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Soft Tissue Pathology Flashcards

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1
Q

What is the function of the perimysium?

A

Surrounds bundles of muscle fibres

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2
Q

Function of the endomysium?

A

Surrounds individual fibres

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3
Q

Function of the epimysium?

A

Surrounds the entire muscle

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4
Q

Ragged red fibres are seen in muscle when?

A

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

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5
Q

Tubular aggregates are seen in muscle when?

A

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

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6
Q

Types of skeletal muscle fibre? (3)

A

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

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7
Q

Muscle Biopsy Indications

A

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

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8
Q

Creatine Kinase High, Intermediate and Low levels

A

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

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9
Q

What is electron microscopy used for?

A

To examine the ultra-structure of the muscle

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10
Q

Duchenne (DMD) method of inheritance and where found?

A

X-linked recessive, pelvic girdle

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11
Q

Becker (BMD) method of inheritance and where found?

A

X-linked recessive, pelvic girdle

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12
Q

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

A

Autosomal recessive, pelvic girdle

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13
Q

Fascioscapulohumeral method of inheritance and where found?

A

Dominant, face, shoulder girdle and arm

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14
Q

Scapulohumeral method of inheritance and where found?

A

Autosomal recessive, shoulder girdle and arm

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15
Q

Oculopharyngeal method of inheritance and where found?

A

Dominant, external ocular and pharynx

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16
Q

Myotonic dystrophy method of inheritance and where found?

A

Dominant, face, respiratory and arms

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17
Q

Signs of Duchennes?

A

Proximal weakness, pseudohypertrophy of calves

RAISED CK

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18
Q

DMD pathogenesis

A
  • 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
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19
Q

Variant of DMD?

A

BMD (Becker)

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20
Q

Are muscular dystrophies inherited?

A

Yes

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21
Q

Which inflammatory marker will be raised in Duchennes?

A

CK!!!

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22
Q

Most common types of Myotonic dystrophy?

A

DM1 and DM2

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23
Q

Non-muscle features of myotonic dystrophy?

A

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

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24
Q

Atrophy of which type of fibres in myotonic dystrophy?

A

Atrophy of type 1 fibres in myotonic dystrophy

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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

Musculoskeletal (44 decks)

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