eMedicine Specialties > Neurology > Neuromuscular Diseases

Dermatomyositis/Polymyositis: Follow-up

Author: Sushma Podila, MD, Resident Physician, Department of Neurology, Columbia Presbyterian Medical Center
Coauthor(s): Thomas H Brannagan III, MD, Associate Professor of Clinical Neurology and Director, Peripheral Neuropathy Center, Columbia University, College of Physicians and Surgeons; Co-Director, EMG Laboratory, New York-Presbyterian Hospital, Columbia Campus, New York
Contributor Information and Disclosures

Updated: Aug 28, 2009

Follow-up

Complications

  • The main complications are related to involvement of organs other than the muscles, such as GI ulcerations, melena, hematemesis, or even infarctions affecting long stretches of the bowel, especially in dermatomyositis.
  • A major problem with dermatomyositis can be the subcutaneous calcifications; when they protrude through the skin, they result in ulcerations, infections, and permanent disfiguring scars.
  • In polymyositis, cardiac abnormalities and interstitial lung disease are common complications, especially in patients with anti–Jo-1 antibodies. Some studies have reported rates of these complications as high as 45%.

Prognosis

  • The natural history of polymyositis and dermatomyositis is unknown because patients are now almost always treated with steroids.
    • Patients with interstitial lung disease may have a high mortality rate.
    • A number of patients still do not respond adequately to therapies and remain disabled.
    • Acute fulminating disease seems to be difficult to treat and resistant to therapies.
    • Patients in whom treatment is initiated soon after the onset of the symptoms have the best prognosis.
  • When treatment is unsuccessful, the patient should be reevaluated and the muscle biopsy specimen reexamined.
    • A second biopsy may be considered to ensure that the diagnosis is correct.
    • The disorders most commonly mistaken for polymyositis are inclusion-body myositis and sporadic limb-girdle muscular dystrophy, which is suspected when the disease has a slow onset and progression and when the muscle-biopsy specimen does not show primary inflammatory features.

Patient Education

  • The Myositis Association of America serves as a resource for patients and the medical community.
  • Polymyositis/dermatomyositis at a glance
    • Polymyositis/dermatomyositis is a chronic inflammatory disease of muscle.
    • Muscle weakness is the most common symptom of polymyositis/dermatomyositis.
    • The cause of polymyositis/dermatomyositis is unknown.
    • Diagnosis of polymyositis/dermatomyositis involves physical examination of muscle strength, blood tests for muscle enzymes, electrical tests of muscle and nerves, and confirmation by muscle biopsy.
    • Treatment of polymyositis/dermatomyositis involves high doses of cortisone-related medications, immune suppression, and physical therapy.

Miscellaneous

Medicolegal Pitfalls

  • The following may increase the medicolegal risk associated with the evaluation and management of this disorder:
    • Delay in diagnosis
    • Misdiagnosis
    • Failure to diagnose malignancy in dermatomyositis
    • Failure to inform patient about potential drug toxicity and medication adverse effects

Special Concerns

  • Pregnancy
    • Polymyositis and dermatomyositis can occur in the last trimester of pregnancy or during puerperium; whether pregnancy is responsible for activating the disease is not known.
    • Pregnant women with dermatomyositis and polymyositis have been treated with steroids and have delivered healthy but small babies, though miscarriages and stillborn babies have also been reported.
  • Overlap syndromes
 
Acknowledgments

The authors and editors of eMedicine gratefully acknowledge the contributions of previous authors Aamir Hashmat, MD, and Zaineb Daud, MD, to the development and writing of this article.



More on Dermatomyositis/Polymyositis

Overview: Dermatomyositis/Polymyositis
Differential Diagnoses & Workup: Dermatomyositis/Polymyositis
Treatment & Medication: Dermatomyositis/Polymyositis
Follow-up: Dermatomyositis/Polymyositis
Multimedia: Dermatomyositis/Polymyositis
References
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Further Reading

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Keywords

dermatomyositis, polymyositis, inflammatory myopathies, primary muscle weakness, endomysial inflammation, elevated levels of serum muscle enzymes, myositis-associated antibodies, MAA, myositis-specific antibodies, MSA, muscle diseases, juvenile dermatomyositis, childhood dermatomyositis, overlap syndrome, polymyositis associated with neoplasia, polymyositis associated with connective tissue disorder, dermatomyositis associated with neoplasia, childhood dermatomyositis with necrotizing vasculitis, childhood myositis with necrotizing vasculitis

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Contributor Information and Disclosures

Author

Sushma Podila, MD, Resident Physician, Department of Neurology, Columbia Presbyterian Medical Center
Disclosure: Nothing to disclose.

Coauthor(s)

Thomas H Brannagan III, MD, Associate Professor of Clinical Neurology and Director, Peripheral Neuropathy Center, Columbia University, College of Physicians and Surgeons; Co-Director, EMG Laboratory, New York-Presbyterian Hospital, Columbia Campus, New York
Thomas H Brannagan III, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, and Peripheral Nerve Society
Disclosure: Nothing to disclose.

Medical Editor

Milind J Kothari, DO, Professor and Vice-Chair, Department of Neurology, Pennsylvania State University College of Medicine; Consulting Staff, Department of Neurology, Hershey Medical Center
Milind J Kothari, DO is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, and American Neurological Association
Disclosure: Nothing to disclose.

Pharmacy Editor

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

Managing Editor

Glenn Lopate, MD, Associate Professor, Department of Neurology, Division of Neuromuscular Diseases, Washington University School of Medicine; Chief of Neurology, St Louis ConnectCare, Consulting Staff, Barnes Jewish Hospital
Glenn Lopate, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, and Phi Beta Kappa
Disclosure: Nothing to disclose.

CME Editor

Selim R Benbadis, MD, Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, University of South Florida School of Medicine, Tampa General Hospital
Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association
Disclosure: Nothing to disclose.

Chief Editor

Nicholas Y Lorenzo, MD, Chief Editor, eMedicine Neurology; Consulting Staff, Neurology Specialists and Consultants
Nicholas Y Lorenzo, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Neurology
Disclosure: Nothing to disclose.

 
 
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