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
The symptoms of polymyositis and dermatomyositis usually develop very slowly and progressively from proximal muscle weakness manifested as difficulty to raise their arms above the shoulders or from chair, or climbing stairs to distal muscle weakness. In dermatomyositis, myositis develops months to years after skin manifestations. Common complications of polymyositis and dermatomyositis include malignancy, cardiac, pulmonary, gastrointestinal, infection, and medication related complications. Prognosis of polymyositis and dermatomyositis varies depends on different studies. Prognosis is generally poor, and the overall mortality rate of patients with polymyositis and dermatomyositis is approximately 22%. Most common cause of death in patients with polymyositis and dermatomyositis include cardiac and pulmonary complications, infections, and cancers. Extramuscular organ involvement, older age of diagnosis, male gender, and non-Caucasian race are associated with a particularly poor prognosis among patients with polymyositis and dermatomyositis. Younger age of diagnosis and quicker therapy initiation are associated with a better prognosis.
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]
- Patients with polymyositis and dermatomyositis usually present with proximal muscle weakness manifested as difficulty to raise their arms above the shoulders or from chair, or climbing stairs. Patients might experience myalgia and muscle tenderness in the early stages of polymyositis.
- Polymyositis and dermatomyositis usually do not affect facial muscles.
- In dermatomyositis, myositis develops months to years after skin manifestations.
Complications
| Complications | Polymyositis | Dermatomyositis |
|---|---|---|
| Malignancy | 5-7 fold more risks than the general population | |
| Cardiac |
| |
| Pulmonary | ||
| Gastrointestinal |
| |
| Medication related | ||
| Infection | ||
| Factors associated with malignancy in patients with myositis | |
|---|---|
| Increased risk of malignancy | Decreased risk of malignancy |
|
|
Prognosis
- Prognosis of polymyositis and dermatomyositis varies depends on different studies.[4]
- Prognosis is generally poor, and the overall mortality rate of patients with polymyositis and dermatomyositis is approximately 22%.[5]
- Five-year survival rates in polymyositis and dermatomyositis have been estimated at more than 80%.
- 10-year survival rates in polymyositis and dermatomyositis have been estimated to be 62%.[6]
- Polymyositis and dermatomyositis have a high morbidity rate in which patients would experience insignificant muscular disability after 3 years.
- Most common cause of death in patients with polymyositis and dermatomyositis include:[6]
- Cardiac complications (22%)
- Pulmonary complications (22%)
- Infections (15%)
- Cancer (11%)
- The presence of following complications and symptoms are associated with a particularly poor prognosis among patients with polymyositis and dermatomyositis:[7]
- Extramuscular organ involvement like:
- Cardiovascular involvement (one of the most common causes of death)
- Pulmonary involvement (increased morbidity and mortality)
- Gastrointestinal involvement
- Cancer
- Older age of diagnosis
- Male gender
- Non-Caucasian race
- Extramuscular organ involvement like:
- The presence of following characteristics are associated with a better prognosis among patients with polymyositis and dermatomyositis:[8]
- Younger age of diagnosis
- Shorter duration of clinical manifestations prior to therapy initiation
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.
- ↑ Tiniakou, Eleni; Mammen, Andrew L. (2015). "Idiopathic Inflammatory Myopathies and Malignancy: a Comprehensive Review". Clinical Reviews in Allergy & Immunology. 52 (1): 20–33. doi:10.1007/s12016-015-8511-x. ISSN 1080-0549.
- ↑ Briani, C.; Doria, A.; Sarzi-Puttini, P.; Dalakas, M.C. (2009). "Update on idiopathic inflammatory myopathies". Autoimmunity. 39 (3): 161–170. doi:10.1080/08916930600622132. ISSN 0891-6934.
- ↑ 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.
- ↑ 6.0 6.1 Findlay, Andrew R.; Goyal, Namita A.; Mozaffar, Tahseen (2015). "An overview of polymyositis and dermatomyositis". Muscle & Nerve. 51 (5): 638–656. doi:10.1002/mus.24566. ISSN 0148-639X.
- ↑ Ascherman, Dana; Hallowell, Robert; Danoff, Sonye (2014). "Pulmonary Manifestations of Polymyositis/Dermatomyositis". Seminars in Respiratory and Critical Care Medicine. 35 (02): 239–248. doi:10.1055/s-0034-1371528. ISSN 1069-3424.
- ↑ 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.