Muscle Diseases Flashcards
What are two endocrine conditions which can cause myopathy?
Hypothyroidism, Cushing’s
What common medication can cause myopathy?
Statins
What causes myositis?
No cause, idiopathic
Are polymyositis and dermatomyositis inflammatory conditions?
Yes
Which sex are polymyositis and dermatomyositis most common in and by how much?
Twice as common in women
What is the age of peak incidence of polymyositis and dermatomyositis?
45-60
What is the difference between polymyositis and dermatomyositis?
The same muscle disease, dermatomyositis causes cutaneous features
What should all patients diagnosed with poly/dermatomyositis be screened for?
Malignancy, these conditions increase risk
Who is dermatomyositis most likely to be a paraneoplastic syndrome in?
Males over 50
What mediates the process of myositis?
Cytotoxic T cells
What cells surround healthy non-necrotic muscle fibres but then eventually invade and destroy them in myositis?
CD8+ T cells and macrophages
What are 4 features of myositis on a biopsy?
- Muscle fibre necrosis - Degeneration - Regeneration - Inflammatory cell infiltrate
What is the main symptom of myositis?
Muscle weakness
Is the muscle weakness in myositis of sudden or gradual onset?
Gradual, worsening over months
Which of the following describes the muscle weakness in myositis? a) Symmetrical or Asymmetrical b) Proximal or Distal
Symmetrical and proximal
Which muscles are generally affected by myositis?
Hip and shoulder girdle
What do the symptoms of myositis relate to?
The functional weakness of the muscles, i.e. difficulty doing particular tasks
Do myositis patients get myalgia?
Mild, in some patients
What is Gottron’s Sign and when is it seen?
Papules over the joints in the hands- dermatomyositis
What are respiratory features of myositis?
ILD/pulmonary fibrosis (5-30% of patients), respiratory muscle weakness
What makes myositis patients more prone to ILD?
Being positive for anti-Jo-1
What GI symptom occurs in a third of myositis patients? What does its presence indicate?
Dysphagia- a poor prognostic sign
What cardiac feature can myositis cause?
Myocarditis
What are some general symptoms of myositis?
Fever, weight loss, Raynaud’s, non-erosive polyarthritis
Which has a higher risk of malignancy, polymyositis or dermatomyositis?
Dermatomyositis (15%) (As opposed to polymyositis at 9%)
What are the cancers which myositis predisposes to?
Ovarian, breast, lung, stomach, oesophageal, bladder, colon
What age/sex is the risk of malignancy in myositis highest?
Males > 45
What % of myositis patients have an increased risk of malignancy? When is this risk highest?
25%- highest in the first 5 years following diagnosis
What past medical history may be of relevance in myositis?
Diabetes, thyroid disease
What drugs may be of relevance in myositis?
Steroids, statins
What is an example of isotonic testing used for myositis?
Sit to stand test- how many times can they do this in 30 seconds
What are you looking for in blood tests for myositis?
- Raised CK (sometimes by 10x) - Raised inflammatory markers - Also check electrolytes, Ca++, PTH, TSH
What is the main antibody which is specific for myositis?
Anti-Jo-1
Auto-antibodies are detected in how many patients with myositis?
60-80%
What other antibody is found in myositis which is sensitive but not specific?
ANA
What is another antibody which is said to be specific for myositis?
Anti-SRP
What is an electromyography?
A fine needle is inserted into 2 areas of muscle and a low electric current is passed through to see if it conducts or not
What is the definitive, gold standard test for myositis?
Muscle biopsy
Is a muscle biopsy the same for poly and dermatomyositis?
No, they have different patterns
MRI can be used for myositis to assess the degree of muscle involvement. What are some things it can show?
- Inflammation - Oedema - Fibrosis - Calcification
What is the main treatment for myositis?
Start with a high dose of prednisone and then reduce the dose. To reduce the amount of steroids, start an immunosuppressant such as methotrexate or azathioprine but make sure the steroid is on until these have time to work.