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Ortho and Rheum > Children Rheum - MSK, PMR/GCA and SLE > Flashcards

Children Rheum - MSK, PMR/GCA and SLE Flashcards

1
Q

What can cause MSK pain in children?

A 
Can be serious lifethreatening conditions such as malignancy, sepsis, vasculitis, and NAI 
Also rheumatic disorders 
IBD, cystic fibrosis 
Ortho syndromes
etc etc
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2
Q

Localised pain in a well child - possible DDX x5

A 
Strains and sprains
Bone tumours
JIA (oligoarthritis)
Localised idiopathic pain syndromes
Growing pains
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3
Q

Localised pain in an unwell child - DDX x2

A

Septic arthritis

Osteomyelitis

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4
Q

Diffuse pain in a well child - DDX x2

A

Hypermobility

Diffuse idiopathic pain syndromes

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5
Q

Diffuse pain in an unwell child x6

A 
Leukaemia 
Neuroblastoma 
JIA (SA and PA)
SLE 
Juvenile dermatomyositis
Vasculitis
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6
Q

Red flags in children with back pain x3

A

Painful scoliosis
Neurological symptoms suggestive of nerve root entrapment or cord compression
Systemic findings suggesting malignancy or sepsis

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7
Q

What can inflammatory back pain in children be a sign of?

A

Late feature of enthesitis-related arthritis
Often presents in late adolescence
Strong HLA-B27 expression association

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8
Q

What is osteochondrosis?

A

Developmental derangement of normal bone growth

Aseptic ischaemic necrosis

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9
Q

What is osteochondritis?

A

Inflammation of the cartilage or bone of a joint

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10
Q

What is Osgood-Schlatter disease?

A

One of commonest causes of knee pain in adolescents - overuse injury causing inflammation (osteochondritis) of the tibial tubercle causing swelling just below the knee

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11
Q

What is Sever’s disease

A

Calcaneal apophysitis
Inflammation of growth plate in the heel of growing children
Especially adolescents

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12
Q

What are non-benign causes of hypermobility?

A

Marfans syndrome
Sticklers syndrome
Ehlers-Danlos syndrome

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13
Q

What is arthritis of SLE like?

A

Usually polyarticular, non-deforming and non-erosive

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14
Q

Presentation of juvenile dermatomyositis?

A

May present at any age with characteristic skin involvement and proximal muscle weakness

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15
Q

What are severe complications of juvenile dermatomyositis?

A

Risk of aspiration pneumonia and interstitial lung disease

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16
Q

Treatment of Juvenile dermatomyositis

A

High dose corticosteroids with methotrexate

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17
Q

What is scleroderma

A 
Systemic sclerosis 
Caused by immune system attacking connective tissue around skin, internal organs and blood vessels 
Causes hardening (sclero) of the skin (derma)
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18
Q

What is the most common presentation of scleroderma in children

A

Localised is most common - can present at any age
Patches of abnormal skin
If untreated then will have active expanding disease, fibrosis and eventual softening with some remission

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19
Q

What can the implications of scleroderma be in children?

A

Cosmetic or functional if interfere with growth of a limb and SC tissues

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20
Q

Treatment of scleroderma in children?

A

Aggressive treatment regimens - corticosteroids and methotrexate - to control disease and limit severe disfigurement and disability

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21
Q

What type of scleroderma is rare in children

A

Systemic scleroderma

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22
Q

What organs are affected in systemic scleroderma

A

Lungs, GI tract, heart and kidneys

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23
Q

Common childhood vasculitides x2

A

HSP

Kawasaki disease

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24
Q

MSK features of vasculitides

A

HSP and Kawasaki often have transient arthritis affecting large joints

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25
Q

Features of HSP

A 
Palpable purpura over legs and buttocks 
Abdominal pain 
Haematuria 
Arthritis 
Usually resolves completely within 4 weeks
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26
Q

What is Chronic recurrent multifocal osteomyelitis

A

Presents similarly to bacterial osteomyelitis
Often multiple sites with recurrent episodes
Bone pain, sometimes swelling too
Most often long bones but ribs, clavicle, vertebrae or mandible can be affected

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27
Q

Treatment of CRMO

A

NSAIDs

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28
Q

What is polymyalgia rheuamatica?

A

Clinical syndrome that affects older patients

Proximal muscle stiffness, especially in the shoulders and systemic features such as fatigue, weight loss and depression

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29
Q

What is Giant Cell arteritis?

A

Systemic vasculitis affects large and medium sized arteries
May involve any artery but propensity to affect branches of external carotid, particularly posterior ciliary arteries (optic nerve) and temporal artery

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30
Q

Why are GCA and PMR related

A

Clinical and pathogenetic links between the two - therefore idea is that they are manifestations on a disease spectrum that affects same disease population

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31
Q

Frequency of GCA and PMR occurring together?

A

PMR has been observed in 40-60% of cases of GCA

GCA been observed in 30-80% of patients with PMR

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32
Q

Environmental factors for PMR and GCA?

A

Acute-onset prodromal events suggest infectious trigger
Possible relationship to mycoplasma pneumoniae, chlamydia pneumoniae , parvovirus B19, respiratory syncytial virus and adenovirus
BUT no causative agent has been found

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33
Q

Genetic influences for PMR and GCA

A

Possible role for HLA-DR4

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34
Q

Gender ratio of PMR and GCA

A

Women:men = 3:1

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35
Q

Age of PMR and GCA patients

A

Rare under age 50

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36
Q

Ethinicity of PMR and GCA patients

A

Mostly northern european patients but can occur in any ethnic group

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37
Q

Diagnosis of PMR and GCA

A

Based on clinical features as there is no diagnostic test
ESR and CRP usually elevated
Can do temporal artery biopsy in GCA

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38
Q

Clinical features of PMR x7

A 
Bilateral shoulder pain, stiffness NOT MUSCLE WEAKNESS
Duration onset 40 
Stiffness >1 hour 
Age >50, typically >65
Depression or weight loss
Bilateral upper arm tenderness
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39
Q

Diagnostic criteria for PMR

A

Probable if 3 or more clinical features or definite if probable PMR responding to corticosteroids

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40
Q

Clinical features of GCA x5

A 
Age >50 
New headache
Temporal artery abnormal to palpation 
Elevated ESR 
Abnormal findings on temporal artery biopsy
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41
Q

Muscle symptoms in PMR

A

Sometimes muscles of upper arms and thighs are tender to palpation
Muscle strength usually unimpaired
Patients may have difficulty turning over in bed, especially early in the morning
May be hard to lift heavy objects, walk upstairs or tender to sit on toilet

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42
Q

Clinical features of GCA x4

A

Scalp is tender to touch
May hurt to wear glasses
Jaw claudication when chewing
Artery features depends on stage, early may be bounding with full pulse but also tenderness
Later fibrosis and repair may predominate and pulse is almost absent with nodular feeling to artery

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43
Q

Eye involvement in GCA

A

Can get diplopia, partial or complete loss of vision and cranial nerve palsy if left untreated

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44
Q

Late complications of GCA

A

If large-vessel involvement occurs

Aortic aneurysms and stenosis

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45
Q

Histopathology of GCA - what sort of lesions are they?

A

Skip lesions - patchy or segmental involvement of arteries

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46
Q

Histopathology of GCA - what is pathology?

A

T-cell CD4 proliferation by unknown antigen in aventitia - CD4 lymphocytes produce interferon, attracts macrophages to arterial wall -they fuse to form multinucleated giant cells in intima-media junction - these cells produce adhesion molecules, nitric oxide and collagenases which cause tissue injury and in situ thrombosis

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47
Q

Histopathology of PMR

A

Similar to GCA

Synovitis which is a non-erosive self-limiting arthritis in 1/3 of patients

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48
Q

Muscles in PMR

A

Muscle does not seem to be considered site of pathology, muscle enzymes and biopsies are normal - although changes have been noted in ultrastructure and mitochondria

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49
Q

Investigations in PMR and GCA - biological

A

ESR and CRP are most frequently raised although both can occur with normal ESR
Both fall with effective treatment - CRP falls faster
Also normocytic normochromic anaemia

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50
Q

What will US and MRI in GCA show?

A

A “halo” around inflamed vessels and maybe even the caliber of the temporal arteries

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51
Q

If untreated what happens to PMR and GCA

A

Will burn out after a mean of 2 years (range 6 months to 10 years)
Long-term vascular complications in GCA common

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52
Q

Treatment of PMR

A

Corticosteroid - prednisolone 10-20mg daily for 1 month
Reduce dose after month by 2.5mg every 2-4weeks until 10mg daily
At 6 months reduce more
Titrate against clinical response
Most require treatment for 3 years but can be less

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53
Q

Treatment of GCA

A

Prednisolone - higher dose than for PMR and treatment for longer
eg. if risk of blindness 80mg daily
Again reduce dose steadily - maintenance at low dose likely for up to 5 years
Can also add low-dose aspirin

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54
Q

What is common when treating GCA and PMR

A

Relapses when reducing corticosteroids

55
Q

What is Systemic Lupus Erythematous?

A

A multi-system autoimmune disease of unknown cause with a wide variety of manifestations - usually characterised by remissions and relapses

56
Q

What diseases are included within the SLE spectrum? x7

A 
Discoid Lupus
Drug-induced Lupus
Neonatal lupus
Sjogrens syndrome
Antiphospholipid antibody syndrome 
Dermatomyositis/polymyositis
Overlap syndromes
57
Q

When can antiphospholipid antibody syndrome occur?

A

May occur as a primary disorder or secondary to autoimmue disease such as SLE, autoimmune hypothyroidism or chronic active hepatitis

58
Q

Pathology of SLE

A

Dendritic, B-cell and T-cell dysfunction resulting in development of autoantibodies and autoreactive T cells
Defective clearance of apoptotic cells and immune complexes
Activation of complement, cytokines, interleukins etc

59
Q

Interleukins involved in SLE and role

A

Interleukin-6, Il-10

Their levels are increased in active disease

60
Q

Cytokine involved in SLE and role

A

Interferon-alpha

Plays a role in activating genes involved in the disease

61
Q

Who is burden of SLE disease highest in? x2

A

Women and non-white ethnic groups

62
Q

Criteria for SLE x11

A 
Malar rash
Discoid rash
Photosensitivity 
Mucosal ulcers
Arthritis (non-erosive)
Serositis = pleuritis or pericarditis
Neurological disorder (psychosis, seizures)
Renal disorder 
Haematological disorder
Immunological disorder
Positive antinuclear antibodies
(Diagnosis with 4 out of 11)
63
Q

What general features are common with SLE x2

A

Fatigue is common
May be associated with secondary depression or fibromyalgia (due to SLE, hypothyroidism, anaemia, pulmonary or CV symptoms)

64
Q

Symptoms of active SLE disease x5

A 
Fever
Malaise
Anorexia
Weight loss
Lymphadenopathy
65
Q

What are the most common mucocutaneous features of SLE? x 4

A

Painful/painless mouth ulcers
Diffuse alopecia
Butterfly/malar rash
Photosensitivity

66
Q

Other mucocutaneous features of SLE?

A

Nasal/vaginal ulcers
Subacute cutaneous lupus erythematous rash
Discoid lesions

67
Q

In whom are mucocutaneous features more common with SLE?

A

White and Asian patients

68
Q

What is subacute cutaneous lupus erythematous?

A

Non-scarring rash found in areas of the body exposed to sunlight (photosensitive dermatosis)

69
Q

What is discoid lupus?

A

Discoid lesions which are chronic scarring lesions that heal with hypo or hyperpigmentation

70
Q

What are the joint features of SLE? x2

A

Generalised non-swelling, early morning stiffness arthralgia - very common.
Non-erosive arthritis with swelling may develop

71
Q

What sort of joint deformities occur with SLE?

A

Deformities are unusual but may occur due to ligamentous laxity rather than due to joint erosions as with RA

72
Q

Muscle features of SLE

A

Myalgia is common
Myositis only occurs in 5% of patients
Secondary causes of myopathy eg. steroids - more common

73
Q

What MSK risk is increased in SLE?

A

Increased risk of osteoporosis and therefore fractures due to minimal trauma

74
Q

What is the most common haematological manifestation in SLE?

A

Leucopenia and lymphopenia caused by lymphotoxic antibodies

75
Q

What is the second most common haematological manifestation of SLE? - Causes x4

A

Anaemia - may be multifactorial 1) Normochromic, normocytic of normal disease

2) Antibody-mediated haemolytic anaemia
3) Iron-deficiency anaemia due to dietary deficiency or blood loss (GI secondary to NSAIDs)
4) Pernicious anaemia

76
Q

What is 3rd haematological manifestation of SLE?

A

Thrombocytopenia - may occur as immune-mediated condition.

Risk of bleeding

77
Q

Incidence of renal disease in SLE?

A

Common - can occur in up to 50% in white patients and 75% of black patients

78
Q

In whom is SLE renal disease more severe?

A

Non-white patients

79
Q

Presentation of SLE renal disease? x3

A

Early nephritis picked up on urinalysis
Nephrotic syndrome
Few with devastating accelerated hypertension and renal shutdown

80
Q

Most common neurological manifestations of SLE? x4

A

Headache, seizures, aseptic meningitis, cerebrovascular accidents
(but many more can occur)

81
Q

What neurological symptom can occur secondary to SLE but also secondary to steroid treatment?

A

Psychosis

82
Q

Common pulmonary manifestation of SLE?

A

Pleurisy often without physical signs

83
Q

Other pulmonary manifestations of SLE? x4

A

Lupus pneumonitis
Pulmonary haemorrhage (sudden, acute & high mortality)
Pulmonary embolism
Pulmonary hypertension (poor prognosis)

84
Q

Common cardiac manifestation of SLE?

A

Pericarditis but often asymptomatic

85
Q

Other cardiac manifestations of SLE? x3

A

Myocarditis
Endocarditis
Rarely - cardiac tamponade

86
Q

Common CVD in SLE?

A

Premature atherosclerosis is common - risk for MI due to coronary artery disease

87
Q

Common GI manifestations of SLE and incidence?

A

50% of SLE patients experience abdominal pain, n and v and diarrhoea at some stage of disease

88
Q

Cause of acute abdomen in SLE?

A

Mesenteric vasculitis - high risk of death

89
Q

SLE and fertility?

A

No evidence that SLE decreases fertility

90
Q

SLE and pregnancy?

A

If antiphospholipid syndrome then increased risk of IUGR, premature delivery, miscarriages and stillbirth

91
Q

SLE treatment and pregnancy?

A

Doses of prednisolone >10mg/day increased risk of pre-eclampsia, isolated HTN, PROM, maternal infection

92
Q

What SLE medication should be stopped in pregnancy?

A

ACEi or mycophenolate mofetil for renal disease

93
Q

What is neonatal lupus syndrome?

A

Occurs in 10% of babies born to mothers with anti-Ro or anti-La antibodies
Rash induced by UV light a few days after birth - resolves if removed from sunlight or UV light

94
Q

What is a more serious version of neonatal lupus syndrome?

A

Much rarer - congenital heart block

Usually detected in utero about 16-28 weeks into pregnancy

95
Q

What is Sjogren’s syndrome?

A

Clinical syndrome characterised by sicca (dryness) symptoms: dry eyes and dry mouth due to failure of salivary and mucosal glands
Often preceded by salivary gland swelling

96
Q

When does Sjogrens occur as secondary disease?

A

May occur secondary in SLE, RA, systemic sclerosis, primary biliary cirrhosis

97
Q

What is primary Sjogrens syndrome?

A

Occurs with features that resemble mild SLE (mild symmetrical arthritis, photosensitivity, fatigue and diffuse alopecia) Also dry upper airways (uncommon - dry vagina and dry skin/hair)

98
Q

Biological features of primary Sjogrens syndrome?

A

Associated with hypergammaglobulinaemia ( IgG levels high)
Positive antinuclear antibodies, rheumatoid factor, anti-Ro and anti-La
Raised amylase if salivary glands are involved

99
Q

Diagnostic criteria for polymyositis? x4

A

Proximal muscle weakness, elevated muscle enzymes, myopathic changes on electromyography, inflammatory changes on muscle biopsy

100
Q

Diagnostic criteria for dermatomyositis?

A

Features of polymyositis (proximal muscle weakness, elevated muscle enzymes, myopathic changes on electromyography, inflammatory changes on biopsy) + characteristic rash

101
Q

What does antiphospholipid syndrome cause? x3

A

Recurrent arterial and venous thrombosis and miscarriages

102
Q

Most common presentation of anti-phospholipid syndrome?

A

Venous thrombosis in arms or legs
Recurrent, multiple and bilateral
Propensity for PE

103
Q

Most common site of arterial thrombosis in anti-phospholipid syndrome?

A

Brain - therefore as stroke or TIA

104
Q

Antiphospholipid syndrome and pregnancy?

A

IUGR due to placental insufficiency and pre-eclampsia
Recurrent losses in 2nd or 3rd trimester
Premature birth therefore plan early delivery

105
Q

Prognosis of SLE and antiphospholipid syndrome?

A

Survival has improved but patients still succumb to late complications of disease or its therapy

106
Q

Contributors to mortality in SLE and antiphospholipid syndrome x5

A

Hyperlipidaemia, hypertension, premature IHD, DM and osteoporotic fractures

107
Q

Investigations for diagnosis and monitoring in SLE

A

FBC with WCC, urinalysis and serum creatinine (detect renal disease - also GFR)

108
Q

Diagnostic tests for SLE

A

ANA

anti-extractable nuclear antigen tests

109
Q

Test to predict risk of developing of renal disease in SLE?

A

Anti-ds DNA antibodies

110
Q

Test which falls with SLE disease activity

A

C3 and C4 - because of complement consumption

111
Q

SLE tests which should be done in women planning pregnancy

A

Anti-Ro, Anti-La and Antiphospholipid antibodies

112
Q

Investigations in antiphospholipid syndrome?

A

80-90% of patients are positive for ab’s to a complex of anticardiolipin antibodies and B2-glycoprotein

113
Q

Management of SLE patients with sun-reactive dermatological problems?

A

Wear sunblock for >6 months of the year

114
Q

What contraception should be used in SLE?

A

Contraceptive pills that contain oestrogen can exacerbate disease therefore barrier methods or progesteron only methods are preferred

115
Q

Treatment of mild SLE

A

Steroid creams, short NSAIDs courses and hydroxycholoroquine

116
Q

Treatment of severe SLE

A

Oral corticosteroids + hydroxycholoroquine

117
Q

What might be needed in very severe SLE

A

IF need >10mg a day of pred + hydroxycholoroquine

Might need azathioprine, cyclosporin, methotrexate or cyclophosphamide

118
Q

Lupus nephritis treatment

A

Mycophenolate mofetil

119
Q

Treatment of SLE in pregnancy? x3

A

Prednisolone
Hydroxycholoroquine
Azathioprine

120
Q

Treatment of SLE during lactation?

A

Pred and hydroxycholoquine

Aza at low doses

121
Q

Contraindication SLE treatment in pregnancy and breast feeding? x4

A

Methotrexate
Mycophenolate
Lefluonamide
Cyclophosphamide

122
Q

Treatment of immunosuppressant resistant SLE x4

A

Cyclosporin A
Tacrolimus
Lefluonamide

123
Q

Preferred anti-hypertensives in SLE x3 and why?

A

Calcium-channel blockers
ACE inhibitors
ARBs
Helpful in management of Raynauds whereas B-blockers make it worse

124
Q

Treatment in antiphospholipid syndrome

A

Aspirin
Avoid oestrogen-containing contraceptives and HRT
Heparin to treat thrombosis, then warfarin (INR aim 2-3 for venous and 3-4 for arterial)

125
Q

Treatment of antiphospholipid syndrome in pregnancy

A

LMWH and low-dose aspirin

126
Q

Pathology behind gland failure in Sjogrens

A

Inflammation and subsequent destruction

127
Q

HLA linked to Sjogrens

A

HLA-B8 and DR3

128
Q

Gender of Sjogrens

A

More common in women than men 9:1

129
Q

Investigations in Sjogrens

A

Raised ESR and amylase

130
Q

What is Shirmers test

A

Test for lacrimal gland dysfunction in sjogrens - filter paper under eyelid and +ve test if less than 10mm wet after 5mins

131
Q

Treatment of sjogrens

A

steroids or immunosuppresants

Rituximab shown to be effective

132
Q

Complications of sjogrens

A

Vasculitis, interstitial nephritis

Lymphoma (usually non-hodgkin b-cell type)

133
Q

Prognosis of SLE

A

10 years is 80-90%

Mortality due to infection or renal failure

Ortho and Rheum (13 decks)

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