Myositis - polymyositis + dermatomyositis Flashcards

1
Q

What is myositis?

A

CTDs characterised by inflammation of muscles

Includes polymyositis and dermatomyositis (most common types)

Other types = necrotising autoimmune myopathy and sporadic inclusion body myositis (IBM)

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2
Q

TRUE OR FALSE
Dermatomyositis mainly affects the skin

A

FALSE

It can affect other body parts e.g., joints, oesophagus, lungs and heart

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3
Q

Which viral infections have been implicated in poly- + dermatomyositis?

A

HIV

Human T-cell lymphotropic virus type I (HTLV-I)

Simian retroviruses

Cocksackievirus B

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4
Q

What is the incidence of dermatomyositis?

A

1.4 cases per 100,000

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5
Q

What is the prevalence of dermatomyositis?

A

5.8 cases per 100,000

Higher prevalence amongst older age group

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6
Q

TRUE OR FALSE

Women are more likely to be affected by dermatomyositis than men

A

TRUE

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7
Q

What is the incidence of polymyositis?

A

3.8 cases per 100,000

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8
Q

What is the prevalence of polymyositis?

A

9.7 cases per 100,000

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9
Q

Is the onset of polymyositis slow or fast?

A

Gradual

Onset over weeks or months

Steady progression

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10
Q

In which muscle groups does muscle weakness develop?

A

Proximal muscles

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11
Q

Which actions does proximal myopathy make more difficult?

A

Rising from a chair

Climbing stairs

Lifting objects

Combing hair

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12
Q

Apart from diffuse weakness, what other symptoms may be present in polymyositis?

A

Fatigue

Myalgia

Muscle cramps

Dysphagia - caused by pharyngeal weakness

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13
Q

Which muscle groups are spared in polymyositis? Hence what are patients still able to do?

A

Distal muscles spared

Patients can still do fine motor movements of hand e.g., :
- buttoning a shirt
- writing
- operating a keyboard
- playing the piano

Affected only late in the disease

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14
Q

Is the weakness in polymyositis always at a constant level?

A

No

May vary from week to week or month to month

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15
Q

What proportion of patients with polymyositis have pain?

A

1/3

Hence not usually painful in most patients

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16
Q

Is there a skin rash in polymyositis?

A

No

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17
Q

What would there be no FHx of in polymyositis?

A

Neuromuscular disease

Also no evidence of endocrine disorder or Hx of exposure to possible toxins

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18
Q

What may you see on examination of a patient with polymyositis?

A

Proximal muscle weakness, sparing of distal muscles until disease is well advanced

External ocular muscles unaffected

Facial muscles affected only in severe disease

Weak forced flexion of neck - there could be difficulty holding the neck up

Muscular atrophy with preservation of tendon reflexes, flexor plantar response and normal sensation

Muscles may be tender on palpation with a nodular grainy feel

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19
Q

What investigations would you do for polymyositis and what would you expect to find?

A

Creatine kinase - up to 50 times normal (rarely normal in active disease - good indication of disease activity)

Anti-Jo-1 antibodies - in 20%

Other enzymes = aldolase, serum glutamic-oxaloacetic transaminase (SOGT), serum glutamic-pyruvic transaminase (SGPT) and LDH

EMG + confirmed by muscle biopsy - definitive test for polymyositis

CA 125, CA 19-9 - useful markers for malignancy

Myositis-specific antibody (MSA) and myositis-associated autoantibodies (MMA) - useful for differentiating patients with underlying malignancy

20
Q

If a patient with polymyositis have anti-Jo-1 antibodies, is this a good or bad prognosis?

A

Poor prognosis, with interstitial lung disease which occurs in about 1/3

21
Q

In polymyositis if SGOT is higher than SGPT what type of cause should be suspected?

A

Myogenic cause

22
Q

What are the other DDx for polymyositis?

A

Hereditary neuromuscular diseases

Endocrine disease inc. thyrotoxicosis and Cushing’s disease

Malabsorption syndromes e.g., alcoholism, cancer, vasculitis, granulomatous disease, sarcoidosis or exposure to drugs or toxins that affect the muscles

23
Q

TRUE OR FALSE
Dermatomyositis affects both children and adults

24
Q

What pattern of muscle weakness does dermatomyositis have?

A

Same as polymyositis

25
Which condition can dermatomyositis resemble?
Scleroderma with Raynaud's phenomenon and dysphagia As it also has systemic upset with fever, arthralgia, malaise and weight loss
26
What possible cardiac diseases can occur in dermatomyositis?
AV conduction defections Tachyarrhythmias Dilated cardiomyopathy
27
What possible GI diseases can occur in dermatomyositis?
GI ulcers and infections
28
What possible lung diseases can occur in dermatomyositis?
Interstitial lung diseases in 30-50% Thoracic muscles may be weak
29
Which features of dermatomyositis do children tend to have more?
Non-muscular features esp GI ulcers and infections
30
What % of new diagnosis of dermatomyositis have an underlying malignancy?
25% More strongly associated with malignancy than polymyositis
31
What are the signs/symptoms on the face of dermatomyositis?
Heliotrope rash - bilateral peri-orbital violaceous colouration with associated swelling
32
What are the signs/symptoms in the eyes of dermatomyositis?
Retinopathy - haemorrhages & cotton-wool spots
33
What are the signs/symptoms on the hands of dermatomyositis?
Gottron's papules - roughened erythematous regions on the hands, elbows and knees Loss of cuticles Subcutaneous calcinosis Nailfold redness Raynaud's sign Mechanics hands - hyperkeratotic changes leading to painful rough skin cracking of the fingertips Photosensitivity
34
What are the signs/symptoms in the thorax of dermatomyositis?
Shawl sign - fixed erythema on photosensitive areas of the back, shoulders, chest and neck
35
Name other e signs/symptoms eyes of dermatomyositis
Bibasal pulmonary fibrosis Pyrexia Myocarditis, arrythmias Arthritis
36
What bedside Ix would you do for dermatomyositis?
EMG - abnormal spontaneous and voluntary activity
37
What bloods would you do for dermatomyositis?
LDH = elevated LFTs (ALT, AST) = elevated Creatine kinase = elevated (in the 1,000s) Serum aldolase = elevated
38
What imaging would you do for dermatomyositis?
High-resolution CT - shows pulmonary fibrosis in anti-synthase syndrome
39
What special Ix would you do for dermatomyositis?
Biopsy of skin and muscle = definitive Ix Malignancy screening = tumour markers, CXR, mammogram, USS, CT
40
What antibodies Ix would you do for dermatomyositis?
ANA = 80% sensitive Anti-Jo-1 = anti-synthase syndrome Anti-SRP = polymyositis >>> dermatomyositis Anti-M2 = dermatomyositis (25%) > polymyositis
41
What are the DDx for dermatomyositis?
SLE in which the skin over the phalanges is involved and the skin of the MCP join is spared - reverse is true in dermatomyositis Systemic sclerosis Rheumatoid arthritis Sjogren's syndrome
42
Which other autoimmune diseases can poly- and dermatomyositis be associated with?
Myasthenia gravis Hashimoto's thyroiditis Systemic sclerosis Waldenstrom's macroglobulinaemia
43
Which cancers are particularly increased in dermatomyositis?
Ovarian Lung Breast Pancreatic
44
Which cancers are particularly increased in polymyositis?
Lymphatic and haematopoietic malignancies e.g., : - non-Hodgkin's disease
45
Sources
https://patient.info/doctor/myositis-polymyositis-and-dermatomyositis https://zerotofinals.com/medicine/rheumatology/myositis/