muscle disease Flashcards
polymyositis causes
statins can cause muscle pain/muscle necrosis
polymyositis pathophysiology
- cell mediated autoimmune disorder in which CD8, cytotoxic lymphocytes and macrophages invade and destroy myofibers expressing MHC-1 antigens
- inflammatory cells found in the endomysium (between and around myofibers)
dermatomyositis cause
statins can cause muscle pain/muscle necrosis
dermatomyositis pathophysiology
- inflammation, vasculitis and perifascicular necrosis
- inflammatory cells (main B cells, with small numbers of CD4 and T cells) that are found around blood vessels, septa between muscles fascicles and in fibroadipose tissue around muscle
dermatomyositis skin manifestations
- Gottron’s sign (scaly, round and pink papules over the knuckles)
- heliotrope rash (lilac around the eyes)
- shawl sign (macular rash over back and shoulders)
polymositis and dermatomyositis presentation
- slow onset of symmetrical, proximal muscle weakness
- mild myalgia
- interstitial lung disease
- dysphagia
- myocarditis
- fever
- weight loss
- Raynaud’s
- polyarthritis
- reduced muscle
- power on confrontational and isotonic testing
polymyositis and dermatomyositis diagnosis
- elevated creatine kinase and inflammatory markers
- anti-jo 1
- electromyography showing increased fibrillations, abnormal motor complexes and complex repetitive discharges
- biopsy showing perivascular inflammation and muscle necrosis
polymyositis and dermatomyositis management
- malignancy screening
- may need IV immunoglobulin
- steroids and DMARDs
what is inclusion body myositis
inflammatory muscle disease that primarily presents with muscle weakness
inclusion body myositis is most common in
men > 50
inclusion body myositis presentation
- weakness weakness tends to be asymmetrical and can affect distal or proximal muscle groups
- biopsy for inclusion bodies
- creatinine kinase elevated but not as high as polymyositis
- respond poorly to treatment
polymyalgia rheumatica is most common in
women > 50
polymyalgia rheumatica presentation
- myalgia and stiffness with onset being over a few weeks
- fatigue, fever, weight loss and depression
- morning stiffness lasting > 1 hour
- distribution mainly over shoulder and pelvic region
- reduction in active but not passive movements
giant cell arteritis presentation
- unilateral headache
- scalp tenderness (sore to brush hair)
- temporomandibular joint claudication
- tongue claudication
- tender, enlarged, non-pulsatile temporal artery
- amaurosis fugax
- diplopia
polymyalgia rheumatica diagnosis
- raised inflammatory markers and creatinine kinase normal
- temporal artery ultrasound
- temporal artery biopsy or MRI