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
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.
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
- Malignancy
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.