intensity abnormalities of muscle due to inflammation, edema, or scarring.
    • Images may be used to guide muscle biopsy.
    • Many clinicians choose the biopsy site on the basis of findings at electromyography and clinical examination and believe that MRIs are not required.


MRI of thighs showing increased signal in the qua...

MRI of thighs showing increased signal in the quadriceps muscles bilaterally consistent with inflammatory myositis.

MRI of thighs showing increased signal in the qua...

MRI of thighs showing increased signal in the quadriceps muscles bilaterally consistent with inflammatory myositis.

  • Chest radiography - Radiographs may show evidence of pulmonary involvement or associated malignancy.
  • Consider mammography and pelvic ultrasonography in screening for associated malignancy.
  • Testing for associated malignancy is based on age and sex and may also include upper and lower gastrointestinal endoscopy.

Other Tests

  • Electromyographs reveal characteristic myopathic abnormalities.
  • Electrocardiography may reveal arrhythmias or conduction disturbances.

Procedures

  • Inflammatory changes are seen at muscle biopsy (eg, deltoid or quadriceps femoris). Findings occasionally may be normal because of patchy involvement. A biopsy should not be performed on muscle that has undergone electromyography. Diagnostic errors are minimized by combining muscle biopsy results with clinical and laboratory findings.


Hematoxylin and eosin paraffin section shows poly...

Hematoxylin and eosin paraffin section shows polymyositis. Patient had dense endomysial inflammation that contains an abundance of plasma cells, which can be observed in patients with chronic polymyositis. Two necrotic myofibers, characterized by dense eosinophilic staining, are observed. Focal fatty infiltration of the muscle is present in the lower left quadrant of the photomicrograph. Image courtesy of Roberta J. Seidman, MD.

Hematoxylin and eosin paraffin section shows poly...

Hematoxylin and eosin paraffin section shows polymyositis. Patient had dense endomysial inflammation that contains an abundance of plasma cells, which can be observed in patients with chronic polymyositis. Two necrotic myofibers, characterized by dense eosinophilic staining, are observed. Focal fatty infiltration of the muscle is present in the lower left quadrant of the photomicrograph. Image courtesy of Roberta J. Seidman, MD.



Hematoxylin and eosin paraffin section shows poly...

Hematoxylin and eosin paraffin section shows polymyositis. Photomicrograph illustrates attack on a nonnecrotic myofiber by autoaggressive T lymphocytes. On the left, the central myofiber is intact. On the right, it is obliterated by a segmental inflammatory attack. If immunohistochemistry were performed, expected findings would include an admixture of CD8 T lymphocytes and macrophages in the inflammatory process. Image courtesy of Roberta J. Seidman, MD.

Hematoxylin and eosin paraffin section shows poly...

Hematoxylin and eosin paraffin section shows polymyositis. Photomicrograph illustrates attack on a nonnecrotic myofiber by autoaggressive T lymphocytes. On the left, the central myofiber is intact. On the right, it is obliterated by a segmental inflammatory attack. If immunohistochemistry were performed, expected findings would include an admixture of CD8 T lymphocytes and macrophages in the inflammatory process. Image courtesy of Roberta J. Seidman, MD.



Hematoxylin and eosin paraffin shows dermatomyosi...

Hematoxylin and eosin paraffin shows dermatomyositis. In dermatomyositis, inflammation is characteristically perivascular and perimysial. Vessel oriented approximately vertically in the center has a mild perivascular chronic inflammatory infiltrate. The endothelium is plump. The wall is not necrotic. A few lymphocytes in the wall of the vessel are probably in transit from the lumen to the external aspect of the vessel. Some observers may interpret this finding as vasculitis, but it is certainly neither necrotizing vasculitis nor arteritis. Image courtesy of Roberta J. Seidman, MD.

Hematoxylin and eosin paraffin shows dermatomyosi...

Hematoxylin and eosin paraffin shows dermatomyositis. In dermatomyositis, inflammation is characteristically perivascular and perimysial. Vessel oriented approximately vertically in the center has a mild perivascular chronic inflammatory infiltrate. The endothelium is plump. The wall is not necrotic. A few lymphocytes in the wall of the vessel are probably in transit from the lumen to the external aspect of the vessel. Some observers may interpret this finding as vasculitis, but it is certainly neither necrotizing vasculitis nor arteritis. Image courtesy of Roberta J. Seidman, MD.

More on Polymyositis

Overview: Polymyositis
Differential Diagnoses & Workup: Polymyositis
Treatment & Medication: Polymyositis
Follow-up: Polymyositis
Multimedia: Polymyositis
References

References

  1. Hengstman GJ, van den Hoogen FH, van Engelen BG. Treatment of the inflammatory myopathies: update and practical recommendations. Expert Opin Pharmacother. May 2009;10(7):1183-90. [Medline].

  2. Bronner IM, van der Meulen MF, de Visser M, Kalmijn S, van Venrooij WJ, Voskuyl AE. Long-term outcome in polymyositis and dermatomyositis. Ann Rheum Dis. Nov 2006;65(11):1456-61. [Medline].

  3. Amato AA, Barohn RJ. Idiopathic inflammatory myopathies. Neurol Clin. Aug 1997;15(3):615-48. [Medline].

  4. Chahin N, Engel AG. Correlation of muscle biopsy, clinical course, and outcome in PM and sporadic IBM. Neurology. Sep 26 2007;[Medline].

  5. Christopher-Stine L, Plotz PH. Myositis: an update on pathogenesis. Curr Opin Rheumatol. Nov 2004;16(6):700-6. [Medline].

  6. Dalakas MC, Hohlfeld R. Polymyositis and dermatomyositis. Lancet. Sep 20 2003;362(9388):971-82. [Medline].

  7. Dalakas MC, Sivakumar K. The immunopathologic and inflammatory differences between dermatomyositis, polymyositis and sporadic inclusion body myositis. Curr Opin Neurol. Jun 1996;9(3):235-9. [Medline].

  8. Hengstman GJ, van Engelen BG. Polymyositis, invasion of non-necrotic muscle fibres, and the art of repetition. BMJ. Dec 18 2004;329(7480):1464-7. [Medline][Full Text].

  9. Koopman WJ. Polymyositis/dermatomyositis classification criteria. In: Arthritis and Allied Conditions. 1997:17-8.

  10. Mastaglia FL, Ojeda VJ. Inflammatory myopathies: Part 1. Ann Neurol. Mar 1985;17(3):215-27. [Medline].

  11. Nirmalananthan N, Holton JL, Hanna MG. Is it really myositis? A consideration of the differential diagnosis. Curr Opin Rheumatol. Nov 2004;16(6):684-91. [Medline].

  12. Olsen NJ, Park JH. Inflammatory myopathies: issues in diagnosis and management. Arthritis Care Res. Jun 1997;10(3):200-7. [Medline].

Further Reading

Keywords

polymyositis, PM, dermatomyositis, DM, inclusion body myositis, idiopathic inflammatory myopathy, inflammatory muscle disease, immune-mediated muscle inflammationproximal muscle weakness, dysphagia, aspiration, arthralgias, muscle atrophy, heliotrope rash, purple-red edematous periorbital eruption, scaly purple-erythematous papular eruption over knuckles, Gottron sign, conduction defects, arrhythmias, myocarditis, interstitial lung disease, aspiration pneumonia, skeletal muscle disease

Contributor Information and Disclosures

Author

Henry Rosenkranz, MD, Assistant Professor, Department of Emergency Medicine, Tufts University, New England Medical Center
Henry Rosenkranz, MD is a member of the following medical societies: American Academy of Emergency Medicine and American College of Emergency Physicians
Disclosure: Nothing to disclose.

Medical Editor

Michael S Beeson, MD, MBA, FACEP, Professor of Emergency Medicine, Northeastern Ohio Universities College of Medicine; Program Director, Emergency Medicine Residency, Summa Health System
Michael S Beeson, MD, MBA, FACEP is a member of the following medical societies: American College of Emergency Physicians, Council of Emergency Medicine Residency Directors, National Association of EMS Physicians, and Society for Academic Emergency Medicine
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Gino A Farina, MD, Program Director, Associate Professor of Clinical Emergency Medicine, Department of Emergency Medicine, Long Island Jewish Medical Center, Albert Einstein College of Medicine
Gino A Farina, MD is a member of the following medical societies: American Academy of Emergency Medicine, American College of Emergency Physicians, and Society for Academic Emergency Medicine
Disclosure: Nothing to disclose.

CME Editor

John D Halamka, MD, MS, Associate Professor of Medicine, Harvard Medical School, Beth Israel Deaconess Medical Center; Chief Information Officer, CareGroup Healthcare System and Harvard Medical School; Attending Physician, Division of Emergency Medicine, Beth Israel Deaconess Medical Center
John D Halamka, MD, MS is a member of the following medical societies: American College of Emergency Physicians, American Medical Informatics Association, Phi Beta Kappa, and Society for Academic Emergency Medicine
Disclosure: Nothing to disclose.

Chief Editor

Robert E O'Connor, MD, MPH, Professor and Chair, Department of Emergency Medicine, University of Virginia Health System
Robert E O'Connor, MD, MPH is a member of the following medical societies: American Academy of Emergency Medicine, American College of Emergency Physicians, American College of Physician Executives, American Heart Association, American Medical Association, Medical Society of Delaware, National Association of EMS Physicians, Society for Academic Emergency Medicine, and Wilderness Medical Society
Disclosure: Nothing to disclose.

 
 
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