Rheum Flashcards

1
Q

What is Paget’s Disease of the Bone?

A

excessive bone turnover (reabsorption and formation) due to increased osteoclast and osteoblast activity.
Turnover is not coordinated, so get patchy (high density) sclerosis and (low density) lysis

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2
Q

Paget’s effect on bones

A
  • Enlarged and misshapen bones
  • Structural problems
  • Increased risk of pathological fractures
  • Particularly affects the axial skeleton (the bones of the head and spine).
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3
Q

Presentation of Paget’s

A

asymptomatic (incidental on xray) or:
- Bone pain
- Bone deformity
- Fractures
- Hearing loss

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4
Q

Paget’s Ix

A

X-ray findings include:
- Bone enlargement and deformity
- Osteoporosis circumscripta
- Cotton wool appearance of the skull
- V-shaped osteolytic defects in the long bones

Blood tests include:
- Raised ALP (needs monitoring)
- Normal calcium
- Normal phosphate

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5
Q

Paget’s Mx

A

Bisphosphonates
- calcitonin if can’t have bisphosphonates
- NSAIDs for bone pain
- calcium and vit D

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6
Q

Paget’s complications

A
  • Hearing loss (if it affects the bones of the ear)
  • Heart failure (due to hypervascularity of the abnormal bone)
  • Osteosarcoma (rare but poor prognosis)
  • Spinal stenosis and spinal cord compression
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7
Q

Define osteomalacia

A

soft bones due to insufficient vit D
low Vit D = low calcium and phosphate
low calcium = high PTH which increases calcium resorption from bones

RF- dark skin, low sunlight exposure, colder climate

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8
Q

Osteomalacia presentation

A

May be asymptomatic
Fatigue
Bone pain
Muscle weakness
Muscle aches
Pathological or abnormal fractures
Looser zones (fragility fractures partially through bone)

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9
Q

Osteomalacia Ix

A
  • serum 25 hydroxyvitamin D (<25= deficiency, 25-50= insufficiency)
  • low serum calcium
  • low serum phosphate
  • high ALP
  • high PTH
  • Xray: osteopenia (radiolucent bones)
  • DEXA: low bone mineral density
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10
Q

Osteomalacia Mx

A

colecalciferol (vit D)
loading dose 50,000 IU once a week for 6 weeks or 4000 IU daily for 10 weeks.
then maintenance dose of 800-2000

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11
Q

Define Osteoporosis

A

reduction in bone density
DEXA <-2.5
-1 to -2.5 = osteopenia
DEXA on femoral neck most important to check

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12
Q

Osteporosis RFs

A
  • older age
  • post-menopausal
  • reduced mobility
  • low BMI
  • low calcium or vit D intake
  • alcohol and smoking
  • long term steroid use (7.5mg and above)
  • fracture hx
  • CKD, hyperthyroidism, RA
  • SSRI, PPI, anti-epileptic, anti-oestrogen
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13
Q

Osteoporosis Ix

A

QFracture tool
FRAX tool
measures 10 year risk of fracture
above 10%= do DEXA

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14
Q

Osteoporosis Mx

A
  • stop smoking and alcohol
  • exercise
  • calcium
  • Vitamin D
  • Bisphosphonates (take on empty stomach to reduce reflux)
  • denosumab
  • HRT
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15
Q

Side effects of bisphosphonates

A
  • reflux and oesophageal erosions
  • atypical fractures
  • osteonecrosis of the jaw
  • osteonecrosis of external auditory canal
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16
Q

Define Gout

A

crystal arthropathy
high uric acid
raised serum urate level
joint aspiration: monosodium urate crystals, needle shaped, negative bifringent)
Xray- maintained joint space, lytic lesions in bone, punched out eosions

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17
Q

Gout presentation

A
  • hot swollen and tender joint
  • gouty tophi in hands elbows and ears (subcut uric acid deposit)
  • base of big toe (MTP joint)
  • base of thumb (CMC joint)
    wrist
  • larger joint (knee/ankle)
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18
Q

Gout RF

A

male
FH
alcohol
obesity
high purine diet (meat/seafood)
diuretic
CVD
Kidney disease

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19
Q

Difference between gout and pseudogout

A

Monosodium urate crystals of gout are needle-shaped and negatively birefringent of polarised light.
The calcium pyrophosphate crystals of pseudogout are rhomboid-shaped and positively birefringent.

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20
Q

Gout Mx

A

acute:
naproxen
colchicine
prednisolone

Chronic
allopurinol
febuxostat

  • lose weight and hydrate
  • no alcohol and purines
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21
Q

Define pseudogout

A

calcium pyrophosphate crystals

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22
Q

Pseudogout presentation

A

Many asymptomatic
chronic pain and stiffness in joints
>65 y/o
hot swollen stiff and painful knee
or shoulders, hips, wrists

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23
Q

Diagnosing pseudogout

A

Need joint aspirate to exclude septic arthritis: rhomboid positive birefringent
Xray: chrondrocalcinosis - calcium deposits in joint cartilage.
Similar xray changes to OA
L: LOJS
O: osteophytes
S: subarticular sclerosis (increased bone density on joint line)
S: subchondral cysts (fluid filled holes in bone)

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24
Q

Pseudogout Mx

A

resolve spontaneously
symptomatic mx:
NSAID
colchicine
joint steroid injection
oral steroids

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25
Q

Define Ehlers-Danlos Syndrome

A

Genetic conditions involving collagen defects
Causes hypermobility and connective tissue abnormality of the skin, bones etc.

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26
Q

Types of EDS

A

Hypermobile EDS
Classical EDS
Vascular EDS
Kyphoscoliotic EDS

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27
Q

what is hypermobile EDS

A

most common
least severe
joint hypermobility and stretchy skin
autosomal dominant

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28
Q

what is classical EDS

A

stretchy skin that feels smooth and velvety
severe hypermobility, joint pain and abnormal wound healing
lumps over pressure points
prone to hernias, prolapses, MR and aortic root dilatation
autosomal dominant

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29
Q

What is vascular EDS

A

most severe and dangerous
blood vessels prone to rupture
thin translucent skin
gastrointestinal perforation
spontaneous pneumothorax
autosomal dominant

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30
Q

Kyphoscoliotic EDS

A

hypotonia as neonate/infant
kyphoscoliosis as they grow
joint hypermobility
joint dislocation
autosomal recessive

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31
Q

What can occur with HEDS

A

postural orthostatic tachycardia syndrome (tachy on sitting/standing)

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32
Q

how to score hypermobility

A

Beighton score

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33
Q

EDS Mx

A

no cure
physio to stabilise the joints
OT

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34
Q

What is Behcet’s disease

A

affects BVs and tissues
recurrent oral and genital ulcers >3x a year (red halo)
can affect eye, skin, GIT, lungs, MSK and CNS
HLA B51 gene

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35
Q

Signs of Behcet’s disease

A
  • halo red ulcer
  • erythema nodosum
  • anterior uveitis
  • arthralgia
  • ulceration of ileum, caecum
  • aseptic meningitis
  • aneurysms
  • DVT and Budd-chiari syndrome
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36
Q

Test for Behcet’s

A

pathergy test
positive test indicates Behcet’s, sweet’s syndrome or pyoderma gangrenosum

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37
Q

Behcet’s disease Mx

A

topical steroids for ulcers
prednisolone
colchicine
topical anaesthetics
azathioprine
biologic: infliximab

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38
Q

Behcet prognosis

A

relapsing-remitting
normal LE

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39
Q

Define vasculitis

A

inflammation of BVs

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40
Q

Types of vasculitis

A

Henoch-Schonlein Purpura
Microscopic Polyangiitis
Granulomatosis with Polyangiitis
Eosinophilic Granulomatosis with Polyangiitis
Polyarteritis Nodosa
Kawasaki Disease
Giant Cell Arteritis
Takayasu’s Arteritis

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41
Q

HSP features

A
  • Small Vessel
  • Purpura (non-blanching rash)
  • IgA nephritis (renal involvement)
  • children
  • joint and abdo pain

Mx- supportive

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42
Q

Microscopic Polyangiitis features

A
  • Small vessel
  • p-ANCA
  • Glomerulonephritis causing renal failure
  • Diffuse alveolar haemorrhage
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43
Q

Granulomatosis with Polyangiitis features

A
  • Small vessel
  • c-ANCA
  • Nasal symptoms
  • Respiratory symptoms
  • Glomerulonephritis
  • saddle shaped nose due to bridge collapse
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44
Q

Eosinophilic Granulomatosis with Polyangiitis features

A
  • Small vessel
  • p-ANCA
  • Raised eosinophils
  • Late-onset severe asthma
  • Sinusitis and rhinitis
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45
Q

Polyarteritis Nodosa features

A
  • Medium vessel
  • idiopathic or due to Hep B
  • Renal impairment
  • Hypertension
  • Cardiovascular events
  • Tender skin nodules
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46
Q

Kawasaki Disease features

A
  • Medium vessels
  • children <5
  • High fever (more than 5 days)
  • Widespread rash
  • Bilateral conjunctivitis
  • Strawberry tongue
  • Desquamation of palms and soles
  • Coronary artery aneurysms is a complication

Mx- aspirin and IVIG

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47
Q

Giant Cell Arteritis features

A
  • Large vessels
  • Raised ESR
  • Unilateral headache
  • Scalp tenderness
  • Vision loss

Mx steroids

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48
Q

Takayasu’s Arteritis features

A
  • Large vessel
  • Aortic arch affected
  • “Pulseless” disease
  • claudication sx

Need CT/MRI angio to diagnose

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49
Q

General vasculitis features

A

Joint and muscle pain
Peripheral neuropathy
Renal impairment
Purpura (purple-coloured non-blanching spots caused by blood leaking from the vessels under the skin)
Necrotic skin ulcers
Gastrointestinal symptoms (e.g., diarrhoea, abdominal pain and bleeding)

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50
Q

Vasculitis Ix

A

inflammatory markers (CRP ESR)
ANCA (p and c)

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51
Q

Vasculitis Mx

A

steroids

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52
Q

Define Sjogren’s syndrome

A

autoimmune condition affecting exocrine glands (lacrimal and salivary)

Primary- in isolation
Secondary- due to SLE or RA

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53
Q

Sx of Sjogren’s

A

dry mouth, eyes and vagina (sicca sx)
dry skin
joint pain and stiffness
women
middle age

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54
Q

Antibodies in sjogren’s

A

anti-Ro
anti-La

55
Q

Mx of Sjogren’s

A

eye drops
artificial saliva
vaginal lubricants
pilocarpine to stimulate tear/saliva
hydroxychloroquine

56
Q

Define Antiphospholipid Syndrome

A

autoimmune caused by antiphospholipid abs
cause inflammation and increase thrombosis risk

57
Q

Name the different antiphospholipid antibodies

A

lupus anticoagulant
anticardiolipin antibodies
anti-beta 2 glycoprotein I antibodies

58
Q

Mx antiphospholipid syndrome

A

primary thromboprophylaxis- aspirin
long term warfarin INR 2-3
LMWH and aspirin used in pregnancy

59
Q

Define polymyositis and dermatomyositis

A

autoimmune disorders causing muscle inflammation presenting with proximal muscle weakness

60
Q

Dermatomyositis signs

A

Gottron papules on back of hands
helitrope rash on eyelids

additional:
Raynaud’s
respiratory muscle weakness
interstitial lung disease: e.g. Fibrosing alveolitis or organising pneumonia
dysphagia, dysphonia

61
Q

Cause of polymositits or dermatomyositis

A

can be due to underlying cancer –> paraneoplastic syndromes
viral infection (coxsackie virus or HIV)
HLA genes risk factors

62
Q

presentation of Polymyositis or dermatomyositis

A

gradual onset
symmetrical
proximal muscle weakness
difficulty standing from chair
myalgia

no skin changes in poly

skin changes in dermato
- gottron lesions, helitrope rash, periobrital oedema, photosensitive erythematous rash

63
Q

What is the test for myositis?

A

creatine kinase in the thousands

64
Q

Mx of polymyositis and dermatomyositis

A

steroids

or
immunosuppressants,
IVIG, biologics

65
Q

Define GCA

A

medium/large systemic vasculitis

> older white demographic

66
Q

what is GCA associated with

A

polymyalgia rheumatica

67
Q

complication of GCA

A

vision loss (irreversible)

68
Q

Presentation of GCA

A

unilateral headache
jaw claudication
blurred/double vision
temporal artery tender/thickened

assoc sx.
- shoulder/pelvic stiffness
- FLAWS
- muscle tenderness
- carpel tunnel syndrome
- peripheral oedema

69
Q

Ix for GCA

A

Raised ESR
Temporal artery biopsy
Duplex USS (halo sign and stenosis of temporal artery)

70
Q

Mx of GCA

A

40-60mg prednisolone (with no visual/jaw sx)
500mg-1000mg methylprednisolone (with visual/jaw sx)

can give
aspirin
PPI
bisphosphonates, calcium vitamin D

71
Q

Define polymyalgia rheumatica

A

inflammatory condition causing pain and stiffness in shoulders, pelvic girdle and neck

72
Q

what is PMR associated with

A

GCA

73
Q

what demographic is PMR more common in

A

older white patients

74
Q

presentation of PMR

A

rapid onset days-weeks of pain/stiffness in shoulders (radiate to upper arm and elbow), pelvic girdle (radiate to thighs) and neck

75
Q

type of pain/stiffness in PMR

A

worse in morning
worse after rest/inactivity
interfere with sleep
takes 45 mins in the morning to ease
improves with activity

can also have
weight loss, fatigue, fever
muscle tenderness
carpal tunnel syndrome
peripheral oedema

76
Q

how to diagnose PMR

A

clinical presentation, response to steroids, excluding differentials
inflammatory markers usually raised but could be normal

77
Q

Mx of PMR

A

15mg prednisolone daily for 1-2 years
need dramatic improvement in sx within 1 week

78
Q

Define systemic sclerosis

A

autoimmune connective tissue disease involving inflammation and fibrosis of connective tissues, skin and internal organs.

79
Q

Name the types of systemic sclerosis

A

limited cutaneous systemic sclerosis
diffuse cutaneous systemic sclerosis

80
Q

Sx of limited cutaneous systemic sclerosis

A

CREST
calcinosis
raynaud’s
oesophageal dysmotility
sclerodactyly
telangiectasia

81
Q

Sx of diffuse cutaneous systemic sclerosis

A

CREST + CVS problems/lung problems/kidney problems

82
Q

What is scleroderma

A

hardening of the skin

83
Q

What is sclerodactyly

A

tightening of skin in hands

84
Q

What is nailfold capillaroscopy

A

magnifies peripheral capillaries at nail bed. microhaemorrhages suggest systemic sclerosis

85
Q

How to treat Raynaud’s

A

keep hands warm
CCB (nifedipine)
losartan, ACEi, sildenafil, fluoxetine

86
Q

Autoantibodies in systemic sclerosis

A

ANA
Anti-centromere (limited)
ANti-Scl70 (diffuse)

87
Q

Mx of systemic sclerosis

A

DMARDS (methotrexate)
biologics (rituximab)
Steroids

88
Q

Define discoid lupus erythematosus

A

autoimmune chronic skin condition
F>M
20-50
darker skinned patients
smokers
increased risk of SLE

89
Q

What can DLE rarely progress to

A

SCC

90
Q

Presentation of DLE

A

photosensitive lesions on face, scalp and ears
scarring alopecia
hyper/hypopigmentation
lesions are inflamed, dry, red, scaling

91
Q

Mx of DLE

A

skin biopsy to diagnose
SPF
topical steroids
intralesional steroid injections
hydroxychloroquine

92
Q

Define SLE

A

inflammatory autoimmune connective tissue disorder
F>M
young-middle aged
asian, african, caribbean, hispanic

93
Q

What type of condition is SLE

A

relapsing-remitting

94
Q

Complications of SLE

A

cardiovascular disease and infection. chronic inflammation reduces life expectancy

95
Q

Autoantibodies in SLE

A

ANA
Anti dsDNA
Anti-Sm
Antiphospholipid can occur secondary to SLE due to increased risk of VTE

96
Q

Sx of SLE

A
  • Serositis (pericarditis, pleuritis
  • Oral ulcers
  • Arthritis (non-erosive, arthralgia, myalgia)
  • Photosensitivity
  • Blood (all are low, anaemia, leukopenia, thrombocytopenia)
  • Renal (proteinuria, glomerulonephritis)
  • ANA
  • Immunological (dsDNA etc.)
  • Neurologic (psuch, seizures)
  • Malar rash (photosensitive, sparing nasolabial fold)
  • Discoid rash

Fatigue
Weight loss
Lymphadenopathy
Splenomegaly
Hair loss
Raynaud’s phenomenon

97
Q

Ix for SLE

A

autoantibodies
CRP/ESR
C3 and C4 decreased in active disease
FBC
urinalysis
renal biopsy

98
Q

Mx of SLE

A

SPF
1st line
hydroxychloroquine
NSAIDs
Steroids

more severe:
DMARDs (methotrexate)
biologics (rituximab, belimumab)

99
Q

Define Ankylosing Spondylitis

A

inflammatory condition affecting axial skeleton causing progressive stiffness and pain

100
Q

Name the seronegative spondyloarthropathies

A

AS
Psoriatic arthritis
Reactive arthritis

101
Q

Joints affected in AS

A

sacroiliac joints
vertebral column joints

102
Q

HLA B27 genes

A

Psoriatic arthritis
AS
IBD
Reactive arthritis

103
Q

Sx of AS

A

20 y/o male
pain and stiffness in lower back
sacroiliac pain in buttock
improves with movement
pain worse at night and in the morning
takes 30 mins for pain to improve

additional
chest pain: costovertebal and sternocostal joints
enthesitis
dactylitis
vertebral fractures
SOB

104
Q

AS associations

A

5As
Anterior uveitis
Aortic regurgitation
Atrioventricular block
Apical lung fibrosis
Anaemia of chronic disease

105
Q

Test for AS

A

Schober’s test

106
Q

Ix for AS

A

CRP, ESR
HLA B27
Xray of spine and sacrum (bamboo spine)
MRI spine

107
Q

Mx of AS

A

1st NSAIDs
2nd anti TNF
3rd secukinumab/ixekizumab
3rd upadacitinib

can give steroid injections for specific joints
physio

108
Q

Define Reactive arthritis

A

synovitis in one or more joints due to infective trigger (gastroenteritis, chlamydia)
causes acute monoarthritis

109
Q

Associations of reactive arthritis

A

bilateral conjunctivitis
anterior uveitis
urethritis
circinate balanitis
“can’t see, pee or climb a tree)

110
Q

Mx of reactive arthritis

A

exclude septic arthritis
joint aspiration
treat trigger
NSAIDs
steroid injection into affected joint
most cases resolve within 6 months

111
Q

Define psoriatic arthritis

A

inflammatory arthritis assoc with psoriasis
can vary from mild stiffening of joints to arthritis mutilans

112
Q

extra-articular manifestations of psoriatic arthritis

A

uveitis
IBD

113
Q

5 patterns of psoriatic arthritis

A
  1. asymmetrical oligoarthritis (1-4 joints on one side of the body)
  2. symmetrical polyarthritis (like RA, more than 4 joints)
  3. DIP predominant pattern (DIP joints)
  4. Spondylitis (back stiffness and pain, axial skeleton)
  5. Arthritis mutilans (most severe, affecting phalanges. Osteolysis around joints and telescoping digit)
114
Q

Signs of psoriatic arthritis

A

psoriasis plaques
nail pitting
onycholysis
dactylitis
enthesitis

115
Q

xray changes in psoriatic arthritis

A

periostitis
ankylosis
osteolysis
dactylitis
pencil in cup in digits

116
Q

Mx of psoriatic arthritis

A

NSAIDs
steroids
DMARDs
anti TNF
ustekinumab

117
Q

define Rheumatoid arthritis

A

autoimmune condition causing chronic inflammation in synovial lining of joints, tendon sheaths and bursa
inflammatory arthritis

118
Q

Signs of RA

A

affect multiple small joints (MCP, PIP, wrist, MTP) symmetrically
F > M
middle age
pain/stiffness/swelling
boggy joints

119
Q

Gene associated with RA

A

HLA DR4

120
Q

autoantibodies in RA

A

Rheumatoid factors
Anti CCP

121
Q

Hand signs in advanced RA

A

Z shaped deformity of thumb
swan neck deformity (hyperextended PIP and flexed DIP_
Boutonniere deformity (hyperextended DIP and flexed PIP)
ulnar deviation at MCP joints

122
Q

Extra articular manifestations of RA

A
  • Pulmonary fibrosis
  • Felty’s syndrome (a triad of rheumatoid arthritis, neutropenia and splenomegaly)
  • Sjögren’s syndrome (with dry eyes and dry mouth)
  • Anaemia of chronic disease
  • Cardiovascular disease
  • Eye manifestations
  • Rheumatoid nodules (firm, painless lumps under the skin, typically on the elbows and fingers)
  • Lymphadenopathy
  • Carpel tunnel syndrome
  • Amyloidosis
  • Bronchiolitis obliterans (small airway destruction and airflow obstruction in the lungs)
  • Caplan syndrome (pulmonary nodules in patients with rheumatoid arthritis exposed to coal, silica or asbestos dust)

Eye manifestations related to rheumatoid arthritis and its treatment include:

  • Dry eye syndrome (keratoconjunctivitis sicca)
  • Episcleritis
  • Scleritis
  • Keratitis
  • Cataracts (secondary to steroids)
  • Retinopathy (secondary to hydroxychloroquine)
123
Q

Xray changes of RA

A

periarticular osteopenia
bony erosions
soft tissue swelling
joint destruction and deformity

124
Q

Mx of RA

A

steroids short term
DMARDs and biologics
hyxroychloroquine can be used in mild disease
NSAIDs for pain relief

125
Q

Side effects of methotrexate

A

Mouth ulcers and mucositis
Liver toxicity
Bone marrow suppression and leukopenia (low white blood cells)
Teratogenic and needs to be avoided before conception in both women and men for 3-6 months

126
Q

Define Osteoarthritis

A

wear and tear in joints

127
Q

joints affected in OA

A

Hips
Knees
Distal interphalangeal (DIP) joints in the hands
Carpometacarpal (CMC) joint at the base of the thumb
Lumbar spine
Cervical spine (cervical spondylosis)

128
Q

Xray changes in OA

A

LOSS
L- loss of joints space
O- osteophytes
S- subchondral cysts (fluid holes in bone)
S- Subarticular sclerosis (increased denosity of bone at joint)

129
Q

Signs of OA

A

pain/stiffness worse at the end of the day
Bulky, bony enlargement of the joint
Restricted range of motion
Crepitus on movement
Effusions (fluid) around the joint

Signs in the Hands
Heberden’s nodes (in the DIP joints)
Bouchard’s nodes (in the PIP joints)
Squaring at the base of the thumb (CMC joint)
Weak grip
Reduced range of motion

130
Q

Mx of OA

A

exercise, weight loss
topical NSAID
Oral NSAID
weak opiate.paracetamol
intr-articular steroid injection
joint replacement

131
Q

Define drug induced SLE

A

not all the typical features of systemic lupus erythematosus are seen, with renal and nervous system involvement being unusual. It usually resolves on stopping the drug.

132
Q

Features of DILE

A

arthralgia
myalgia
skin (e.g. malar rash) and pulmonary involvement (e.g. pleurisy) are common
ANA positive in 100%, dsDNA negative
anti-histone antibodies are found in 80-90%
anti-Ro, anti-Smith positive in around 5%

133
Q

Causes of DILE

A

most common:
procainamide
hydralazine

less common:
isoniazid
minocycline
phenytoin