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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
What is anti-scl70 autoantibody related to in SSc?
Lung fibrosis
26
Treatment of lung fibrosis in SSc?
Immunosuppressive treatment with cyclophosphamide has been shown to have modest effect Better treatments are needed
27
Diagnosis of lung fibrosis in SSc?
HRCT - high resolution computed tomography
28
How does scleroderma renal crisis present?
Often with headaches and visual disturbances before full-blown encephalopathy, cardiac failure or acute oliguric renal failure develop
29
Treatment of scleroderma renal crisis
ACEi | 50% require dialysis which is often temporary
30
What is the most affected organ in SSc?
GIT
31
Most common presentation of GIT disturbances in SSc?
Oesophageal dysmotility with reflux - treat with PPI
32
What is fibromyalgia syndrome?
Widespread musculoskeletal pain and hyperalgesic tender spots with no single identifiable organic cause
33
What is a more mild form of fibromyalgia on the same spectrum?
Chronic widespread pain
34
What other symptoms are present with fibromyalgia?
Stiffness, fatigue, sleep disturbance as well as some other physical and psychological symptoms
35
Prevalence of fibromyalgia
2%, chronic widespread pain is 12%
36
General criteria for fibromyalgia
Widespread pain in all 4 quadrants of the body Present for at least 3 months Hyperalgesic points, bilatearlly
37
Where are the hyperalgesic points in fibromyalgia? x9
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
38
Investigations in fibromyalgia
To exclude other DDX - TFTs, FBC, inflammatory markers, serum calcium and ALP, CK, BG No diagnostic test
39
Typical clinical picture for fibromyalgia
Female, aged 30-50, with long standing diffuse pain History of physical or psychological trauma Symptoms exacerbated by other stressors in her life
40
What is cause of fibromyalgia
No cause been found - likely to be multifactorial characterised by abnormal processing of pain = central sensitisation
41
Management of fibromyalgia
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
What is graded exercise in management of fibromyalgia?
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
Drug therapy in fibromyalgia x5
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
What is osteoporosis?
Systemic skeletal disease characterised by low bone mass and micro-architectural deterioration of bone tissue - resulting in high risk of fracture
45
Pathophysiology of osteoporosis
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
Which are the classical osteoporotic fractures?
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
Two classifications of osteoporosis
Primary - including post-menopausal and age-related | Secondary - where bone loss is accelerated by presence of underlying disease
48
What % of osteoporosis is primary and what % is secondary in each gender?
40% of osteoporosis is secondary in women | 60% of osteoporosis is secondary in men
49
Diagnosis of osteoporosis according to BMD on DXA scan
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
Endocrine causes of secondary osteoporosis x5
``` Thyrotoxicosis Primary hyperparathyroidism Cushing's syndrome Hypogonadism (including Anorexia) T1DM ```
51
GI causes of secondary osteoporosis x3
Malabsorption syndrome IBD Liver disease eg. primary biliary cirrhosis
52
Rheumatological causes of secondary osteoporosis x2
RA | Ankylosing spondylitis
53
Malignancy causes of secondary osteoporosis x2
Multiple myeloma | Cancer treatment induced bone loss
54
Drug causes of secondary osteoporosis x5
Glucocorticoids, Anticonvulsants, Heparin Aromatase inhibitors (breast and ovarian cancer ttt) Androgen-deprivation therapy (prostate cancer ttt, decreases testosterone)
55
Factors included in FRAX tool x12
Age, sex, weight, height, previous fracture, parent hip fracture, smoking, alcohol >3units/day, glucocorticoids, RA, secondary osteoporosis, femoral neck BMD
56
What does FRAX tool calculate?
Risk of major osteoporotic fracture (hip, wrist, proximal humerus or spine) and risk of hip fracture - 10 year probability
57
What are 3 major determinants of persons risk of fracture?
Age Prior fragility fracture (spinal radiographs can pick these asymptomatic fractures up) BMD
58
What are bisphosphonates?
Antiresorptive agents
59
Once-weekly bisphosphonates x2
Alendronate (1st line) and risedronate
60
What is Ibandronate
Once-monthly bisphosphonate or 3-monthly IV infusion
61
What is Zoledronate
once-yearly short infusion bisphosphonate
62
How do bisphosphonates need to be taken?
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
When is HRT used in osteoporosis?
Now only thought to be appropriate in women under 50 who have undergone early menopause or to control climacteric symptoms
64
What is Raloxifene
Selective oestrogen receptor modulator - anti-resorptive Without effect on breast and endometrial tissue Still small increased risk of thromboembolic events though
65
What are two nutritional treatments for osteoporsis and how they work?
Calcium (adherence can be problematic) Vitamin D (given to all housebound or in resident care elderly patients) Anti-resorptive agents
66
What does calcitonin do?
Reduce risk of vertebral fracture | Also has analgesic properties
67
What are teriparatide and parathyroid hormone?
Formation-stimulating agents | Can increase bone formation - especially trabecular bone
68
When are teriparatide and parathyroid hormone used?
Very expensive therefore only used in patients with severe progressive osteoporosis despite treatment with anti-resorptive agents
69
In what type of osteoporosis is teriparatide licensed?
Glucocorticoid-induced osteoporsis - because been shown to increase spine and hip BMD greater than alendronate
70
Pain relief in osteoporosis? x4
Analgesics Hydrotherapy Transcutaneous nerve stimulators Low-dose antidepressants
71
What is sarcoidosis?
Multisystem granulomatous inflammatory disorder
72
Aetiology of sarcoidosis
Unknown
73
Epidemiology of sarcoidosis
Uncommon, more common in 20-40 and in Africans and females
74
Which glands swell in sarcoidosis
Parotid glands
75
Type of cough in sarcoidosis
Usually unproductive
76
What happens in bones in sarcoid
Bone cysts eg. dactylitis in phalanges
77
Eye symptoms in sarcoid
Keratoconjuncitivitis sicca (dry eyes), papilloedema, uveitis
78
Skin in sarcoid
``` Lupus pernio (red-blue infiltrations of nose, cheek, ears, terminal phalanges) Erythema nodosum Maculopapular eruptions ```
79
Pathology of sarcoidosis
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
What is raised in serum in sarcoid
Serum ACE and Calcium
81
What will show in 24h urine collection in sarcoid
Hypercalciuria
82
CXR in sarcoid
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
Type of CT in sarcoid
High resolution CT showing diffuse lung involvement
84
Type of scan to show in the inflammation in sarcoid
Gallium 67 scan - shows the classic parotid and eye swelling
85
Pulmonary function tests in sarcoid
Reduced FEV1, FVC and gas transfer - showing a restrictive pattern
86
What is seen on bronchoscopy and bronchoalveolar lavage
Increased lymphocytes with increased CD4:CD8 ratio
87
What is seen on transbronchial lung biopsy or lymph node biopsy
Non-caseating granulomas composed of epithelial cells (activated macrophages) multinucleate Langhans cells and mononuclear cells (lymphocytes)
88
Management of sarcoid
Corticosteroids for symptomatic pulmonary, cardiac and neurologic sarcoidosis
89
MSK sarcoid management
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
Eye management in sarcoid
Mydriatic (atropine) | Topical corticosteroids
91
Lupus pernio treatment
Chloroquine or methotrexate
92
What is CREST
``` What limited systemic sclerosis used to be known as Calcinosis Raynauds Esophageal dysmotility Sclerodactyly Telangectasia ```
93
Management of calcinosis in SSc
Diltiazem
94
Management of Raynauds
Avoid cold, stress, nicotine, caffeine and vasoconstrictor drugs Nifedipine or amlodipine Then add transdermal nitroglycerin or phosphodiesterase inhibitors (sidenafil)
95
Management of pulmonary hypertension in SSc
Bosentan or sildenafil
96
Renal disease in SSc management
ACEi
97
Prognosis of SSc
variable | 5 year survival 35-75%
98
Primary cause of scleroderma related deaths
Lung (pulmonary hypertension or fibrosis

Ortho and Rheum (13 decks)

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