Related Subjects:
|Relapsing Polychondritis
|Reactive Arthritis
|Raynaud's Phenomenon
|Polymyositis
|Dermatomyositis
|Polyarteritis nodosa
|Osteoporosis
|Rheumatoid Arthritis
|Systemic Sclerosis (Scleroderma)
|Rheumatology Autoantibodies
|Overlap Syndrome
|Inclusion Body Myositis
|Inflammatory Myopathies
๐ช Polymyositis = inflamed striated muscle with proximal weakness.
If the skin is involved, it is termed Dermatomyositis (see topic).
โ ๏ธ Both may be associated with underlying malignancy.
๐ About
- Characterised by inflammation and necrosis of skeletal muscle fibres.
- Polymyositis: Muscle inflammation only.
- Dermatomyositis: Muscle inflammation + distinctive skin changes.
๐งฌ Aetiology
- Possible viral trigger.
- Genetic associations: HLA-B8, DR3.
- Anti-Jo-1 antibody (antiโtRNA synthetase) โ linked to interstitial lung disease, arthritis, Raynaudโs.
- Most common age 40โ60; female:male = 3:1.
๐ฉบ Clinical Presentation
- Systemic: fever, malaise, weight loss.
- Progressive proximal muscle weakness (shoulders, hips) โ difficulty climbing stairs, rising from a chair, lifting arms.
- Can involve: hands, feet, face, bulbar muscles, pharynx, upper oesophagus โ dysphagia.
- Respiratory muscle involvement โ risk of respiratory failure.
- Rare: cardiac involvement.
โจ Key distinction: Dermatomyositis shows heliotrope rash, Gottronโs papules, โshawl signโ, and has a stronger cancer association.
๐ฌ Investigations
- FBC: may show anaemia; ESR often normal.
- Muscle enzymes: CK โโ (up to 50-fold), LDH, AST elevated.
- Muscle biopsy: chronic inflammation, fibre necrosis.
- EMG: fibrillation potentials, low-amplitude polyphasic potentials, high-frequency discharges.
- MRI: localises inflamed muscle.
- Autoantibodies: ANA positive (~80%), Anti-Jo-1 positive in ~30% (poor prognosis, lung involvement).
- Screen for malignancy (especially in older patients).
๐ Differential Diagnosis
Related Subjects:
|Inclusion Body Myositis
|Inflammatory Myopathies
|Polymyositis
|Dermatomyositis
๐งพ Comparison of Inflammatory Myopathies
|
|
| Feature |
๐ Polymyositis |
๐ธ Dermatomyositis |
๐ง Inclusion Body Myositis |
| Sex |
โ โฅ โ |
โ โฅ โ |
โ โฅ โ |
| Age |
Usually adults |
Any age (children & adults) |
> 50 years |
| Onset |
Acute / insidious |
Acute / insidious |
Slow, insidious |
| Distribution of Weakness |
Proximal โฅ distal |
Proximal โฅ distal |
Selective โ finger flexors & quadriceps |
| Course |
Often rapid |
Often rapid |
Gradual, progressive |
| Serum CK |
โโ Very high |
โโ Very high |
Normal / mild โ (โค12-fold) |
| EMG |
Myopathic ยฑ neurogenic |
Myopathic ยฑ neurogenic |
Myopathic ยฑ neurogenic |
| Response to Tx |
Good ๐ |
Good ๐ |
Poor ๐ |
| Skin Changes |
No โ |
Yes โ
(heliotrope rash, Gottronโs papules) |
No โ |
| Malignancy Risk |
No โ |
Yes โ
(paraneoplastic association) |
No โ |
| Biopsy |
Intrafascicular CD8+ T cell infiltrates |
Perifascicular atrophy + CD4+/B-cell infiltrates |
Endomysial CD8+ T cells + rimmed vacuoles |
๐ก Clinical Pearls
- ๐ธ Dermatomyositis: Think skin + cancer risk (do a malignancy screen).
- ๐ Polymyositis: Proximal weakness, raised CK, responds well to steroids.
- ๐ง Inclusion Body Myositis: Older males, selective weakness (finger flexors, quadriceps), poor response to therapy.
๐ Management
- Steroids: Prednisolone 0.5โ1 mg/kg ร 1 month, then gradual taper.
CK falls quickly; strength recovery takes weeks.
- Immunosuppressants: azathioprine, methotrexate, ciclosporin for resistant disease.
- Some cases may be self-limiting over time.
Cases โ Polymyositis
- Case 1 โ Classic proximal weakness ๐ช: A 49-year-old woman presents with gradual onset of difficulty climbing stairs and lifting shopping bags. No rash. Exam: symmetric proximal muscle weakness, especially in thighs and shoulders. Bloods: CK 3500 IU/L, aldolase raised, ANA positive. EMG: myopathic pattern. Diagnosis: polymyositis. Managed with high-dose corticosteroids and physiotherapy.
- Case 2 โ Malignancy-associated ๐๏ธ: A 62-year-old man reports 3 months of proximal weakness and dysphagia. No cutaneous signs. Investigations: CK elevated, antiโTIF1-ฮณ antibody positive. CT chest/abdomen/pelvis: bronchogenic carcinoma. Diagnosis: paraneoplastic polymyositis. Treated with immunosuppression and cancer-directed therapy.
- Case 3 โ Interstitial lung disease link ๐ซ: A 40-year-old woman with polymyositis presents with progressive exertional dyspnoea and dry cough. HRCT: interstitial lung disease. Serology: anti-Jo-1 antibody positive. Diagnosis: anti-synthetase syndrome with polymyositis. Managed with corticosteroids, azathioprine, and respiratory follow-up.
Teaching Point ๐ฉบ: Polymyositis is an idiopathic inflammatory myopathy causing proximal muscle weakness without the skin signs of dermatomyositis. Key associations include malignancy and interstitial lung disease. Always check autoantibodies (e.g. anti-Jo-1, anti-SRP, anti-TIF1-ฮณ).