Myositis
Musculoskeletal System Confirmed Autoimmune

Myositis

Also known as: Idiopathic Inflammatory Myopathy (IIM), Inflammatory myositis, Autoimmune myopathy, Polymyositis (PM), Dermatomyositis (DM), Inclusion Body Myositis (IBM), Immune-Mediated Necrotizing Myopathy (IMNM)

Primary organ/tissue: Skeletal muscles; Skin (in dermatomyositis); Lungs (in interstitial lung disease, a common complication)

Overview

Myositis refers to a group of rare autoimmune diseases characterized by chronic inflammation of the muscles. This inflammation leads to muscle weakness, fatigue, and sometimes pain. The main types include polymyositis, dermatomyositis, inclusion body myositis, and immune-mediated necrotizing myopathy. Each type has slightly different symptoms and underlying immune activity, but all involve the immune system mistakenly attacking muscle tissue.

Symptoms

Common features: Progressive muscle weakness (especially in the hips, thighs, shoulders, and neck); Muscle fatigue or pain; Difficulty climbing stairs, lifting arms, or rising from a seated position; Trouble swallowing (dysphagia); Shortness of breath. Additional symptoms by type: Dermatomyositis: red or purple rash, skin thickening; IBM: asymmetrical weakness, particularly in forearms or quadriceps; Polymyositis: symmetrical proximal weakness without skin involvement; IMNM: sudden, severe muscle weakness.

Causes

Caused by the immune system mistakenly attacking healthy muscle tissue. Specific triggers are often unknown but may include viral infections (e.g., HIV, HTLV-1, Coxsackie virus), genetic predisposition, certain medications (e.g., statins in IMNM), cancer (in paraneoplastic dermatomyositis), or environmental exposures.

Diagnosis

Blood tests: Elevated creatine kinase (CK), aldolase, AST/ALT; Autoantibody panel: Myositis-specific and associated antibodies; EMG: Shows muscle irritation; MRI: Identifies inflamed muscles; Muscle biopsy: Confirms inflammation, necrosis, or inclusion bodies; Skin biopsy in cases of dermatomyositis; Cancer screening in older adults or dermatomyositis patients.

Treatment

First-line: Corticosteroids (e.g., prednisone); Immunosuppressive drugs (methotrexate, azathioprine). Other therapies: IVIG; Rituximab or other biologics for refractory cases; Physical and occupational therapy; Sun protection and topical steroids (for dermatomyositis rash). IBM is typically resistant to treatment.

Prognosis

Dermatomyositis and polymyositis: Good prognosis with early treatment; some achieve remission. IMNM: Varies, but often responds to aggressive immunotherapy. IBM: Progressive and often treatment-resistant, though progression is slow. Respiratory and swallowing difficulties can be life-threatening if not managed.

Prevalence

Estimated 5-10 cases per 100,000 people. More common in women and people aged 30-60 (except IBM, which affects older men more often).

Autoantibodies

AutoantibodyNotes
ANA (Antinuclear Antibodies)
Anti-Jo-1 Most common, seen in antisynthetase syndrome
Anti-Mi-2 Specific for dermatomyositis
Anti-SRP Associated with IMNM
Anti-TIF1-gamma Linked to cancer-associated dermatomyositis
Anti-HMGCR Seen in statin-related necrotizing myopathy

Citations

  1. Dalakas MC. Inflammatory Muscle Diseases. N Engl J Med. 2015;372:1734-47.
  2. Tiniakou E, Mammen AL. Idiopathic Inflammatory Myopathies and Autoantibodies. Clin Rev Allergy Immunol. 2017.
  3. Lundberg IE, et al. 2017 European League Against Rheumatism/American College of Rheumatology Classification Criteria for Adult and Juvenile Idiopathic Inflammatory Myopathies. Arthritis Rheumatol. 2017.