Soft Tissue Pathology Flashcards Preview

Musculoskeletal > Soft Tissue Pathology > Flashcards

Flashcards in Soft Tissue Pathology Deck (100):
1

What is the function of the perimysium?

Surrounds bundles of muscle fibres

2

Function of the endomysium?

Surrounds individual fibres

3

Function of the epimysium?

Surrounds the entire muscle

4

Ragged red fibres are seen in muscle when?

Mitochondrial disease, metabolic disease, normal features in small numbers at the extremes of age

5

Tubular aggregates are seen in muscle when?

Tubular aggregate myopathy, periodic paralysis, alcoholic myopathy, incidental finding

6

Types of skeletal muscle fibre? (3)

Red (Type 1, slow twitch, lots of mitochondria & myoglobin, good for marathon runners, able to regenerate ATP v quickly)
White (Fast twitch, Type 2A; fast oxidative glycolytic, type 2b; fast glycolytic)
Intermediate

7

Muscle Biopsy Indications

Evidence of muscle disease
-Weakness
-Muscle symptoms (atrophy, fasciculation)
-Elevate creatine kinase (CK)
Presence of neuropathy (+ nerve biopsy)
Presence of vascular disorder (vasculitis)

8

Creatine Kinase High, Intermediate and Low levels

High: (e.g. Dystrophies) 200-300 times of normal.
Intermediate: (e.g. Inflammatory myopathy) 20-30 times of normal.
Low: (e.g. Neurogenic disorder) 2-5 times of normal

9

What is electron microscopy used for?

To examine the ultra-structure of the muscle

10

Duchenne (DMD) method of inheritance and where found?

X-linked recessive, pelvic girdle

11

Becker (BMD) method of inheritance and where found?

X-linked recessive, pelvic girdle

12

Limb girdle (LGMD) method of inheritance and where found?

Autosomal recessive, pelvic girdle

13

Fascioscapulohumeral method of inheritance and where found?

Dominant, face, shoulder girdle and arm

14

Scapulohumeral method of inheritance and where found?

Autosomal recessive, shoulder girdle and arm

15

Oculopharyngeal method of inheritance and where found?

Dominant, external ocular and pharynx

16

Myotonic dystrophy method of inheritance and where found?

Dominant, face, respiratory and arms

17

Signs of Duchennes?

Proximal weakness, pseudohypertrophy of calves
RAISED CK

18

DMD pathogenesis

-Mutations in dystrophin gene on long arm chromosome X
-Alterations in anchorage of actin cytoskeleton to basement membrane
-Fibres liable to tearing
-Uncontrolled Ca2+ entry into cells

19

Variant of DMD?

BMD (Becker)

20

Are muscular dystrophies inherited?

Yes

21

Which inflammatory marker will be raised in Duchennes?

CK!!!

22

Most common types of Myotonic dystrophy?

DM1 and DM2

23

Non-muscle features of myotonic dystrophy?

Frontal baldness (in men), low intelligence, cataracts (clouding of the lens), cariomyopathies

24

Atrophy of which type of fibres in myotonic dystrophy?

Atrophy of type 1 fibres in myotonic dystrophy

25

Myotonic dystrophy histological features?

Atrophy of type 1 fibres
Ring fibres
Central nuclei
Fibrofatty replacement
Fibre necrosis

26

In which condition might you see ring fibres?

Myotonic dystrophy

27

Primary inflammation of muscle inflammatory myopathies

Infective agents
Polymyositis
Dermatopolymyositis

28

Most common form of muscular dystrophy?

Myotonic dystrophy
-Characterised by progressive muscle wasting and weakness

29

At what age do the features of myotonic dystrophy tend to develop? (bearing in mind they can actually develop at any age)

20's-30's

30

Where does DM1 affect?

Distal

31

Where does DM2 affect?

Proximal

32

Features of Polymyositis?

Progressive muscular weakness, PAIN and TENDERNESS :(

33

Genes involved in myotonic dystroophy?

Ch19 and Ch3

34

Polymyositis histological findings?

Endomysial lymphocytic infiltrate, invasion of muscle by CD8+ T lymphocytes
Segmental fibre necrosis

35

What is dermatositis?

Polymyositis and skin changes

36

What does dermatomysitis share a 10% link with?

Malignancy

37

Skin features of dermatmyositis?

Upper body erythema
Eyelid swelling with purple discolouration

38

Which condition would you find immune complex and complement deposition within & around capillaries within muscle?

Polymyositis
You would also find perifasicular muscle fibre injury
and B-lymphocytes and CD4 + T cells > cf polymyositis

39

Neurogenic disorders of muscle (histological findings)

-Small, angulated muscle fibres (adults)
-Small, round muscle fibres (infants)
-Target fibres
-Fibre type grouping
-Grouped atrophy

40

Motor Neurone Disease

Progressive degeneration of anterior horn cells
Denervation atrophy, fasciculation and weakness

41

Which cells progressively degenerate in motor neurone disease?

Anterior horn cells

42

Method of Spinal Muscular Atrophy Inheritance?

Autosomal recessive

43

What gene is affected in spinal muscular atrophy?

Ch5

44

How many types of muscular atrophy are there?

4

45

Age and gender usually affected by myasthenia gravis?

Women aged 20-40

46

Which thymus conditions are linked with myasthenia gravis?

25% have thymoma
Others have thymic hyperplasia

47

Myasthenia gravis symptoms?

Fatigue, muscle weakness, proptosis and DYSPHAGIA

48

Rhabdomyolysis histological findings

Breakdown of skeletal muscle:
-Myoglobinuria
-Hyperkalaemia
-Necrosis & shock

49

What might you test the urine for in rhabdomyolysis?

Myoglobin
(myoglobulinuria)

50

Outcome of rhabdomyolysis?

Acute Renal Failure
Hypovolaemia & hyperkalaemia
Metabolic acidosis
Disseminated intravascular coagulation

51

Which condition might present with acute kidney failure and disseminated intravascular co-agulation?

Rhabdomyolysis (acute renal failure so u would probs see hypovolaemia and hyperkalaemia aswell)

52

What kind of drugs do connective tissue disorders usually respond to?

Anti-inflammatory

53

What anti-bodies do you see in SLE?

Anti-nuclear! omgeeee

54

What drugs can induce SLE?

Hydralazine and pracainamide

55

Lol, every single SLE symptom

Skin –“butterfly” rash (sun exposed areas), discoid lupus erythematosus (DLE)
Joints – arthralgia
Kidneys – glomerulonephritis
CNS – psychiatric symptoms, focal neurological symptoms
CVS – pericarditis, myocarditis, necrotising vasculitis
Lymphoreticular – lymphadenopathy & splenomegaly
Lungs – pleuritis, pleural effusions
Haematological – anaemia, leucopenia, thrombophilia

56

Hypersensitivity in SLE

Visceral lesions – mediated by Type III hypersensitivity
Haematological effects – mediated by Type II hypersensitivity

57

What does PAN stand for?

Polyarteritis nodosa

58

What is PAN?

Inflammation and fibrinoid necrosis of small/medium arteries

59

Target organs for PAN?

Major - kidneys, heart, liver, GI tract
Minor – skin, joints, muscles, nerves, lungs

60

Which infection is PAN often associated with?

Streptococcal infection

61

Diagnosis of PAN

Biopsy- FIBRINOID NECROSIS OF VESSELS

62

Which condition is characterised by fibrinoid necrosis of vessels (found from bioopsy?)

PAN! Polyarteritis nodosa

63

Which antibody would you find in PAN?

pANCA (perinuclear antineutrophil cytoplasmic autoantibody)

64

Clinical Features of PAN?

Non-specific +/- organ specific features such as hypertension, haematuria, abdominal pain, melaena, diarrhoea, mononeuritis multiplex, rash, cough, dyspnoea

65

Clinical features of polymyalgia rheumatica?

Pain, stiffness in shoulder & pelvic girdles
No muscle weakness
Responds to corticosteroids

66

Where is the pain in polymyalgia rheumatica?

Shoulders and pelvic girdle

67

Who is affected by polymyalgia rheumatica?

The elderly!

68

What kind of medication does polymyalgia rheumatica respond to?

Corticosteroids

69

Another name for Giant Cell Arteritis?

Temporal arteritis

70

Diagnosis of temporal arteritis?

raised ESR
Temporal artery biopsy; inflammation,+/- giant cells, fragmentation of internal elastic lamina

71

What is scleroderma?

Excessive fibrosis of organs and tissues (excessive collagen production)

72

CREST syndrome

-Calcinosis
-Raynaud’s
-Oesophageal dysfunction
-Sclerodactyly
-Telangectasia

73

Death due to Scleroderma

-Renal failure secondary to malignant hypertension
-Severe respiratory compromise
-Cor pulmonale
-Cardiac failure or arrhythmias secondary to myocardial fibrosis

74

Which condition is associated with CREST?

Scleroderma

75

Epstein-Barr virus could predispose to which connective tissue disease?

SLE

76

Silica dust (found in cleaning powders, cigarette smoke and cement) may increase risk of developing what?

Silica dust (found in cleaning powders, cigarette smoke and cement) may increase risk of developing SLE

77

Aetiology of SLE

Environmental factors:
Viruses eg Epstein-Barr Virus
UV light may stimulate skin cells to secrete cytokines stimulating B-cells
Silica dust (found in cleaning powders, cigarette smoke and cement) may increase risk of developing SLE

78

In SLE, what is the significance of nuclear material release from necrotic cells?

The nuclear material released from necrotic cells may act autoantigens
Autoimmunity possibly results by the extended exposure to nuclear and intracellular autoantigens

79

Possible source of autoantigens in SLE?

Nuclear material released from necrotic cells may act as autoantigens
Autoimmunity possibly results by the extended exposure to nuclear and intracellular autoantigens

80

Coombes test

Coombes test involves taking a blood sample and checking it for the presence of antibodies which may destroy red blood cells
-Do not count in the presence of haemolytic anemia

81

When would you not count coombes test?

In the presence of haemolytic anemia

82

Requirements for classification of lupus?

At least 4 clinical and 1 laboratory finding
Or biopsy proven lupus nephritis with positive ANA or anti-DNA

83

Serositis in SLE?

Pericarditis, pleuritis, peritonitis

84

Levels of C3, C4 and CH50 in active SLE?

Levels of C3, C4 and CH50 will decrease because SLE is highly active and body will use them up

85

What is lupus nephritis?

Kidney inflammation caused by lupus

86

Levels of IgG and anti-dsDNA in active SLE?

Levels of IgG and anti-dsDNA will increase

87

Jaccoud arthropathy

Jaccoud arthropahy (JA) is a deforming non erosive arthropathy characterised by ulnar deviation of the second to 5th fingers with MCP subluxation.
-Can be seen in SLE

88

Pulmonary features of SLE?

-Pleurisy
-Infections
-Pulmonary hypertension
-Pulmonary infarct
-Diffuse lung infiltration and fibrosis

89

Which type of endocarditis might be seen in SLE?

Libman-sach endocarditis

90

SLE cardiac features

Cardiomyopathy
Libman-sach endocarditis
Pulmonary hypertension
Pericarditis

91

Screening tests for suspected SLE

Full blood count
Renal function incl. urine
ANA
ENA
Anti-DNA
Complement levels

92

Which immunological thingy is specific for SLE?

Anti-DNA
-Correlates with disease activity
-may be associated with lupus nephritis
Anti-Sm also very specific

93

Anti-DNA is highly specific for what?

SLEEEEEE
-Correlates with activity
-may be associated with lupus nephritis
Anti-Sm is also very specific

94

Anti-Ro is associated with which manifestations

Cutaneous

95

Anti-RNP overlap features

Overlap features – sclerodermatous skin lesions, Raynaud’s phenomenon, low grade myositis

96

If you have SLE should you go to the desert?

No, you want to avoid excessive sun exposure

97

Anti-CD20

Rituximab (one of the biologics used in SLE)

98

Anti-Blys

Belimumab

99

Ciclosporin A and tacrolimus are what kind of drug?

Calcineurin inhibitors

100

Name 2 calcineurin inhibitor

Ciclosporin A and tacrolimus