Polymyositis

Polymyositis
Classification and external resources
Micrograph of polymyositis. Muscle biopsy. H&E stain.
ICD-10	M33.2
ICD-9	710.4
DiseasesDB	10343
MedlinePlus	000428
eMedicine	med/3441 emerg/474
MeSH	D017285

Polymyositis (PM)("inflammation of many muscles") is a type of chronic inflammation of the muscles (inflammatory myopathy) related to dermatomyositis and inclusion body myositis.

Signs and symptoms

Symptoms include pain, with marked weakness and/or loss of muscle mass in the proximal musculature, particularly in the shoulder and pelvic girdle. The hip extensors are often severely affected, leading to particular difficulty in ascending stairs and rising from a seated position. Thickening of the skin on the fingers and hands (sclerodactyly) is a frequent feature, although this is non-specific and occurs in other autoimmune connective tissue disorders. Dysphagia (difficulty swallowing) and/or other aspects of oesophageal dysmotility occur in as many as 1/3 of patients. Low grade fever and peripheral adenopathy may be present. Foot drop in one or both feet can be a symptom of advanced polymyositis and inclusion body myositis. Polymyositis is also associated with interstitial lung disease.

Polymyositis is linked to an increase in the occurrence of certain cancers (particularly Non-Hodgkin lymphoma, lung and bladder cancers), but the overall association is weaker than in the related condition Dermatomyositis. The latter is linked to a significant increase in the risk of a variety of malignances, including ovarian, lung, and pancreatic cancer.

Polymyositis^[1] tends to become evident in adulthood, presenting with bilateral proximal muscle weakness often noted in the upper legs due to early fatigue while walking. Sometimes the weakness presents itself as an inability to rise from a seated position without help or an inability to raise one's arms above one's head. The weakness is generally progressive, accompanied by lymphocytic inflammation (mainly cytotoxic T lymphocytes).

Polymyositis, like dermatomyositis, strikes females with greater frequency than males. The skin involvement of dermatomyositis is absent in polymyositis.

Another concern is Interstitial lung disease (ILD).

Causes

The cause of polymyositis is unknown and may involve viruses and autoimmune factors. Cancer may trigger polymyositis and dermatomyositis, possibly through an immune reaction against cancer that also attacks a component of muscles.^[2] In rare cases, the cause is known to be infectious, associated with the pathogens that cause Lyme disease, toxoplasmosis, and other infectious agents. Polymyositis usually is considered non-fatal in the absence of ILD.

It is hypothesized that an initial injury causes release of muscle auto antigen, which is subsequently taken up by macrophages and presented to CD4+ TH cells. Activated TH cells synthesize IFN-γ that stimulate further macrophages and further inflammatory mediator release like IL-1 and TNF-α

Another important event in the pathogenesis of Polymyositis is the increased expression of MHC proteins by m/s cells. Auto-Ag is presented in association with MHC-I molecules on the surface of Myocytes and is recognized by CD8 cytotoxic T cells that subsequently initiate m/s destruction.^{[citation needed]}

Diagnosis

Diagnosis is fourfold, including elevation of creatine kinase, history and physical examination, electromyograph (EMG) alteration, and a positive muscle biopsy.

Sporadic inclusion body myositis (sIBM): IBM is often confused with (misdiagnosed as) polymyositis and dermatomyositis that does not respond to treatment is likely IBM. sIBM comes on over months to years; polymyositis comes on over weeks to months. It appears that sIBM and polymyositis share some common features, especially the initial sequence of immune system activation; however, polymyositis does not display the subsequent muscle degeneration and protein abnormalities as seen in IBM. As well, polymyositis tends to respond well to treatments; IBM does not. IBM and polymyositis apparently involve different disease mechanisms than are seen in dermatomyositis.

Laboratory findings

Presence of Anti Jo antibodies in >65% of patients. Elevated serum creatine kinase is characteristic, but not specific to polymyositis.

Anti-signal recognition particle antibodies (anti-SRP antibodies) are associated with polymyositis, but are not very specific for it.^[3] For individuals with polymyositis, the presence of anti-SRP antibodies are associated with more prominent muscle weakness and atrophy.^[3]

Treatment

The first line treatment for polymyositis is corticosteroids. A course of high dose prednisone, tapered according to response will often bring about a dramatic improvement. It is also useful to distinguish from inclusion body myositis. Unresponsive patients may be tried on other immunosuppressive medications. IVIG (intravenous immunoglobulin) has also shown to be a beneficial treatment, as has Acthar Gel, adrenocorticotrophic hormone (ACTH) for injection. Specialized exercise therapy may supplement treatment to enhance quality of life.

In people who fail to respond to corticosteroids, medications to suppress the immune system, such as methotrexate and azathioprine, may be used, as well as certain biological agents such as Enbrel.

Notable individuals with polymyositis

• Dan Christensen painter of abstract art. Died due to heart failure caused by polymyositis.^[4]
• Robert Erickson American composer and teacher who was a leading modernist exponent of "12-tone" composition. Died from the effects of polymyositis.^[5]

See also

• Limb girdle syndrome

References

External links

• The Myositis Association