Polymyositis and dermatomyositis natural history, complications and prognosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sadaf Sharfaei M.D.[2]

Overview

If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].

OR

Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].

OR

Prognosis is generally excellent/good/poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [#]%.

Natural History, Complications, and Prognosis

Natural History

The symptoms of polymyositis and dermatomyositis usually develop very slowly and progressively from proximal muscle weakness to distal muscle weakness.^[1]
Polymyositis and dermatomyositis usually do not affect facial muscles.
Patients might experience myalgia and muscle tenderness in the early stages of polymyositis.
In dermatomyositis, skin manifestations develop and after few months to years myositis will occur.
The symptoms of (disease name) typically develop ___ years after exposure to ___.
If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].

Complications

Common complications of polymyositis and dermatomyositis include:^[2]
- Malignancy
  - Risk of malignancy in dermatomyositis is 5-7 fold more than the general population
- Cardiac complications
  - Arrhythmia
  - Conduction abnormalities
  - Cardiac arrest
  - Congestive heart failure (CHF)
  - Myocarditis
  - Pericarditis
  - Angina
  - Secondary fibrosis
- Pulmonary complications
  - Hypoventilation and respiratory failure
  - Aspiration pneumonia
  - Interstitial lung disease
- Gastrointestinal complications
  - Dysphagia
  - Proximal esophageal skeletal muscle dysfunction

Prognosis

Prognosis is generally poor, and the overall mortality rate of patients with polymyositis and dermatomyositis is approximately 22%.^[3]
Polymyositis and dermatomyositis have a high morbidity rate in which patients would experience insignificant muscular disability after 3 years.
The presence of extramuscular organ involvement, such as lung and cardiac involvement are associated with a particularly poor prognosis among patients with polymyositis and dermatomyositis.
Cardiovascular involvement is associated with the poor prognosis and is one of the most common causes of death in patients with polymyositis and dermatomyositis.
Pulmonary involvement is associated with increased morbidity and mortality in patients with polymyositis and dermatomyositis.
Patient with polymyositis and dermatomyositis at younger age and shorter duration of clinical manifestations prior to therapy initiation have a better prognosis.^[4]

References

↑ Khan, Sabiha; Christopher-Stine, Lisa (2011). "Polymyositis, Dermatomyositis, and Autoimmune Necrotizing Myopathy: Clinical Features". Rheumatic Disease Clinics of North America. 37 (2): 143–158. doi:10.1016/j.rdc.2011.01.001. ISSN 0889-857X.
↑ Barnes BE, Mawr B (January 1976). "Dermatomyositis and malignancy. A review of the literature". Ann. Intern. Med. 84 (1): 68–76. PMID 1106291.
↑ Marie I, Hachulla E, Hatron PY, Hellot MF, Levesque H, Devulder B, Courtois H (October 2001). "Polymyositis and dermatomyositis: short term and longterm outcome, and predictive factors of prognosis". J. Rheumatol. 28 (10): 2230–7. PMID 11669162.
↑ Marie I, Hachulla E, Hatron PY, Hellot MF, Levesque H, Devulder B, Courtois H (October 2001). "Polymyositis and dermatomyositis: short term and longterm outcome, and predictive factors of prognosis". J. Rheumatol. 28 (10): 2230–7. PMID 11669162.

[KhanChristopher-Stine2011-1] Khan, Sabiha; Christopher-Stine, Lisa (2011). "Polymyositis, Dermatomyositis, and Autoimmune Necrotizing Myopathy: Clinical Features". Rheumatic Disease Clinics of North America. 37 (2): 143–158. doi:10.1016/j.rdc.2011.01.001. ISSN 0889-857X.

[pmid1106291-2] Barnes BE, Mawr B (January 1976). "Dermatomyositis and malignancy. A review of the literature". Ann. Intern. Med. 84 (1): 68–76. PMID 1106291.

[pmid11669162-3] Marie I, Hachulla E, Hatron PY, Hellot MF, Levesque H, Devulder B, Courtois H (October 2001). "Polymyositis and dermatomyositis: short term and longterm outcome, and predictive factors of prognosis". J. Rheumatol. 28 (10): 2230–7. PMID 11669162.

[pmid116691622-4] Marie I, Hachulla E, Hatron PY, Hellot MF, Levesque H, Devulder B, Courtois H (October 2001). "Polymyositis and dermatomyositis: short term and longterm outcome, and predictive factors of prognosis". J. Rheumatol. 28 (10): 2230–7. PMID 11669162.

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