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Orthopaedics > Connective Tissue Disorders > Flashcards

Connective Tissue Disorders Flashcards

1
Q

What are connective tissue diseases?

A

Autoimmune conditions of the connective tissue

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2
Q

Name some autoimmune polygenic diseases.

A 
Rhuematoid arthritis
Systemic sclerosis
Polymyositis
Dermatomyositis
SLE
Sjorgren Syndrome
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3
Q

What is polymyositis?

A

Chronic inflammation of striated muscle

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4
Q

What is dermatomyositis?

A

Chronic inflammation of striated muscle with a rash

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5
Q

What is dermatomyositis associated with?

A

Elderly
- malignancy (paraneoplastic synrome)
Children
- nothing

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6
Q

What are the diagnostic features of polymyositis and dermatomyositis?

A

1) Painful proximal myopathy with weakness
2) Evidence of inflammation in the muscle (biopsy or MRI)
3) Elevation of muscle enzymes
4) Characteristic EMG pattern
5) Characteristic JDM rash - this makes it dermatomyositis

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7
Q

At what age is dermatomyositis most common?

A

incidence increases rapidly after the age of 35 and peaks between the ages of 55-65

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8
Q

Describe the heliotrope rash in dermatomyositis.

A

Can be itchy or burning
Purple eyelids
- can cross the bridge of the nose
- patches of erythema on the torso, upper limbs and buttocks may occur

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9
Q

What are Gottron’s papules?

A

Scaly skin with a red, vasculitic base
- appears on extensor surfaces (PIP and MCP joints), also the knees and elbows
When these heal they can cause atrophic scarring

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10
Q

What nail fold changes are typical in children with Junveille dermatomyositis?

A

Nailfold capillary dilation

- formation of mega capillaries followed by areas of infarct and drop out

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11
Q

What is calcinosis in relation to dermatomyositis?

A

Most common in JDMS than adult disease
Subcutaneous calcification deposits
Can cause secondary prevention and functional loss (when deposited in the hands)

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12
Q

What is calcinosis universalis?

A

Long bands or sheets of symmetrical subcutaneous calcification
- causes fatigue, muscle pain and stiffness

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13
Q

What are the clinical features of proximal muscle weakness in children?

A

Failure to achieve milestones or loose milestones already achieved
Muscle pain (60%)
Children maintain flexed elbows and knees (promotes contracture development)
Gower’s sign
- pushing himself up on his legs

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14
Q

Name some investigations you can do in dermatomyositis?

A 
Muscle enzymes
- creatinine kinase
- alanine transaminase 
- lactate dehydrogenase
MRI of the muscle
Muscle biopsy
Clinical monitoring 
- pulmonary function tests
- tests of swallowing and video fluroscopy
- sequential power testing
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15
Q

How can dermatomyositis be seen on MRI?

A

T2 weighted image

  • water is bright in inflamed muscle
  • muscle brightness ratio to fat is normally 0.4 or less
  • an increase in the ratio indicates active disease
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16
Q

List the symptoms associated with dermatomyositis/myositis.

A 
Proximal muscle weakness
Muscle pain
General fatigue and tiredness
Difficulty swallowing/breathing 
Gottron's papules
Dry cough
Rash on knuckles, chest, face and back
Heliotrope rash
Calcinosis
 Nail fold capillary dilation
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17
Q

Describe the rash associated with SLE.

A

Butterfly rash across the face
- malor (looks photosensitive)
- blackheads in the area
Rash spares the nares, but covers the bridge of the nose

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18
Q

What is generalised acute lupus?

A

Generalised macular papular rash (representing a photosensitive dermatitis)

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19
Q

List some of the symptoms of systemic lupus erythematosus.

A 
Oral mucosal ulceration
Alopecia - diffuse or scarring
Skin vasculitis
Skin rash (including butterfly rash)
Joint pain and stiffness
Fever
Extreme fatigue 
Weight loss
Capillary dilation
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20
Q

What is discoid systemic lupus erythematosus?

A

This is a subtype of chronic cutaneous lupus erythematosus
This presents with erythematous-to-violaceous, scaly plaues
- prominent follicular plugging
- results in scarring and atrophy
Can present in the absence of systemic disease

21
Q

What is the difference between chronic, subacute and acute cutaneous lupus skin symptoms?

A 
Chronic
- thick, red, scaly patches
Subacute
- red, scaly patches of skin
Acute
- rash (classically malar rash on the face)
22
Q

What are the dermatological manifestations of juvenile SLE?

A

Malar rash (butterfly)
Photosensitivity
Alopecia (diffuse)
Raynauds

23
Q

What is the treatment for juvenile SLE?

A

High SPF sunblock
Anti-malarials
Topical steriods
Low dose prednisolone

24
Q

Describe the systemic involvement with Juvenile SLE.

A 
Dermatitis (60-90%)
Renal (60-80%)
Liver 
Spleen
CNS (10-25%)
Systemic vasculitis 
Serositis
- pleurisy
- pericarditis
- peritonitis 
Arthritis (70-90%)
25
Q

What is neuropsychiatric lupus?

A

A description of the ways in which lupus can affect the neurological systems

26
Q

List the effects of neuropsychiatric lupus in children.

A 
Headache
- severe (cerebral vein thrombosis)
- pseudotumour cerebri
- migraine
Depression 
Organic syndromes
- cognitive impairment
- seizures
- cranial neuropathies (II, III, IV, VI)
- mononeuritis multiplex
- Guillain-Barre
Psychosis
27
Q

What are the classification criteria in a diagnosis of SLE?

A 
Malar rash
Discoid rash
Photosensitivity
Oral ulcers
Arthritis
Serositis
Renal disorder
Neurological disorder
Haematological disorder
- haemolytic anaemia 
- leukopenia 
- thrombocytopenia
Immunological disorder
ANA
28
Q

What immunological tests can be done in a person with suspected lupus?

A

Anti-dsDNA
Anti SM
False positive serology for syphylis
Positive LE preparation

29
Q

If a person has an ANA negative test, can they have lupus?

A

Yes
- sensitive not specific
Found in healthy people and other conditions
ANA doesn’t diagnose SLE, but supports the diagnosis
A diagnosis of SLE in the absence of ANA is very unlikely

30
Q

If a person has a positive dsDNA autoantibody test, do they have Lupus?

A

Yes
- specific, not sensitive
So, 50% of people without dsDNA positive results may still have SLE
Used for disease monitoring as it goes away when patients improve

31
Q

What are the three types of Scleroderma?

A

Localised
Systemic
CREST

32
Q

At what age is there highest incidence of systemic scleroderma?

A

30-50 years

33
Q

What are the clinical signs of systemic scleroderma?

A 
Vascular compromise 
- collagen excess causes vascular leakiness/oedema 
Tight skin
Digital infarcts
Raynaud's
34
Q

Which systems are affected in systemic scleroderma?

A

Lung
Renal
GIT
Joints

35
Q

What are the sub-groups of localised scleroderma?

A

Morphea
Linear scleroderma
CREST

36
Q

Describe morphea scleroderma.

A

Several patches of scleroderma appear on the trunk, abdomen or proximal limb
The lesions are oval patches of skin
They start of mauve in colour and then become ivory with a lilac halo

37
Q

Describe linear scleroderma.

A

Sclerodermic lesions appear in lines/bands
They start of mauve in colour and then become ivory with a lilac halo
Usually occur more distally (starts as a band of discoloured skin)

38
Q

What are the complications of localised scleroderma linear?

A

Duration of activity tends to be longer
Can limit joint movement when crossing a joint margin
The band of skin may feel warm and have some erythema

39
Q

Why is limited cutaneous scleroderma sometimes referred to as CREST syndrome scleroderma?

A

It is an acronym that refers to the five main features

  • Calcinosis
  • Raynaud’s
  • oEsophageal dysmotility
  • Sclerodactyly
  • Telangiectasia
40
Q

What is a main difference between systemic scleroderma and CREST syndrome scleroderma?

A

Lack of involvement of the kidneys in CREST

41
Q

What is Sjogren’s syndrome?

A

An autoimmune disorder where the immune system attacks glands that secrete fluids
- e.g. tear and saliva glands

42
Q

What are the symptoms of Sjorgren’s syndrome?

A

Dry eyes
Dry mouth
Salivary glands enlarge and become tender
Dry mucosal membranes (e.g. vagina)

43
Q

What non-Sicca symptoms can be associated with Sjorgren’s syndrome?

A 
Arthalgia 
Raynaud's
Neurological
- cranial or peripheral neuropathy
- e.g. trigeminal neuralgia 
Kidneys
Lung
Lymphoma
44
Q

Which autoantibodies are associated with SLE?

A

dsDNA
Ro
Smith

45
Q

Which autoantibodies are associated with Sjorgren’s syndrome?

A

Ro

La

46
Q

Which autoantibodies are associated with dermatomyositis/polymyositis?

A

Jo-1

47
Q

Which autoantibodies are associated with scleroderma?

A

Scl-70

48
Q

Which autoantibodies are associated with mixed connective tissue diseases?

A

Ribonucleoprotein

49
Q

Which autoantibodies are associated with CREST syndrome?

A

centromere

Orthopaedics (13 decks)

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