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MUSCULOSKELETAL > CONNECTIVE TISSUE DISEASE > Flashcards

CONNECTIVE TISSUE DISEASE Flashcards

1
Q

Examples of heritable connective tissue disorders?

A

Marian’s syndrome
Ehlers-danlos syndrome
Osteogenesis imperfecta
Alport syndrome

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2
Q

How is marfan’s syndrome inherited?

A

Autosomal dominant

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3
Q

Pathophysiology of Marfan syndrome?

A

Defect in the FBN1 gene on chromosome 15 that codes for the protein Fibrillin-1 which is an important component of connective tissue = abnormal connective tissue

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4
Q

Features of marfan’s syndrome?

A

Tall stature with arm span:height >1.05
Long neck and limbs
High-arched palate
Arachnodactyly (long fingers)
Pes planus
Pes excavatum
Scoliosis >20 degrees
Heart problems - aortic and mitral valve disease
Hypermobile
Lung - repeated pneumothoraces
Eyes - upward lens disclocation, blue sclera and myopia
Dural ectasia

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5
Q

How can marfan’s syndrome affect the heart?

A

It can cause dilatation of the aortic sinuses in 90% -> aortic aneurysm, aortic dissection, aortic regurgitation
Can also cause mitral valve prolapse in 75%

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6
Q

How can marfan’s syndrome affect the lungs?

A

It can cause repeat pneumothoraces

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7
Q

How can marfan’s syndrome affect the eyes?

A

It can cause superotemporal ectopic lentis (upward lens dislocation)
Blue sclera
Myopia

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8
Q

Associated conditions to marfan’s syndrome?

A

Lens dislocation in the eye
Joint dislocations and pain due to hypermobility
Scoliosis of the spine
Pneumothorax
Gastro-oesophageal reflux
Mitral valve prolapse with regurgitation
Aortic valve prolapse with regurgitation
Aortic aneurysms

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9
Q

Management of marfan’s syndrome?

A

Minimise BP and HR - lifestyle changes, antihypertensives
PT to strength joints and reduce symptoms from hypermobility
Genetic counselling

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10
Q

Monitoring for marfan’s syndrome?

A

Annual echocardiograms and ophthalmology review - to check for complications

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11
Q

Bigegst risk of marfan’s syndrome?

A

Cardiac conditions e.g. aortic dissection and valve prolapses

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12
Q

Life expectancy of marfan’s syndrome?

A

It’s now nearly 70

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13
Q

What is Ehlers-Danlos syndrome?

A

A group of genetic conditions involving defects in type III collagen which results in the tissue being more elastic than normal = joint hypermobility and increased elasticity of the skin

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14
Q

Types of Ehlers-Danlos syndrome?

A

Hypermobile Ehlers-Danlos syndrome
Classical Ehlers-Danlos syndrome
Vascular Ehlers-Danlos syndrome
Kyphoscoliotic Ehlers-Danlos syndrome

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15
Q

Most common and least severe type of Ehlers-Danlos syndrome?

A

Hypermobile Ehlers-Danlos syndrome

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16
Q

Key features of Hypermobile Ehlers-Danlos syndrome?

A

Joint hypermobility -> may cause recurrent joint dislocation, joint pain or clicking
Soft, stretchy skin - fragile, easy bruising
Postural orthostatic tachycardia syndrome can occur alongside due to autonomic dysfunction
May also cause organ prolapses, mitral valve problems or urinary incontinence

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17
Q

Features of classical Ehlers-Danlos syndrome?

A

Stretchy skin that feels smooth and velvety, can split easily especially over forehead/knees/shins/elbows. Can also bruise easily
Severe joint hypermobility, joint pain, easily dislocate
Abnormal wound healing leaving wide scars
Lumps often develop over pressure points e.g. elbows
Prone to hernias, prolapses, mitral regurgitation and aortic root dilatation

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18
Q

Inheritance pattern of Ehlers-Danlos syndrome?

A

All autosomal dominant except for kyphoscoliotic Ehlers-Danlos syndrome which is autosomal recessive

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19
Q

Most severe and dangerous form of x Ehlers-Danlos syndrome?

A

Vascular Ehlers-Danlos syndrome

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20
Q

Features of Ehlers-Danlos syndrome?

A

Thin, translucent skin with visible small blood vessels especially on upper chest and legs
Blood vessel rupture -> serious internal bleeding
GI perforation
Spontaneous pneumothorax

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21
Q

Features of kyphoscoliotic Ehlers-Danlos syndrome?

A

Hypotonia as a neonate and infant
Kyphoscoliosis as they grow
Significant joint hypermobility and join dislocation is common
Soft, velvety skin that is stretchy, bruises easily and scars

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22
Q

What is POTS?

A

Postural orthostatic tachycardia syndrome
When significant tachycardia occurs on sitting or standing and symptoms include presyncope, syncope, headaches, disorientation, nausea and tremor

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23
Q

What is the Beighton score?

A

A score used to assess for hypermobility and to support a diagnosis of ehlers-danlos syndrome

One point is scored for each side of the body, with a maximum score of 9, if the patient can:
Place their palms flat on the floor with their straight legs (scores only 1)
Hyperextend their elbows
Hyperextend their knees
Bend their thumb to touch their forearm
Hyperextend their little finger past 90 degrees

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24
Q

Management of Ehlers-danlos syndrome?

A

No cure
Management focuses on maintaining healthy joints, managing symptoms, supporting ADLs and monitoring for complications

PT, OT, moderating activity, psychology

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25
Q

Complications of ehlers-danlos syndrome?

A

Hypermobility leads to premature osteoarthritis and joint dislocations
Prominent scarring due to fragile skin
Arterial rupture
Organ rupture
Chronic pain and fatigue

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26
Q

What is osteogenesis imperfecta also known as?

A

Brittle bone disease

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27
Q

What is osteogenesis imperfecta?

A

A group of inherited disorders of collagen metabolism resulting in bone fragility and fractures

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28
Q

Most common type of osteogenesis imperfecta?

A

Type 1

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29
Q

Inheritance pattern of osteogenesis imperfecta?

A

AD

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30
Q

Pathogenesis of osteogenesis imperfecta?

A

Decreased synthesis of pro-alpha 1 or pro-alpha 2 collagen polypeptides = abnormalities in type 1 collagen

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31
Q

When does osteogenesis imperfecta present?

A

In children

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32
Q

Presentation of osteogenesis imperfecta?

A

Fractures following minor trauma
Blue sclera (due to thinning of sclera which reveals the coloured vasculature underneath)
Deafness from early adulthood (secondary to osteosclerosis)
Dental imperfections particularly with formation of teeth
Hypermobility
Short stature
Bone deformities e.g. bowed legs, scoliosis, joint pain

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33
Q

Investigtaions for osteogenesis imperfecta?

A

None are required but you may want to rule out other causes:
FBC
Vitamin D
Bone profile - Adjusted calcium and Phosphate should be normal
Parathyroid hormone - should be normal
LFTs - ALP normal
Xrays and bone density scans may be done - to diagnose fractures and bone deformities
Genetic testing can be done but isnt routine

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34
Q

Management of osteogenesis imperfecta?

A

Bisphosphonates to improve bone density
Vitamin D supplements
PT and OT
Mange fractures

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35
Q

What is Alports syndrome?

A

An inherited condition which causes an abnormal glomerular-basement membrane

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36
Q

Pathophysiology of Alports syndrome?

A

Defect in the gene which codes for type 4 collagen resulting in abnormal GBM

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37
Q

How is Alports syndrome inherited?

A

X-linked dominant

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38
Q

Who typically gets Alports syndrome?

A

More severe in males as X-linked
Females rarely develop renal failure
Presents in childhood usually

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39
Q

Features of Alport syndrome?

A

microscopic haematuria
progressive renal failure
bilateral sensorineural deafness
lenticonus: protrusion of the lens surface into the anterior chamber
retinitis pigmentosa

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40
Q

Diagnosis of Alports syndrome?

A

Molecular genetic testing
Renal biopsy

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41
Q

What is seen on renal biopsy in Alports syndrome?

A

Longitudinal splitting of the lamina densa of the GBM resulting in a ‘basket weave’ appearance

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42
Q

Examples of autoimmune connective tissue disorders?

A

SLE
RA
Scleroderma
Sjögren’s syndrome
Mixed connective tissue disease
Myosotis
Antiphospholipid syndrome may also be considered it

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43
Q

Who does SLE typically affect?

A

Females 9:1
Afro-Caribbean’s and Asian communities
20-40 year olds

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44
Q

Pathophysiology of SLE?

A

Autoimmune - a type 3 hypersensitivity reaction = immune system dysregulationleading to immune complex formation = immune complex deposition can affect any organ (skin, joints, kidneys, brain etc)
Associated with HLA B8, DR2 and DR3

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45
Q

Antibodies in SLE?

A

Anti-nuclear antibodies
Anti-dsDNA
Anti-sm

Others: anti-Ro and anti-La, antiphospholipid antibodies (bear in mind these aren’t specific)

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46
Q

Course of disease in SLE?

A

Relapsing-remitting course with flares of worse symptoms and periods where symptoms settle
The result of chronic inflammation can shorten life expectancy

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47
Q

Features of SLE?

A

General - fever, fatigue, mouth ulcers, lymphadenopathy

Skin - malar rash that spares nasolabial folds, discoid rash, photosensitivity, raynauds, livedo reticularis or non-scarring Alopecia

MSK - arthralgia and non-erosive arthritis

CVD - pericarditis and myocarditis

Resp - pleurisy and fibrosing alveolitis

Renal - proteinuria or glomerulonephritis

Neuro - anxiety, depression, psychosis, seizures

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48
Q

Investigations for SLE?

A

Urinalysis - proteinuria
FBC - anaemic of chronic disease, low WCC, low Plt
Antibodies - ANA, RF, anti-dsDNA, anti-Smith
ESR and CRP
Complement levels - C3 and C4
Renal biopsy may be done

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49
Q

What is used in SLE to look at disease activity, CRP or ESR and why?

A

ESR
As during active disease the CRP may be Normal. If CRP is raised then it may indicate an underlying infection

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50
Q

Why are complement levels low during active SLE?

A

As the formation of complexes leads to consumption of the complements

51
Q

Which antibody has the highest sensitivity for SLE?

A

ANA - 99% are positive

52
Q

Which antibodies are highly specific for SLE?

A

Anti-dsDNA - >99% specificity
Anti-Smith - >99% specificity

53
Q

Management of SLE?

A

NSAIDs
Sunblock to avoid the malar rash
Hydroxychloroquine

If internal organ involvement e.g. eyes, kidneys, then consider cyclophosphamide

Others: biological therapies and other DMARDs

54
Q

Leading cause of death in SLE?

A

Cardiovascular disease - chronic inflammation in blood vessels leads to hypertension and CAD

55
Q

Complication sof SLE?

A

Cardiovascular - hypertension, CAD, pericarditis
Infections
Anemia
Lungs - pleuritic, interstitial lung disease
Kidneys - lupus nephritis
Neuropsychiatric SLE - optic neuritis, transverse myelitis, psychosis
Recurrent miscarriage
VTE

56
Q

What is lupus nephritis?

A

Kidney disease caused by SLE that can progress to end-stage renal disease

57
Q

How are SLE pt monitored?

A

Urinalysis as regular appointments to rule out proteinuria for lupus nephritis

58
Q

Most common type of lupus nephritis?

A

Diffuse proliferative glomerulonephritis - most common & most severe form

59
Q

What is discoid lupud erythematous?

A

A benign autoimmune disorder seen in younger females that can very rarely progress to SLE
It’s characterised by follicular keratin plugs = erythematous, raised, scaly rash that may be photosensitivity. On face/neck/ears/scalp
Lesions heal with atrophy, scarring and pigmentation

60
Q

Management of discoid lupus erythematosus?

A

Topical steroids
Avoid sun exposure

Hydroxychloroquine is second line

61
Q

What is systemic sclerosis?

A

A condition of unknown aetiology characterised by hardened, sclerotic skin and other connective tissue
Aka scleroderma

62
Q

Which gender is systemic sclerosis more common in?

A

Females

63
Q

What are thw 3 patterns of disease in systemic sclerosis?

A

Limited cutaneous systemic sclerosis
Diffuse cutaneous systemic sclerosis
Scleroderma

64
Q

What is scleroderma?

A

Tightening and fibrosis of the skin that may manifest as plaques or linear
Most pt with scleroderma have systemic sclerosis but a localised version of scleroderma only affects the skin and no other organs

65
Q

What are the features of limited cutaneous systemic sclerosis?

A

CREST

Calcinosis - mostly seen in fingers
Raynaud’s phenomenon - often the first sign
Esophageal dysmotility
Sclerodactyly
Telangiectasia

Affects the face and distal limbs mostly

66
Q

Antibodies associated with limited cutaneous systemic sclerosis?

A

Anti-centromere antibodies

67
Q

Clinical features of diffuse cutaneous systemic sclerosis?

A

Affects trunk and proximal limbs mostly

Has CREST features and affects internal organs. Affects the cardiovadcular system, lungs and kidneys

68
Q

Most common cause of death associated with diffuse cutaneous systemic sclerosis?

A

Respiratory involvement is in 80% of pt - interstitial lung disease and pulmonary arterial hypertension

69
Q

Antibodies associated with diffuse cutaneous systemic sclerosis?

A

Anti scl-70 antibodies

70
Q

What is sclerodactyly?

A

Localized thickening and tightness of the skin of the fingers or toes that yields a characteristic claw-like appearance and spindle shape of the affected digits, and renders them immobile or of limited mobility.

Skin can break and ulcerate

71
Q

What is Calcinosis?

A

Calcium deposits under the skin, most commonly found on the fingertips

72
Q

What is oesophageal dysmotility caused by in systemic sclerosis?

A

Atrophy and dysfunction of the smooth muscle, and fibrosis of the oesophagus
Causes swallowing difficult, chest pain, acid reflux and oesophagitis

73
Q

What is Raynaud’s phenomenon?

A

When fingertips change colour in response to even mildly cold triggers - caused by vasoconstriction of the vessels supplying the fingers
Causes white -> blue -> red

74
Q

What is Raynaud’s disease?

A

When Raynaud’s phenomenon occurs without an associated systemic disease
Idiopathic
Makes up nearly 90% of pt with Raynaud’s phenomenon

75
Q

Systemic diseases that can cause Raynaud’s phenomenon?

A

Systemic sclerosis
SLE - less commonly
RA

76
Q

Treatment options for Raynaud’s?

A

Keeping hands warm
CCB

77
Q

Antibodies positive in systemic sclerosis?

A

ANA is positive in most cases
Anticentromere antibodies are most associated with limited cutaneous systemic sclerosis
Anti-scl-70 antibodies are most associated with diffuse cutaneous systemic sclerosis

78
Q

What test can be done to determine Raynaud’s disease from Raynaud’s phenomenon secondary to systemic sclerosis ?

A

Nailfold capillaroscopy - magnifies and examines the peripheral capillaries where the skin meets the base of the fingernail
Abnormal capillaries, avascular area and micro-haemorrhages suggest systemic sclerosis s

79
Q

Diagnosis of systemic sclerosis?

A

Clinical features e.g. Resp exam to look for interstitial lung disease or pulmonary hypertension
Antibodies
Nailfold capillaroscopy

80
Q

Management of systemic sclerosis?

A

Non-medical : avoid smoking, gently skin stretching to maintain range of motion, regular emollients, avoid cold triggers for raynauds, physio to help maintain healthy joints, OT

Manage based on symptoms:
Nifedpine for raynauds
PPI for acid reflux
Analgesia for joint pain
Antihypertensives to treat hypertension
Etc

DMARDs and biological therapies
Steroids may be considered but increase risk of renal crisis

81
Q

What is Sjögren’s syndrome?

A

An autoimmune condition affecting the exocrine glands causing dry mucosal surfaces, notable the lacrimal and salivary glands

82
Q

What are sicca symptoms?

A

Dry eyes and dry mouth

83
Q

What are the 2 types of Sjögren’s syndrome?

A

Primary - occurs in isoalation
Secondary - usually develops about 10 years after the onset of RA or other CTD

84
Q

Who is Sjögren’s syndrome most common in?

A

Female f:m 9:1
Middle age
Those with CTD e.g. RA

85
Q

What complication is markedly increased risk of in Sjögren’s syndrome?

A

Lymphoid malignancy - risk increases by 40-60 fold

86
Q

Clinical features of Sjögren’s syndrome?

A

Keratoconjunctivits sicca - dry eyes
Dry mouth
Dry vagina
Arthralgia
Raynaud’s
Sensory polyneuropathy
Recurrent episodes of parotitis
Renal tubular acidosis

87
Q

Investigtaions for Sjögren’s syndrome?

A

ANA - positive in 70%
Anti-Ro - positive in 70%
Anti-La - positive in 30%
RF - positive in nearly 50%
Schirmers test
Salivary gland biopsy may be done to confirm the diagnosis but is not usually necessary
Ophthalmology assessment

Will also have hypergammaglobulinaemia and low C4

88
Q

What is schirmers test?

A

The Schirmer test involves inserting folded filter paper under the lower eyelid with the end hanging out. Moisture from the eye will travel by diffusion along the filter paper. After 5 minutes, the distance that the moisture travels along the filter paper is measured. In a healthy young adult, 15mm is expected. Less than 10mm is significant = suggests sjogrens

89
Q

Management of Sjögren’s syndrome?

A

artificial saliva and tears
Vaginal lubricants
pilocarpine may stimulate saliva and tear production

Hydroxychloroquine may be considered in pt with associated joint pain

90
Q

How does pilocarpine help in Sjogren syndrome>

A

Pilocarpine stimulates muscarinic receptors, stimulating the parasympathetic nerves and promoting salivary and lacrimal gland secretion.

91
Q

Complications of Sjögren’s syndrome?

A

Complications relate to the exocrine gland dysfunction:
Eye problems, such as keratoconjunctivitis sicca and corneal ulcers
Oral problems, such as dental cavities and candida infections
Vaginal problems, such as candida infection and sexual dysfunction

Others: non-hodgkin lymphoma, pneumonia, bronchiectasis, peripheral neuropathy, vascultiis, renal impairment

92
Q

What is autoimmune sialednitis?

A

Aka Sjögren’s syndrome

93
Q

What is mixed connective tissue disease?

A

Mixture of signs and symptoms from other CTD e.g. lupus, scleroderma and myositis

94
Q

What is scurvy?

A

Deifivcnecy in vitamin C that leads to impaired collagen synthesis and disordered connective tissue (as ascorbic acid is a cofactor for enzymes used in the production of proline and lysine)

95
Q

Who gets scurvy?

A

Those with severe malnutrition
Drug and alcohol abuse
Those living with poverty with limited access to fruit and vegetables

96
Q

Symptoms and signs of scurvy?

A

Follicular hyperkeratosis and perifollicular haemorrhage
Ecchymosis, easy bruising
Poor wound healing
Gingivitis with bleeding and receding gums
Sjogren’s syndrome
Arthralgia
Oedema
Impaired wound healing
Generalised symptoms such as weakness, malaise, anorexia and depression

97
Q

What is Peyronie’s disease?

A

A disease that causes the penis to become curved when its erect due to fibrous plaque formation in the corpus cavernous
Mostly affects men >40
Cause isnt really known; May be autoimmune or caused by trauma

98
Q

What is myositis?

A

Muscle inflammation

99
Q

Pathophysiology of polymyositis?

A

An inflamamtory disorder caused by T-cell mediated cytotoxic processes directed against muscle fibres
May be idiopathic or associated with CTD
May be caused by an underlying cancer, making is a paraneoplastic syndrome

100
Q

Who does polymyositis typically affect?

A

Middle aged females (f:m 3:1)

101
Q

Features of polymyositis?

A

Gradual onset, symmetrical proximal muscle weakness and tenderness
Raynauds
Respiratory muscle weakness
Interstitial lung disease e.g. fibrosing alveolitis or pneumonia
Dysphagia and dysphonia

102
Q

Investigtaions for polymyositis?

A

Elevated CK
Other muscle enzymes elevated - LDH, aldolase, AST, ALT
EMG
Muscle biopsy
Anti-synthetase antibodies: anti-Jo1 antibodies are seen in pattern of disease associated with lung involvement, raynauds and fever

103
Q

Management of polymyositis?

A

high-dose corticosteroids tapered as symptoms improve
azathioprine may be used as a steroid-sparing agent

104
Q

What is dermatomyositis?

A

an inflammatory disorder causing symmetrical, proximal muscle weakness and characteristic skin lesions

105
Q

What can cause dermatomyositis?

A

May be idiopathic
May be associated with a CTD or underlying malignancy (latter in up to 25%) so screening for cancer is usual performed

106
Q

Which malignancies are most commonly associated with dermatomyositis?

A

Ovarian
Breast
Lung

107
Q

Skin features of dermatomyosiits?

A

photosensitive
macular rash over back and shoulder
heliotrope rash in the periorbital region
Gottron’s papules - roughened red papules over extensor surfaces of fingers
‘mechanic’s hands’: extremely dry and scaly hands with linear ‘cracks’ on the palmar and lateral aspects of the fingers
nail fold capillary dilatation

108
Q

Investigations for dermatomyositis?

A

80% ANA positive
30% have anti-synthetase antibodies e.g. anti-Jo1, anti-Mi-2 and SRP antibodies

109
Q

Presentation of dermatomyositis?

A

Classic skin features

Other features the same as polymyositis e.g. proximal muscle weakness and tendernes, raynauds, resp muscle weakness, interstitial lung disease, dysphagia and dysphonia

110
Q

Causes of raised CK?

A

Myositis
Rhabdomyolysis
AKI
MI
Statins
Strenuous exercise

111
Q

Most common antibod associated with myositis?

A

Anti-jo-1 for polymyositis

112
Q

Management of polymyositis and dermatomyositis?

A

Corticosteroids

113
Q

Epidemiology for stills disease?

A

Bifocal 15-25 and 35-46

114
Q

What is adult onset-stills disease?

A

A rare inflammatory arthritis

115
Q

Presentation of adult onset-stills disease?

A

Arthralgia
Salmon-pink maculopapular rash
Pyrexia in late afternoon/early evening in a daily pattern and accompanies a worsening of joint symptoms
Lymphadenopathy

116
Q

What diagnostic criteria is used for adult onset-stills disease?

A

Yamaguchi criteria

117
Q

What is yamaguchi criteria for adult onset-stills disease?

A

To diagnose adult onset-stills disease you need 4 major criteria or 3 major & 2 minor

Major:
Fever >39 lasting 1 week at least
Arthralgia or arthritis >2 weeks
Typical rash
Leukocytosis >10,000 with >80% polymorphonuclear cells

Minor:
Sore throat
Recent development of lymphadenopathy
Hepato/splenomegaly
Abnormal LFTs
Negative ANA and RF

118
Q

Management of adult onset-stills disease?

A

NSAIDs - try for a week before adding steroids
Steroids

If sympotms persist you can consider methotrexate, IL-1 or anti-TNF

119
Q

What causes antiphospholipid syndrome?

A

It may occur as a primary disorder or secondary to other conditions e.g. SLE

120
Q

Features of antiphospholipid syndrome?

A

venous or arterial thrombosis
Recurrent miscarriages
Livedo reticularis
Pre-eclampsia
Pulmonary hypertension

121
Q

Investigations for antiphospholipid syndrome?

A

Antibiotics - anticardiolipin, anti-beta2 glycoprotein I, lupus anticoagulant
Thrombocytopenia
Prolonged APTT

122
Q

Primary thromboprophylaxis of antiphospholipid syndrome?

A

Low-dose aspirin

123
Q

Secondary thromboprophylaxis of antiphospholipid syndrome?

A

After 1 thromboembolic event - Lifelong warfarin

After multiple thromboembolic events - consider adding low-dose aspirin

124
Q

INR target for antiphospholipid syndrome?

A

After 1 thromboembolic event 2-3
After multiple 3-4

MUSCULOSKELETAL (13 decks)

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