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Polymyositis

Also Known As: Idiopathic inflammatory myopathy (IIM), Inflammatory muscle disease (subtype), Autoimmune myositis
System: Immune System and Musculoskeletal System
Autoantibodies:
  • Anti-Jo-1 (most common, associated with lung involvement)

  • Anti-SRP, Anti-Mi-2, Anti-PL-7, Anti-PL-12 (less common)

Primary Organ or Body Part Affected:
  • Skeletal muscles (especially proximal muscles)

  • Sometimes lungs (in association with interstitial lung disease)

  • Esophagus (in cases with swallowing difficulty)

Acceptance as Autoimmune: Confirmed
Polymyositis

What is Polymyositis?

Polymyositis is a rare autoimmune muscle disease that causes chronic inflammation and weakness in the skeletal muscles, particularly those closest to the center of the body (like the thighs, hips, shoulders, and upper arms). It typically develops gradually and can make everyday tasks—like climbing stairs or lifting objects—more difficult over time.

It is one of a group of diseases called inflammatory myopathies and usually affects adults, especially those between 30 and 60 years old.

What are the signs and symptoms of Polymyositis?

  • Muscle weakness (especially in hips, thighs, shoulders, upper arms, and neck)

  • Difficulty rising from a chair or climbing stairs

  • Fatigue after walking or standing

  • Trouble swallowing (dysphagia)

  • Muscle aches or tenderness (less common than weakness)

  • Low-grade fever or weight loss (in some cases)

  • Breathing problems if respiratory muscles are affected

What are the causes of Polymyositis?

Polymyositis is autoimmune, meaning the immune system attacks the body’s own muscle tissue.
While the exact cause is unknown, possible contributing factors include:

  • Genetic predisposition

  • Viral infections (e.g., HIV, HTLV-1, Coxsackievirus)

  • Other autoimmune diseases (e.g., lupus, scleroderma, rheumatoid arthritis)

  • Certain medications or toxins (rarely)

Diagnosis

  • Blood tests: Elevated muscle enzymes (CK, aldolase), positive autoantibodies

  • EMG (electromyography): To detect muscle abnormalities

  • Muscle biopsy: Confirms inflammation and damage

  • MRI: Identifies areas of muscle inflammation

  • Autoantibody testing: May include anti-Jo-1 and other myositis-specific antibodies

Treatment

  • Corticosteroids (e.g., prednisone) – first-line therapy

  • Immunosuppressants (e.g., methotrexate, azathioprine)

  • Physical and occupational therapy to maintain strength and function

  • IVIG or biologics (like rituximab) for resistant cases

  • Regular monitoring of lung and heart function if involved

Prognosis

  • Many patients respond well to treatment, especially when diagnosed early

  • May require long-term medication to control inflammation and prevent relapse

  • In some cases, permanent muscle weakness or disability can occur

  • Complications can include lung involvement (interstitial lung disease)

Prevalence

  • Roughly 1 to 8 people per 100,000

  • More common in women

  • Onset typically between 30–60 years of age

Citations

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