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Ortho and Rheum > Raynauds, Fibromyalgia, Osteoporosis, Sarcoid > Flashcards

Raynauds, Fibromyalgia, Osteoporosis, Sarcoid Flashcards

1
Q

What is sclerosis?

A

Hardening of a tissue

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2
Q

What is scleroderma?

A

Hardening of skin

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3
Q

What is included in the scleroderma spectrum?

A

Localised dermal sclerosis, through systemic conditions (cutaneous, internal organ fibrosis and vascular dysfunction) and purely vascular disorders eg. Raynauds

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4
Q

What is calcinosis

A

Formation of calcium deposits in skin- Sceloderma - small white calcium lumps form under the skin on fingers and other areas of the body

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5
Q

What is Raynaud’s phenomenon?

A

Episodic cold-induced vasospasm - triggered by cold or emotional stress

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6
Q

Incidence of Raynauds

A

It affects 5% of adult population, especially young females

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7
Q

What is Primary Raynauds and what % of Raynauds is this?

A

No other clinical or investigational abnormality in primary raynauds - this is 90%

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8
Q

What is secondary raynauds?

A

Implies other features - normally autoimmune rheumatic disease

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9
Q

Other causes of Raynauds?

A

Vibrating machine tools
Thoracic-outlet obstruction
Drugs - b-blockers
Haematological abnormalities- cryoglobulinaemia

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10
Q

Which isolated Raynauds patients are at increased risk of developing connective tissue disease?

A

Those that have positive antinuclear antibodies and abnormal nailfold capillaroscopy

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11
Q

What % of patients with connective tissue disease have Raynauds?

A

Many
Up to 95% in systemic scleroderma
Lupus and dermato/polymyositis - 50%
Less in Sjogrens and RA

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12
Q

What is most important disease on scleroderma spectrum?

A 
Systemic sclerosis (SSc)
High mortality
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13
Q

Cardinal features of SSc

A

Skin sclerosis with Raynauds and internal organ involvement

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14
Q

What are two subsets of SSc

A

Diffuse and limited cutaneous systemic sclerosis dcSSc and lcSSc

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15
Q

Features of dcSSc

A

Most important complications develop in first few years, skin sclerosis maximal 18-30months
After 5 years everything is stable or regressing - may have progression of existing visceral disease but new complications unlikely

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16
Q

Features of lcSSc

A

Skin involvement less extensive than dcSSc - may be limited to sclerodactlyly, face or neck
Raynauds very prominent and may present years before the rest
More severe symptoms develop after 5 years or so

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17
Q

Antibodies present in SSc and feature

A

Anti-centromere antibodies (more in localised) and scl-70 (more in diffuse)
Also anti-topoisomerase 1 - RNApol
Occur mutally exclusively, therefore if have one unlikely to have another

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18
Q

What is associated with poor 5 year survival in SSc? x3

A 
Systemic inflammation (high ESR)
Pulmonary disease (impaired diffusion capacity)
Renal involvement (proteinuria)
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19
Q

What are anti-topoisomerase antibodies associated with greater risk of in SSc?

A

Lung fibrosis

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20
Q

What are anti-RNA polymerase antibodies associated with greater risk of in SSc?

A

Renal crisis

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21
Q

What are anti-Th/To anitbodies associated with greater risk of in lcSSc?

A

Respiratory involvement

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22
Q

Management of SSc?

A

No disease modifying agents identified

Immunosuppression in early and aggressive dcSSc or if major organ complication

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23
Q

Importance of pulmonary arterial hypertension in SSc?

A

Single largest cause of death directly attributable to SSc

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24
Q

Drugs for pulmonary arterial hypertension in SSc x3

A

Endothelin receptor antagonists (bosentan, sitaxentan)

Selective phosphodiesterase inhibitors (sildenafil)

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25
Q

What is anti-scl70 autoantibody related to in SSc?

A

Lung fibrosis

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26
Q

Treatment of lung fibrosis in SSc?

A

Immunosuppressive treatment with cyclophosphamide has been shown to have modest effect
Better treatments are needed

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27
Q

Diagnosis of lung fibrosis in SSc?

A

HRCT - high resolution computed tomography

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28
Q

How does scleroderma renal crisis present?

A

Often with headaches and visual disturbances before full-blown encephalopathy, cardiac failure or acute oliguric renal failure develop

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29
Q

Treatment of scleroderma renal crisis

A

ACEi

50% require dialysis which is often temporary

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30
Q

What is the most affected organ in SSc?

A

GIT

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31
Q

Most common presentation of GIT disturbances in SSc?

A

Oesophageal dysmotility with reflux - treat with PPI

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32
Q

What is fibromyalgia syndrome?

A

Widespread musculoskeletal pain and hyperalgesic tender spots with no single identifiable organic cause

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33
Q

What is a more mild form of fibromyalgia on the same spectrum?

A

Chronic widespread pain

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34
Q

What other symptoms are present with fibromyalgia?

A

Stiffness, fatigue, sleep disturbance as well as some other physical and psychological symptoms

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35
Q

Prevalence of fibromyalgia

A

2%, chronic widespread pain is 12%

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36
Q

General criteria for fibromyalgia

A

Widespread pain in all 4 quadrants of the body
Present for at least 3 months
Hyperalgesic points, bilatearlly

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37
Q

Where are the hyperalgesic points in fibromyalgia? x9

A

Suboccipital muscle insertions
Low c-spine c5-c7 interspinous ligaments
Trapezius muscles at midpoint of upper border
Supraspinatous origins above scapula spine
Second costochondral junctions
2cm distal to lat.epiconyles
Upper outer quadrants of buttocks
Greater trochanters
Medial fat pads of knee proximal to joint margin

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38
Q

Investigations in fibromyalgia

A

To exclude other DDX
- TFTs, FBC, inflammatory markers, serum calcium and ALP, CK, BG
No diagnostic test

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39
Q

Typical clinical picture for fibromyalgia

A

Female, aged 30-50, with long standing diffuse pain
History of physical or psychological trauma
Symptoms exacerbated by other stressors in her life

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40
Q

What is cause of fibromyalgia

A

No cause been found - likely to be multifactorial characterised by abnormal processing of pain = central sensitisation

41
Q

Management of fibromyalgia

A

Assess and remove any psychological or social influences on pain (work issue, emotional distress, anxiety etc)
Educate patient that ned to balance life, exercise etc
CBT can be helpful
Graded exercise
Pacing activities
Relaxation
Developing sleep routine

42
Q

What is graded exercise in management of fibromyalgia?

A

Gradually increasing exercise as patients are generally physically de-conditioned and fearful that exercise will increase pain
Therefore gradually increase fitness and muscle strength
Increasing activity will have a positive effect on well-being and sleep

43
Q

Drug therapy in fibromyalgia x5

A

Not especially helpful
Amitriptyline may help with sleep, should help within 2 weeks, effect on pain may take 3-4 months
SSRI can help with energy
Duloxetine - can reduce pain
Pregabalin can help reduce pain and fatigue and improve sleep
Paracetamol may help with pain but little evidence for stronger narcotics

44
Q

What is osteoporosis?

A

Systemic skeletal disease characterised by low bone mass and micro-architectural deterioration of bone tissue - resulting in high risk of fracture

45
Q

Pathophysiology of osteoporosis

A

Increased remodelling of bone post-menopause and imbalance of resorption and formation in old age (trabecular bone more metabolically active therefore more commonly affected)
Trabecular perforation occurs leading to bone fragility

46
Q

Which are the classical osteoporotic fractures?

A

Spine, wrist and hip - but all fragility fractures in the elderly can be regarded as osteoporotic fractures once pathological (metastatic) fracture has been excluded

47
Q

Two classifications of osteoporosis

A

Primary - including post-menopausal and age-related

Secondary - where bone loss is accelerated by presence of underlying disease

48
Q

What % of osteoporosis is primary and what % is secondary in each gender?

A

40% of osteoporosis is secondary in women

60% of osteoporosis is secondary in men

49
Q

Diagnosis of osteoporosis according to BMD on DXA scan

A

Normal is within 1 SD
Osteopenia is 1-2.5 SD
Osteoporosis is greater than 2.5 SD
Severe osteoporosis is greater than 2.5 SD plus one or more fragility fractures

50
Q

Endocrine causes of secondary osteoporosis x5

A 
Thyrotoxicosis 
Primary hyperparathyroidism 
Cushing's syndrome 
Hypogonadism (including Anorexia)
T1DM
51
Q

GI causes of secondary osteoporosis x3

A

Malabsorption syndrome
IBD
Liver disease eg. primary biliary cirrhosis

52
Q

Rheumatological causes of secondary osteoporosis x2

A

RA

Ankylosing spondylitis

53
Q

Malignancy causes of secondary osteoporosis x2

A

Multiple myeloma

Cancer treatment induced bone loss

54
Q

Drug causes of secondary osteoporosis x5

A

Glucocorticoids, Anticonvulsants,
Heparin
Aromatase inhibitors (breast and ovarian cancer ttt)
Androgen-deprivation therapy (prostate cancer ttt, decreases testosterone)

55
Q

Factors included in FRAX tool x12

A

Age, sex, weight, height, previous fracture, parent hip fracture, smoking, alcohol >3units/day, glucocorticoids, RA, secondary osteoporosis, femoral neck BMD

56
Q

What does FRAX tool calculate?

A

Risk of major osteoporotic fracture (hip, wrist, proximal humerus or spine) and risk of hip fracture - 10 year probability

57
Q

What are 3 major determinants of persons risk of fracture?

A

Age
Prior fragility fracture (spinal radiographs can pick these asymptomatic fractures up)
BMD

58
Q

What are bisphosphonates?

A

Antiresorptive agents

59
Q

Once-weekly bisphosphonates x2

A

Alendronate (1st line) and risedronate

60
Q

What is Ibandronate

A

Once-monthly bisphosphonate or 3-monthly IV infusion

61
Q

What is Zoledronate

A

once-yearly short infusion bisphosphonate

62
Q

How do bisphosphonates need to be taken?

A

On an empty stomach before breakfast (30mins for alendronate and risedronate but 60mins for ibandronate)
Because of poor absorption and food reduces absorption
Also standing up to reduce oesophageal erosion

63
Q

When is HRT used in osteoporosis?

A

Now only thought to be appropriate in women under 50 who have undergone early menopause or to control climacteric symptoms

64
Q

What is Raloxifene

A

Selective oestrogen receptor modulator - anti-resorptive
Without effect on breast and endometrial tissue
Still small increased risk of thromboembolic events though

65
Q

What are two nutritional treatments for osteoporsis and how they work?

A

Calcium (adherence can be problematic)
Vitamin D (given to all housebound or in resident care elderly patients)
Anti-resorptive agents

66
Q

What does calcitonin do?

A

Reduce risk of vertebral fracture

Also has analgesic properties

67
Q

What are teriparatide and parathyroid hormone?

A

Formation-stimulating agents

Can increase bone formation - especially trabecular bone

68
Q

When are teriparatide and parathyroid hormone used?

A

Very expensive therefore only used in patients with severe progressive osteoporosis despite treatment with anti-resorptive agents

69
Q

In what type of osteoporosis is teriparatide licensed?

A

Glucocorticoid-induced osteoporsis - because been shown to increase spine and hip BMD greater than alendronate

70
Q

Pain relief in osteoporosis? x4

A

Analgesics
Hydrotherapy
Transcutaneous nerve stimulators
Low-dose antidepressants

71
Q

What is sarcoidosis?

A

Multisystem granulomatous inflammatory disorder

72
Q

Aetiology of sarcoidosis

A

Unknown

73
Q

Epidemiology of sarcoidosis

A

Uncommon, more common in 20-40 and in Africans and females

74
Q

Which glands swell in sarcoidosis

A

Parotid glands

75
Q

Type of cough in sarcoidosis

A

Usually unproductive

76
Q

What happens in bones in sarcoid

A

Bone cysts eg. dactylitis in phalanges

77
Q

Eye symptoms in sarcoid

A

Keratoconjuncitivitis sicca (dry eyes), papilloedema, uveitis

78
Q

Skin in sarcoid

A 
Lupus pernio (red-blue infiltrations of nose, cheek, ears, terminal phalanges) 
Erythema nodosum 
Maculopapular eruptions
79
Q

Pathology of sarcoidosis

A

Macrophage activation of CD4 lymphocytes - accumulate and release cytokines IL-1 and 2
Results in formation of non-caseating granulomas in variety of organs

80
Q

What is raised in serum in sarcoid

A

Serum ACE and Calcium

81
Q

What will show in 24h urine collection in sarcoid

A

Hypercalciuria

82
Q

CXR in sarcoid

A

Stage 0 - may be clear
Stage 1 - Bilateral hilar lymphadenopathy
Stage 2 - Stage 1 + pulmonary infiltration and paratracheal node enlargement
Stage 3 - Pulmonary infiltration and fibrosis

83
Q

Type of CT in sarcoid

A

High resolution CT showing diffuse lung involvement

84
Q

Type of scan to show in the inflammation in sarcoid

A

Gallium 67 scan - shows the classic parotid and eye swelling

85
Q

Pulmonary function tests in sarcoid

A

Reduced FEV1, FVC and gas transfer - showing a restrictive pattern

86
Q

What is seen on bronchoscopy and bronchoalveolar lavage

A

Increased lymphocytes with increased CD4:CD8 ratio

87
Q

What is seen on transbronchial lung biopsy or lymph node biopsy

A

Non-caseating granulomas composed of epithelial cells (activated macrophages) multinucleate Langhans cells and mononuclear cells (lymphocytes)

88
Q

Management of sarcoid

A

Corticosteroids for symptomatic pulmonary, cardiac and neurologic sarcoidosis

89
Q

MSK sarcoid management

A

NSAIDs
if no relief can try colchicine, hydroxychloroquine or prednisolone
If not enough or needing high dose steroids then can try methotrexate + folic acid
Next step - infliximab

90
Q

Eye management in sarcoid

A

Mydriatic (atropine)

Topical corticosteroids

91
Q

Lupus pernio treatment

A

Chloroquine or methotrexate

92
Q

What is CREST

A 
What limited systemic sclerosis used to be known as 
Calcinosis
Raynauds
Esophageal dysmotility
Sclerodactyly
Telangectasia
93
Q

Management of calcinosis in SSc

A

Diltiazem

94
Q

Management of Raynauds

A

Avoid cold, stress, nicotine, caffeine and vasoconstrictor drugs
Nifedipine or amlodipine
Then add transdermal nitroglycerin or phosphodiesterase inhibitors (sidenafil)

95
Q

Management of pulmonary hypertension in SSc

A

Bosentan or sildenafil

96
Q

Renal disease in SSc management

A

ACEi

97
Q

Prognosis of SSc

A

variable

5 year survival 35-75%

98
Q

Primary cause of scleroderma related deaths

A

Lung (pulmonary hypertension or fibrosis

Ortho and Rheum (13 decks)

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