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Year 2 > MSK soft tissue pathology > Flashcards

MSK soft tissue pathology Flashcards

1
Q

What are the indications of muscle disease?

A

Raised creatinine kinase
Muscle weakness
Muscle atrophy
Fasciculation

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2
Q

Describe the patterns of serum creatinine kinase level with respect to pathology

A

Raised 200x - muscular dystrophy
Raised 20x - inflammatory myopathy
Decreased 2x - neurogenic

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3
Q

How can we categorise the two types of muscle pathology?

A

Muscle disease

Neurogenic disease causing muscle pathology

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4
Q

List the different categories of myopathy

A 
Muscular dystrophy
Inflammatory myopathy
Congenital 
Secondary
Metabolic
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5
Q

Which age group presents with duchenne’s muscular dystrophy?

A

2-4 year olds

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6
Q

How is duchenne’s muscular dystrophy inherited?

A

X-linked (so only males)

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7
Q

What is the average life expectancy for someone with duchenne’s?

A

20 years old

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8
Q

How does duchenne’s muscular dystrophy present?

A

Proximal muscle weakness
Pseudohypertrophied calf muscles
Gower’s sign

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9
Q

How will creatinine kinase be affected in duchenne’s?

A

Grossly raised

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10
Q

What gene is affected in DMD? What does this result in?

A

Dystrophin

Poor cytoskeleton anchorage to the basement membrane and thus muscle tearing

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11
Q

How does DMD appear histologically?

A

Necrosis & regeneration
Chronic inflammation & fibrosis
Hypertrophy

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12
Q

What is becker’s muscular dystrophy?

A

A less severe variant of DMD

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13
Q

When does becker’s muscular dystrophy (BMD) present?

A

Later than DMD

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14
Q

How fast does BMD progress?

A

Slowly

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15
Q

What gene is affected in BMD?

A

Dystrophin (less severe mutation than DMD)

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16
Q

What are the two types of myotonic dystrophy? How can they be clinically differentiated?

A

DM1 - distal muscles affected

DM2 - proximal muscles affected

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17
Q

How does myotonic dystrophy present?

A

Muscle weakness
Myotonia (inability to relax muscle)
Extra-muscular features

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18
Q

What are the extra muscular features of myotonic dystrophy?

A

Cataracts
Frontal baldness (males only)
Cardiomyopathy
Lowered intelligence

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19
Q

How is myotonic dystrophy inherited? Which chromosome does it affect?

A

Autosomal dominant

C19 & C3

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20
Q

Describe the natural history of myotonic dystrophy

A

Young patients - face and distal limbs affected

Older patients - respiratory muscles affected

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21
Q

How does myotonic dystrophy present histologically?

A

Type 1 (red) muscle cell atrophy
Central nuclei
Necrosis
Fatty replacement

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22
Q

What are the three main types of inflammatory myopathies?

A

Infective
Polymyositis
Dermatomyositis

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23
Q

What is polymyositis?

A

A chronic inflammatory disease of the muscles

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24
Q

How does polymyositis present?

A

Progressive muscle weakness
Progressive pain
Tenderness

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25
Q

Describe the immunological component of polymyositis

A

Cell mediated immune response (lymphocytic and CD8 T cells) to muscle

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26
Q

How does polymyositis present histologically?

A

Segmental fibre necrosis

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27
Q

What is dermatomyositis?

A

Polymyositis plus skin changes

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28
Q

What is dermatomyositis associated with?

A

Malignancy!!

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29
Q

Describe the immunological component of dermatomyositis

A

Immune complex deposition in muscle capillaries (CD4 T cells and B cells)

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30
Q

What are neurogenic disorders of the muscle?

A

Nerve damage resulting in disorders of the muscle following re-innervation

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31
Q

List the categories of neurogenic muscle disorders

A

Motor neurone disease
Spinal muscular atrophy
Peripheral neuropathy
Misc. spinal disorders

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32
Q

How do the muscle fibres appear histologically with respect to neurogenic muscle disorders in adults and children respectively?

A

Adults - small and angulated
Children - small and round
Fibre type grouping and atrophy

33
Q

What is motor neurone disease?

A

Progressive degeneration of anterior horn cells

34
Q

How does motor neurone disease present (generally)?

A

Muscle atrophy (due to denervation)
Fasciculation
Weakness

35
Q

How is spinal muscle atrophy inherited? Which chromosome is affected?

A

Recessive

Chromosome 5

36
Q

What is spinal muscle atrophy?

A

Degeneration of the anterior horn cells within the spinal cord causing denervation of muscle

37
Q

What is myasthenia gravis?

A

An autoimmune condition against post synaptic acetylcholine receptors

38
Q

How does myasthenia gravis present?

A

Muscle weakness
Proptosis
Fatigue
Dysphagia

39
Q

Who gets myasthenia gravis?

A

Women

20-40 y/o

40
Q

What is associated with myasthenia gravis?

A

Thymoma OR

Thymic hyperplasia

41
Q

How can myasthenia gravis be treated?

A

Anti-cholinesterase drugs

42
Q

What is rhabdomyolysis?

A

Skeletal muscle breakdown

43
Q

What biochemical changes does rhabdomyolysis cause?

A

Raised myoglobin (coca cola urine)
Hyperkalaemia
Muscle necrosis

44
Q

What complications can arise from rhabdomyolysis?

A

Acute kidney injury –> hypovolaemia
Metabolic acidosis
Disseminated intravascular coagulation

45
Q

Are connective tissue diseases more common in males or females?

A

Females

46
Q

How can connective tissue diseases be treated?

A

Anti-inflammatory drugs

47
Q

What is systemic lupus erythmatosus?

A

Multi-system autoimmune condition

48
Q

Which antibodies are positive in SLE?

A

ANA (anti nuclear antibodies)

49
Q

Is SLE genetic?

A

It can play a part in inheritance

50
Q

Which two drugs can cause SLE?

A

Hydralazine (anti-hypertensive)

Pracainamide (anti-arrhythmic)

51
Q

What are the dermatological manifestations of SLE?

A

Malar butterfly rash
UV hypersensitivity
Discoid lupus erythematosis

52
Q

How does SLE affect the joints?

A

Arthralgia

53
Q

How does SLE affect the kidneys?

A

Glomerulonephritis

54
Q

How does SLE affect the nervous system?

A

Psychiatric problems

Focal neurological

55
Q

How does SLE affect the CVS?

A

Pericarditis
Myocarditis
Necrotising vasculitis

56
Q

Can SLE present with lymphadenopathy?

A

Yes

57
Q

How does SLE affect the lungs?

A

Pleuritis

Pleural effusion

58
Q

How does SLE affect the haematological system?

A

Anaemia
Leucopenia (low white cells)
Thrombophilia (inc. clotting risk)

59
Q

What two types of hypersensitivity are at play with regard to SLE?

A

Visceral - immune complex mediated (type III)

Haematological - type II

60
Q

What is polyarteritis nodosa?

A

Inflammation and necrosis of small to medium arteries

61
Q

Which organs are commonly affected by polyarteritis nodosa?

A

Kidneys
Heart
Liver
GI tract

62
Q

How might polyarteritis nodosa present?

A 
Secondary hypertension (from kidneys)
Haematuria
Abdominal pain
Melaena
Diarrhoea 
Mononeuritis multiplex
Rash
Cough 
Dysphonea
63
Q

What can be found on biopsy of polyarteritis nodosa?

A

Fibrinoid necrosis

64
Q

What antibody is positive in polyarteritis nodosa?

A

pANCA

65
Q

Who gets polymyalgia rheumatica?

A

Old people

66
Q

How does polymyalgia rheumatica present?

A

Pain and stiffness in shoulder and pelvic girdle

NO muscle weakness

67
Q

How is polymyalgia rheumatica treated?

A

Corticosteroids

68
Q

What is temporal arteritis?

A

Vasculitic inflammation of the cranial vessels

69
Q

What is the risk with temporal arteritis?

A

Blindness

70
Q

How is temporal arteritis investigated?

A

Raised inflammatory markers

Temporal artery biopsy (skip lesions)

71
Q

What is scleroderma?

A

Excessive collagen deposition (fibrosis) of organs/tissues

72
Q

How does scleroderma affect the skin?

A

Tight immobile skin

Reduced movement

73
Q

How does scleroderma affect the GI tract?

A

Fibrosis of muscles (dysphagia, etc)

74
Q

How does scleroderma affect the CVS?

A

Pericarditis

Myocardial fibrosis –> HF/arrhythmias

75
Q

How does scleroderma affect the lungs?

A

Interstitial fibrosis

Cor pulmonale

76
Q

How does scleroderma affect the kidneys?

A

Attacks blood supply –> secondary hypertension –> CKD

77
Q

How does scleroderma affect the MSK?

A

Polyarteritis

Myositis

78
Q

What is the mnemonic used to remember the symptoms of scleroderma?

A 
C alcinosis
R aynaud's
E sophageal dysphagia
S clerodactyly
T elangectasia

Year 2 (72 decks)

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