Muscle Diseases Flashcards
What are two signs of polymyalgia rheumatic?
Pain and stiffness.
Where does polymyalgia rheumatica present?
Shoulder and hip girdles.
How long does morning stiffness present for in polymyalgia rheumatic?
Lasts for over an hour.
What condition is strongly associated with polymylagia rheumatic?
Giant Cell Arteritis
What is base line treatment for PMR with no giant cell arteritis?
15mg prednisolone daily
responds very rapidly <24 hours
PMR What is the length of the prednisolone course.
18 months and the dosage is gradually reduced over that period.
Giant cell arteritis pathology
Inflammation of vessel walls with immune infiltration.
Thickening results I luminal narrowing and subsequent distal ischaemia.
Giant Cell Arteritis
Presentation
Unilateral acute temporal headaches. Scalp tenderness Visual disturbances Jaw claudication Prominent non pulsatile temporal artery.
Main risk of GCA
Blindness due to ischaemia of optic nerve.
What artery results in jaw claudication?
Maxillary Artery
GCA Diagnosis
Raised plasma viscosity and CRP
MR/CT angiogram
Temporal artery biopsy is gold standard.
What cells on biopsy are seen to have infiltrated the vessel walls.
Lymphocytes macrophages and multinucleate giant cells
Treatment for GCA with no visual impairment?
40mg of prednisolone
Treatment of GCA with visual impairment?
60mg of prednisolone
Period of prednisolone treatment in GCA?
Over 2 years gradually decreasing.
Treatment started as soon as diagnosis suspected DON’T wait for biopsy result.
Muscle tone is polymyalgia reumatica?
Reduced muscle movement but strength is normal.
In polymyalgia rheumatica what is commonly used as a diagnostic tool?
15mg dose of prednisolone due to rapid response.
Other symptoms related to polymyalgia rheumatic?
Systemic
Fatigue, anorexia, weight loss and fever
What is Polymyositis?
Idiopathic inflammatory myopathy.
2:1 F:M 40-50 years usually
What is dermatopolymyositis?
Clinically very similar to polymyositis but with skin involvement.
Pathogenesis behind polymyositis?
T-cell mediated necrosis of muscle fibres.
What antibodies are associated with polymyositis?
anti-Jo-1
anti-SRP
Presentation of polymyositis
Symmetrical and proximal gradual muscle weakness.
Often described by inability to perform actions which were once easy.
Up to half of patients will have myalgia.
Systemic symptoms of polymyositis
Lung- Interstitial lung disease, generally pulmonary fibrosis
- respiratory muscle weakness
Oesophageal- dysphagia
Cardiac- Myocarditis
Systemic - Fever weight loss, raynauds, inflammatory arthritis.
What is there an increased risk of in dermatomyositis?
Malignancy specifically ovarian breast and bowel.
Who are at most risk of malignancy in dermatomyositis?
Men over 50 years old.
Investigations for polymyositis
MRI- shows oedema and scarring
FBC
Electromyograph- abnormal finding low voltage short duration.
Biopsy- gold standard
- shows varying degrees of inflammation and necrosis
Full blood count and immunology findings in polymyositis?
Raised CRP and plasma viscosity.
Serum creatinine kinase levels increased showing muscle breakdown.
ANA, anti-JO-1 and anti-SRP
Polymyositis treatment
Prednisolone usually 40mg combined with methotrexate azathioprine etc
What other features are typical of dermatomyositis?
Shawl sign - V-shaped rash over chest
Gottrons papules- rash over MCP joints and back of hands.
Heliotrope Rash- Rash around the eyes.
Fibromyalgia
Chronic widespread muscle pain and fatigue. Common in young and middle aged women.
Name some triggers for fibromyalgia
IBS emotional or physical trauma , lights sounds heat etc
Pathogenesis of fibromyalgia
Unknow, but thought to be an abnormality in pain processing and sensitivity due to biochemical metabolic or immunological reasons.
What conditions are often linked to fibromyalgia?
Systemic Lupus Erythematosis
Rheumatoid arthritis
Presentation of fibromyalgia
Persistant and widespread pain >3 months.
Fatigue with disrupted and unrefreshing sleep.
Cognitive impairment ‘fuzzy brain’
Anxiety depression
Are there any investigations in fibromyalgia?
No its based entirely of clinical bases however tests must be conducted to outrule clinically similar conditions.
What conditions can present like fibromyalgia?
Hypothyroidism, rheumatoid arthritis, systemic lupus erythematosus.
Management of fibromyalgia
Education in self management and exercise.
Validating concerns and acknowledgment of patients pain.
Counselling and behavioural therapy.
Analgesia
What analgesics are used in fibromyalgia?
Gabapentin Pregabalin and amitriptyline.
