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Flashcards in MSK soft tissue pathology Deck (78):
1

What are the indications of muscle disease?

Raised creatinine kinase
Muscle weakness
Muscle atrophy
Fasciculation

2

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

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

3

How can we categorise the two types of muscle pathology?

Muscle disease
Neurogenic disease causing muscle pathology

4

List the different categories of myopathy

Muscular dystrophy
Inflammatory myopathy
Congenital
Secondary
Metabolic

5

Which age group presents with duchenne's muscular dystrophy?

2-4 year olds

6

How is duchenne's muscular dystrophy inherited?

X-linked (so only males)

7

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

20 years old

8

How does duchenne's muscular dystrophy present?

Proximal muscle weakness
Pseudohypertrophied calf muscles
Gower's sign

9

How will creatinine kinase be affected in duchenne's?

Grossly raised

10

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

Dystrophin
Poor cytoskeleton anchorage to the basement membrane and thus muscle tearing

11

How does DMD appear histologically?

Necrosis & regeneration
Chronic inflammation & fibrosis
Hypertrophy

12

What is becker's muscular dystrophy?

A less severe variant of DMD

13

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

Later than DMD

14

How fast does BMD progress?

Slowly

15

What gene is affected in BMD?

Dystrophin (less severe mutation than DMD)

16

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

DM1 - distal muscles affected
DM2 - proximal muscles affected

17

How does myotonic dystrophy present?

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

18

What are the extra muscular features of myotonic dystrophy?

Cataracts
Frontal baldness (males only)
Cardiomyopathy
Lowered intelligence

19

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

Autosomal dominant
C19 & C3

20

Describe the natural history of myotonic dystrophy

Young patients - face and distal limbs affected
Older patients - respiratory muscles affected

21

How does myotonic dystrophy present histologically?

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

22

What are the three main types of inflammatory myopathies?

Infective
Polymyositis
Dermatomyositis

23

What is polymyositis?

A chronic inflammatory disease of the muscles

24

How does polymyositis present?

Progressive muscle weakness
Progressive pain
Tenderness

25

Describe the immunological component of polymyositis

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

26

How does polymyositis present histologically?

Segmental fibre necrosis

27

What is dermatomyositis?

Polymyositis plus skin changes

28

What is dermatomyositis associated with?

Malignancy!!

29

Describe the immunological component of dermatomyositis

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

30

What are neurogenic disorders of the muscle?

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

31

List the categories of neurogenic muscle disorders

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

32

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

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

33

What is motor neurone disease?

Progressive degeneration of anterior horn cells

34

How does motor neurone disease present (generally)?

Muscle atrophy (due to denervation)
Fasciculation
Weakness

35

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

Recessive
Chromosome 5

36

What is spinal muscle atrophy?

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

37

What is myasthenia gravis?

An autoimmune condition against post synaptic acetylcholine receptors

38

How does myasthenia gravis present?

Muscle weakness
Proptosis
Fatigue
Dysphagia

39

Who gets myasthenia gravis?

Women
20-40 y/o

40

What is associated with myasthenia gravis?

Thymoma OR
Thymic hyperplasia

41

How can myasthenia gravis be treated?

Anti-cholinesterase drugs

42

What is rhabdomyolysis?

Skeletal muscle breakdown

43

What biochemical changes does rhabdomyolysis cause?

Raised myoglobin (coca cola urine)
Hyperkalaemia
Muscle necrosis

44

What complications can arise from rhabdomyolysis?

Acute kidney injury --> hypovolaemia
Metabolic acidosis
Disseminated intravascular coagulation

45

Are connective tissue diseases more common in males or females?

Females

46

How can connective tissue diseases be treated?

Anti-inflammatory drugs

47

What is systemic lupus erythmatosus?

Multi-system autoimmune condition

48

Which antibodies are positive in SLE?

ANA (anti nuclear antibodies)

49

Is SLE genetic?

It can play a part in inheritance

50

Which two drugs can cause SLE?

Hydralazine (anti-hypertensive)
Pracainamide (anti-arrhythmic)

51

What are the dermatological manifestations of SLE?

Malar butterfly rash
UV hypersensitivity
Discoid lupus erythematosis

52

How does SLE affect the joints?

Arthralgia

53

How does SLE affect the kidneys?

Glomerulonephritis

54

How does SLE affect the nervous system?

Psychiatric problems
Focal neurological

55

How does SLE affect the CVS?

Pericarditis
Myocarditis
Necrotising vasculitis

56

Can SLE present with lymphadenopathy?

Yes

57

How does SLE affect the lungs?

Pleuritis
Pleural effusion

58

How does SLE affect the haematological system?

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

59

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

Visceral - immune complex mediated (type III)
Haematological - type II

60

What is polyarteritis nodosa?

Inflammation and necrosis of small to medium arteries

61

Which organs are commonly affected by polyarteritis nodosa?

Kidneys
Heart
Liver
GI tract

62

How might polyarteritis nodosa present?

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

63

What can be found on biopsy of polyarteritis nodosa?

Fibrinoid necrosis

64

What antibody is positive in polyarteritis nodosa?

pANCA

65

Who gets polymyalgia rheumatica?

Old people

66

How does polymyalgia rheumatica present?

Pain and stiffness in shoulder and pelvic girdle
NO muscle weakness

67

How is polymyalgia rheumatica treated?

Corticosteroids

68

What is temporal arteritis?

Vasculitic inflammation of the cranial vessels

69

What is the risk with temporal arteritis?

Blindness

70

How is temporal arteritis investigated?

Raised inflammatory markers
Temporal artery biopsy (skip lesions)

71

What is scleroderma?

Excessive collagen deposition (fibrosis) of organs/tissues

72

How does scleroderma affect the skin?

Tight immobile skin
Reduced movement

73

How does scleroderma affect the GI tract?

Fibrosis of muscles (dysphagia, etc)

74

How does scleroderma affect the CVS?

Pericarditis
Myocardial fibrosis --> HF/arrhythmias

75

How does scleroderma affect the lungs?

Interstitial fibrosis
Cor pulmonale

76

How does scleroderma affect the kidneys?

Attacks blood supply --> secondary hypertension --> CKD

77

How does scleroderma affect the MSK?

Polyarteritis
Myositis

78

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

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

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