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MRCP > Rheumatology > Flashcards

Rheumatology Flashcards

1
Q

cytoplasmic inclusions on muscle biopsy1st line treatment of osteoporosis?

A

alendronate

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2
Q

2nd line treatment of osteoporosis if unable to tolerate alendronate due to upper GI side effects?

A

risedronate/ etidronate

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3
Q

3rd line treatment of osteoporosis if cannot take bisphosphonates?

A

Strontium Ralenate (dual action: promotes differentiation from pre-osteoblast to osteoblast + inhibits osteoclasts) or Raloxifene (Selective oestrogen receptor modulator)

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4
Q

what e.g. is a once only oral bisphosphonate?

A

ibandronate

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5
Q

denosumab?

A

human monoclonal antibody: anti-RANK ligand -> inhibits maturation of osteoclasts

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6
Q

what protein is defective in Marfans?

A

fibrillin-1

autosomal dominant- defect in FBN1 gene on chr 15

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7
Q

oral + genital ulcers + anterior uveitis + thrombophlebitis?

A

Behcet’s syndrome

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8
Q

classic triad of Behcet’s syndrome?

A

oral ulcers + genital ulcers + anterior uveitis

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9
Q

Features of Behcets?

A
  • oral + genital ulcers - anterior uveitis - thrombophlebitis +/- DVT - arthritis - neuro involvement e.g. aseptic meningitis - GI: abdo pain, diarrhoea - erythema nodosum *presumed autoimmune-mediated inflammation of arteries and veins
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10
Q

What HLA is assoc w Behcets?

A

HLA-B51 (a split antigen of HLA B5)

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11
Q

Diagnosis of Behcets?

A

clinical: no definitive test - positive pathergy test is suggestive (ie. puncture site following needle prick becomes inflamed with small pustule forming)

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12
Q

what joints are most commonly implicated in osteoarthritis?

A

carpometacarpal + DIP joints

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13
Q

What are Heberden’s and Bouchard’s nodes?

A

Heberden: DIPJ Bouchard: PIPJ result of osteophyte formation.

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14
Q

features of osteoarthritis?

A
  • usually bilateral - provoked by movement, relieved by resting - stiffness: Worse after long periods of inactivity (only lasts few mins) - Heberdens + Bouchards - squaring of the thumbs
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15
Q

What T score suggests osteoporosis? vs osteopenia?

A

> -1.0 = normal

-1.0 to -2.5 = osteopaenia

< -2.5 = osteoporosis

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16
Q

most useful investigation in diagnosis of Ankylosing spondylitis?

A

Plain x-ray of the sacroiliac joints

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17
Q

features of ankylosing spondylitis on X-ray?

A
  • sacroiliitis: subchondral erosions, sclerosis
  • squaring of lumbar vertebrae
  • ‘bamboo spine’ (late & uncommon)
  • syndesmophytes: due to ossification of outer fibers of annulus fibrosus
  • chest x-ray: apical fibrosis
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18
Q

Ix of choice if X-ray is negative for Ank spondylitis but clinical suspicion remains high?

A

MRI: Signs of early inflammation involving sacroiliac joints (bone marrow oedema) confirm the diagnosis

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19
Q

1st line mx of Ank Spond?

A

NSAIDs + exercise / physio

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20
Q

What management should be considered in persistently high disease activity in Ank Spond despite conventional treatment?

A

Anti-TNF therapies e.g. etanercept / adalimumab

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21
Q

mix of SLE + Systemic sclerosis + myositis?

A

mixed connective tissue disease ie. Sharp’s syndrome

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22
Q

what antibody is most sensitive for mixed connective tissue disease?

A

anti-U1 RNP antibodies RNP = ribonucleoprotein

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23
Q

most common features of mixed connective tissue disease?

A
  • Raynaud’s often precedes (occurs in 90%) - polyarthralgia/ arthritis - myalgia - dactylitis - other dermatological (e.g. photosensitive rash), oesophageal dysfunction, respiratory, haematological, cardiac, renal, neuropsych features
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24
Q

osteogenesis imperfecta - abnormality in what?

A

type 1 collagen - which is main component of the organic part of bone + skin & tendons

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25
Q

Disorder in type 5 collagen?

A

Ehlers-Danlos syndrome - Type 5 collagen found in cell surfaces, hair, placentas

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26
Q

Type 4 collagen mutations cause?

A

Alpert’s syndrome, Goodpasture’s *defect in basal lamina

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27
Q

Defect in type 2 collagen?

A

Chondrodysplasias - type 2 collagen is main component of cartilage

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28
Q

Defect in type 3 collagen?

A

can lead to a type of Ehlers-Danlos *not the classic type Type 3 collagen is main component of reticular fibres

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29
Q

features of osteogenesis imperfecta?

A
  • fractures following minor trauma - blue sclera - deafness 2dry to osteosclerosis - dental imperfections
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30
Q

Ca/ Phosphate/ PTH/ Alk Phos results in osteogenesis imperfecta?

A

usually all normal

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31
Q

common causes of drug-induced lupus?

A

Most common: procainamide hydralazine

less common: isoniazid minocycline phenytoin

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32
Q

most sensitive antibodies for drug induced lupus?

A

anti-histone abs (in 80-90%) *ANA+ (100%), dsDNA -ve, anti Ro/ Smith +ve in 5%

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33
Q

xray changes in rheumatoid arthritis?

A

Early x-ray findings: - loss of joint space - juxta-articular osteoporosis - soft-tissue swelling Late x-ray findings: - periarticular erosions - subluxation

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34
Q

most specific antibodies for dermatomyositis?

A

anti-Mi-2 antibodies

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35
Q

most specific antibodies for polymyositis?

A

anti-Jo1 antibodies

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36
Q

Management of dermatomyositis?

A

prednisolone

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37
Q

anti-La antibodies?

A

Sjogrens

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38
Q

Anti-Ro antibodies?

A

Sjogrens, SLE, Congenital Heart block

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39
Q

SEs of bisphosphonates?

A

oesophageal reactions: ulcer/ oesophagitis osteonecrosis of the jaw increased risk atypical stress #s of proximal femoral shaft acute phase response: fever, myalgia, arthralgia following administration hypoCa: reduced ca efflux from bone, usually clincally unimportant

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40
Q

management of vit D deficiency?

A

Oral Vit D loading then maintenance

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41
Q

features of Antiphospholipid syndrome?

A

recurring fetal loss, venous/ arterial thrombosis

  • livedo reticularis
  • thrombocytopenia
  • prolonged APTT
  • pulmonary HTN, pre-eclampsia
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42
Q

primary thromboprophylaxis in antiphospholipid syndrome?

A

low dose aspirin

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43
Q

secondary thromboprophylaxis in antiphospholipid syndrome?

A

lifelong warfarin w target INR 2-3 to start

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44
Q

what type of hypersensitivity reaction is SLE?

A

type 3 immune complex dysregulation -> immune complex formation -> deposition can affect any organ (skin, joints, kidney, brain)

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45
Q

SLE assoc w which HLA?

A

HLA B8, DR2, DR3

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46
Q

SE of raloxifene?

A

increased risk of VTE may worsen menopausal symptoms

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47
Q

raloxifene and risk of breast ca?

A

decreases risk

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48
Q

causes of decreased excretion of uric acid? thus predisposing to gout

A

drugs: diuretics CKD lead toxicity Lesch-Nyhan syndrome

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49
Q

Gout: causes of increased production of uric acid?

A

myeloproliferative/ lymphoproliferative disorders cytotoxics severe psoriasis

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50
Q

IBD drugs: which are safe to use in pregnancy?

A

azathioprine, mesalazine and sulfasalazine (w folic acid) safe to use in pregnancy and breastfeeding

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51
Q

SE of Azathioprine?

A

bone marrow suppression N+V pancreatitis

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52
Q

azathioprine increases risk of what type of ca?

A

non melanoma skin cancer

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53
Q

azathioprine may interact w which drug?

A

allopurinol - lower doses of azathioprine should be used if taking allopurinol

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54
Q

management of methotrexate toxicity?

A

folinic acid

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55
Q

What medication may interact with methotrexate to increase the risk of methotrexate toxicity secondary to reduced excretion?

A

high dose aspirin

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56
Q

what antibody is found in polyarteritis nodosa?

A

assoc w p-ANCA perinuclear-antineutrophil cytoplasmic abs

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57
Q

what infection is assoc w polyarteritis nodosa?

A

hep B

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58
Q

CK and EMG findings in polymyalgia rheumatica?

A

Normal! Pts may have raised inflammatory markers e.g. ESR>40

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59
Q

features of pseudoxanthoma elasticum?

A
  • retinal angioid streaks
  • ‘plucked chicken skin’ appearance - small yellow papules on the neck, antecubital fossa and axillae
  • cardiac: mitral valve prolapse, increased risk of ischaemic heart disease
  • GI haemorrhage
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60
Q

most common organism causing iliopsoas abscess?

A

staph aureus

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61
Q

most common secondary cause of iliopsoas asbcess?

A

crohns disease

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62
Q

examination findings of iliopsoas abscess?

A

hyperextension of hip -> pain as psoas muscle is stretched

ask pt to lift thigh against hand -> pain due to contraction of psoas muscle

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63
Q

ix of choice for iliopsoas abscess?

A

CT abdomen

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64
Q

management of iliopsoas abscess?

A

abx

percutaneous drainage initial approach

surgery if: failure of percutaneous draining/ presence of another intra-abdo pathology which requires surgery

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65
Q

when are topical NSAIDs indicated in osteoarthritis?

A

for OA of knee/ hand

66
Q

what antibody titre can be used for disease monitoring of SLE?

A

anti-dsDNA titres

67
Q

what is the major target within the cell of p-ANCA?

A

myeloperoxidase

68
Q

what is the main target within the cell for cANCA?

A

proteinase-3

69
Q

What is the minimum steroid intake a patient should be taking before they are offered osteoporosis prophylaxis?

A

equivalent of pred 7.5mg or more for 3 months or longer

70
Q

proximal myopathy + mechanic hands + interstitial lung disease?

A

anti-synthetase syndrome

71
Q

SE of hydroxychloroquine?

A

bulls eye retinopathy - might result in severe and permanent visual loss

baseline exam + annual screening recommended

72
Q

management of patients at risk of corticosteroid- induced osteoporosis?

if pt is >65 OR had fragility fracture in the past

A

offer bone protection ie. bisphosphonates

73
Q

management of patients at risk of corticosteroid-induced osteoporosis?

A

offer bone density scan

if T score bone protection

if between 0 and -1.5 -> repeat bone density scan in 1-3 years

74
Q

MOA of mycophenolate mofetil?

A

inhibits inosine-5-monophosphate dehydrogenase which is needed for purine synthesis

  • > reduces lymphocyte production
  • > used in organ transplant to preven rejection/ autoimmune conditions
75
Q

sjogrens - increased risk of which malignancy?

A

lymphoid (40-60 fold)

76
Q

osteoporosis in males - check what hormone level?

A

testosterone

  • testosterone deficiency
77
Q

muscle pain and stiffness following exercise, cramps, myoglobinuria, low lactate levels during exercise

A

McArdle’s disease

  • autosomal recessive type V glycogen storage disease
  • caused by myophosphorylase deficiency
78
Q

allergy to which other classes should caution you to starting sulfasalazine?

A

aspirin, sulphonamides

79
Q

SEs of sulfasalazine?

A
  • oligospermia
  • Stevens-Johnson syndrome
  • pneumonitis / lung fibrosis
  • myelosuppression, Heinz body anaemia, megaloblastic anaemia
  • may colour tears → stained contact lenses
80
Q

key features of relapsing polychondritis?

A

ears: auricular chondritis, hearing loss, vertigo
nasal: saddle nose deformity

Resp tract: e.g. wheeze/ stridor/ hoarseness

ocular: episcleritis/ scleritis/ iritis

arthalgia

less commonly: cardiac valvular regurg, cranial n palsies, peripheral neuropathies, renal dysfunction

81
Q

What is the greatest predictor of future thrombosis in patients with anti-phospholipid syndrome?

A

Lupus anticoagulant

Lupus anticoagulants have an odds ratio for thrombosis 5 to 16 times higher than controls.

82
Q

most useful characteristic sign of gout on US?

A

double contour sign

  • a hyperechoic, irregular band over the superficial margin of the joint cartilage, produced by the deposition of monosodium urate crystals on the surface of the hyaline cartilage
83
Q

most common cardiac abnormality of Marfans syndrome?

A

aortic root dilatation

84
Q

what management might you start in Adult onset stills disease if symptoms persist despite initial management?

A

anti IL1 e.g. anakinra

/ TNF therapy

or methotrexate

85
Q

monitoring while pt is on leflunomide (a DMARD)?

A

FBC/ LFTs and BP

86
Q

topical NSAIDs used for OA of..?

A

knees/ hand

87
Q

ank spond, what type of pulmonary fibrosis?

A

apical

88
Q

Which one of the following cells secretes the majority of tumour necrosis factor in humans?

A

macrophages

89
Q

lens dislocation in Marfans’s?

A

UP

90
Q

MOA of azathioprine?

A

inhibits purine synthesis

91
Q

osteopetrosis - dysfunction in?

A

defective osteoclast function -> failure of normal bone resorption

-> results in dense, thick bones that are prone to fracture

aka Marble bone disease

92
Q

features of osteopetrosis?

A

bone pain/ neuropathies common

prone to fractures

Ca, PO4, ALP normal

stem cell transplant and IFN-gamma have been used for treatment

93
Q

Pseudogout associated factors?

A

increasing age

haemochromatosis

hyperparathyroidism

low magnesium, low phosphate

acromegaly, Wilson’s disease

94
Q

Ehlers-Danlos: what type of collagen is affected?

A

type 3 collagen

95
Q

complications of Paget’s disease?

A

deafness (cranial nerve entrapment)

bone sarcoma (1% if affected for > 10 years)

fractures

skull thickening

high-output cardiac failure

96
Q

SEs of TNFa inhibitors e.g. etanercept/ infliximab?

A

reactivation of TB

etanercept: can cause demyelination

97
Q

which anti-TB medication causes drug induced SLE?

A

isoniazid

98
Q

pathophysiology of discoid lupus erythematous?

A
  • benign disorder, rarely progresses to SLE
  • characterised by follicular keratin plugs
  • autoimmune aetiology
99
Q

Radial tunnel syndrome vs Lateral epicondylitis?

A
  • similar presentation
  • Radial tunnel syndrome: pain tends to be around 4-5cm distal to the lateral epicondyle
  • symptoms may be worsened by extending the elbow and pronating the forearm
  • compression of posterior interosseous branch of the radial nerve
100
Q

most common cause of death in sustemic sclerosis?

A

respiratory involvement

(80%)

  • interstitial lung disease and pulmonary arterial hypertension
101
Q

causes of avascular necrosis of hip?

A

long-term steroid use

chemotherapy

alcohol excess

trauma

102
Q

what autoantibody is most likely to be positive in dermatomyositis?

A

ANA

  • positive in 60%

Anti-Mi-2 only seen in ~25%

103
Q

1st step management of Rheumatoid arthritis?

A

DMARD monotherapy (most often methotrexate) +/- short course of bridging prednisolone

104
Q

Rheumatoid arthritis: monitoring response to treatment?

A

CRP + DAS28 score to monitor disease activity

105
Q

Features of polyarteritis nodosa?

A

fever, malaise, arthralgia

weight loss

hypertension

mononeuritis multiplex, sensorimotor polyneuropathy

testicular pain

livedo reticularis

haematuria, renal failure

  • pANCA +ve in 20%, Hep B +ve in 30%
106
Q

Dural ectasia in Marfan’s syndrome?

A

dural ectasia = ballooning of the dural sac at the lumbosacral lvl

may cause lower back pain associated with neurological problems such as bladder and bowel dysfunction.

107
Q

Features of McArdle’s disease?

A
  • decreased muscle glycogenolysis. auto recessive. myophosphorylase deficiency.

muscle pain and stiffness following exercise

muscle cramps

myoglobinuria

low lactate levels during exercise

108
Q

pathophysiology of Langerhans cell histiocytosis?

A

abnormal proliferation of histiocytes

typically presents in childhood with bony lesions

109
Q

Features of Langerhans cells histiocytosis?

A

bone pain, typically in the skull or proximal femur

cutaneous nodules

recurrent otitis media/mastoiditis

tennis racket-shaped Birbeck granules on electromicroscopy

110
Q

main target of pANCA?

A

myeloperoxidase (MPO)

111
Q

main target of cANCA?

A

serine proteinase 3 (PR3)

112
Q

Mx of discoid lupus erythematous?

A

1st line: topical steroid cream

oral antimalarials may be used second-line e.g. hydroxychloroquine

avoid sun exposure

113
Q

poor prognostic features of Rheumatoid arthritis?

A

rheumatoid factor +, anti-CCP antibodies

poor functional status at presentation

X-ray: early erosions (e.g. after < 2 years)

extra articular features e.g. nodules

HLA DR4

insidious onset

114
Q

Which biochemical marker (other than alk phos) could be tested to support a diagnosis of Paget’s disease of the bone?

A

serum/ urine hydroxypoline (raised)

  • markers of bone turnover
115
Q

What is used for disease monitoring of SLE?

A
  • ESR
  • C3/4 = low
  • anti-dsDNA titres
116
Q

Autoantibodies in adult onset Still’s disease?

A

rheumatoid factor and ANA

are NEGATIVE

  • its a diagnosis of exclusion
117
Q

Features of adult onset Still’s disease?

A

arthralgia

raised ferritin

macpap, salmon-pink rash

pyrexia: typically rises late afternoon

lymphadenopathy

118
Q

mx of familial mediterranean fever?

A

colchicine may help

119
Q

what conveys poor prognosis in polymyositis?

A

interstitial lung disease

  • major risk factor for premature death
120
Q

Features of Polymyositis?

A

proximal muscle weakness +/- tenderness

Raynaud’s

respiratory muscle weakness

interstitial lung disease: e.g. fibrosing alveolitis or organising pneumonia

dysphagia, dysphonia

121
Q

Wegeners/ Granulomatosis with polyangiitis: treatment of choice?

A

cyclophosphamide and streroids

122
Q

what type of GN does Churg-strauss (Eosinophilic GPA) usually cause?

A

focal segmental glomerulosclerosis

123
Q

What renal abnormalities are associated with Henoch-Schonlein purpura?

A

glomerular IgA deposition

  • usually managed with NSAIDs. if severe: steroids
124
Q

Ankylosing spondylitis: earliest sign on X-ray

A

sacroiliitis: subchondral erosions and sclerosis

125
Q

Antiphospholipid syndrome: what autoantibodies are associated?

A

anti-cardiolipin

lupus anticoagulant

anti-B2 glycoprotein 1

126
Q

most common type of psoriatic arthropathy at presentation?

A

peripheral asymmetric oligoarthropathy

127
Q

Sjogren’s syndrome: what medication can stimulate saliva production?

A

pilocarpine

128
Q

Features of Familial Mediterranean Fever?

A
  • auto recessive
  • attacks lasting 1-3 days
  • pyrexia
  • abdo pain (due to peritonitis)
  • pleurisy
  • pericarditis
  • arthritis
  • erysipeloid rash on lower limbs
129
Q

Features of Scheuermann’s disease?

A

usually affects 13-16yo

  • affects normal ossification of ring epiphyses of several thoracic vertebrae
  • Vertebrae narrower anteriorly -> Kyphosis
130
Q

Gout: prophylaxis in those where allopurinol is not tolerated?

A

febuxostat

(also a xanthine oxidase inhibitor)

131
Q

Gout: what are other prophylactic medications in refractory cases?

A
  1. uricase
  2. pegloticase (polyethylene glycol modified mammalian uricase) > given as an infusion once every two weeks
132
Q

in which group of patients would you start alendronate before DEXA scan confirms osteoporosis?

A

in adults ≥75 with fragility #

133
Q

Perthes disease: management?

A
  • keep the femoral head within the acetabulum: cast, braces
  • If < 6 years: observation
  • Older: surgical management with moderate results
  • Operate on severe deformities
134
Q

scleroderma renal crisis causing hypertensive emergency: mx?

A

oral ACEi

  • aim gradual reduction of blood pressure at a rate of 10-15 mmHg per day
135
Q

most definitive way of confirming the diagnosis of primary Sjogren’s syndrome?

A

Salivary gland biopsy

  • will show focal lymphocytic sialadenitis
136
Q

management of methotrexate if pt develops new oral ulceration?

A

hold MTX + discuss with rheum

137
Q

Fibromyalgia: pharmacological therapies?

A

pregabalin, duloxetine, amitriptyline

138
Q

Ix of Paget’s disease that reflects increased bone turnover?

A

Serum C-telopeptide (CTx) - useful in monitoring disease progression or treatment efficacy Others: - procollagen type I N-terminal propeptide (PINP) - urinary N-telopeptide (NTx) - urinary hydroxyproline

139
Q

Features of familial Mediterranean fever aka recurrent polyserositis?

A

more common in people of Turkish, Armenian and Arabic descent Attacks lasting 1-3 days: - fever - abdo pain (peritonitis) - Pleurisy - pericarditis - arthritis - erysipeloid rash on lower limbs

140
Q

Mx of familial Mediterranean fever aka recurrent polyserositis?

A

Colchicine

141
Q

Features of jaccouds arthropathy?

A

‘reversible’ joint deformities such as swan neck, thumb subluxation, ulnar deviation, ‘boutonniere’ and hallux valgus + NO articular erosions on XR

142
Q

Rheumatoid arthritis: When is a third agent required?

A

starting of biologic therapy when the patient has been on at least 2 DMARDs, including methotrexate > with two DAS 28 scores of > 5.1 at least one month apart

143
Q

What ix will lead to diagnosis of polyarteritis nodosa?

A

Angiography Ie renal angiogram

144
Q

Tx of choice in SLE?

A

Hydroxychloroquine

145
Q

Tx to consider in SLE if f internal organ involvement e.g. renal, neuro, eye?

A

prednisolone, cyclophosphamide

146
Q

What autoantibody is assoc with high risk of scleroderma renal crisis?

A

anti-RNA polymerase III

147
Q

What antibodies are assoc with high risk of pulmonary hypertension in systemic sclerosis?

A

anti-U3-RNP

148
Q

What autoantibodies are assoc with Systemic sclerosis assoc myositis?

A

anti-PM-Scl

149
Q

Management of stills disease?

A

1st line: NSAIDs (to manage fever, joint pain, serositis) - trial for at least 1 wk before adding steroids if symptoms persist, the use of methotrexate, IL-1 or anti-TNF therapy can be considered

150
Q

Steroid sparing agent for management of giant cell arteritis?

A

Methotrexate

151
Q

First line management of adult onset stills disease?

A

NSAIDs first

152
Q

First line management of adult onset stills disease?

A

NSAIDs first

153
Q

Medications to treat LUPUS nephritis?

A

Mycophenolate mofetil

154
Q

Ocular features in ehler Danlos?

A

angioid retinal streaks

155
Q

Muscle biopsy: Endomysial lymphocytic infiltrates that invade nonnecrotic muscle fibres

A

Polymyositis

156
Q

Muscle biopsy: Perimysial inflammation of lymphocytes and parafascicular atrophy

A

Dermatomyositis

157
Q

Muscle biopsy: cytoplasmic inclusions

A

Inclusion body myositis

158
Q

Features of Langerhans cell histiocytosis ? (abnormal proliferation of histiocytes)

A
  1. bone pain, typically in the skull or proximal femur 2. cutaneous nodules 3. recurrent otitis media/mastoiditis 4. tennis racket-shaped Birbeck granules on electromicroscopy
159
Q

first line tx of psoriatic arthropathy?

A

Same as rheumatoid arthritis Ie DMARD like methotrexate

160
Q

What medication might precipitate a scleroderma renal crisis?

A

Steroids

161
Q

Ankylosing spondylitis: cardiac abnormalities?

A

AV node block Aortic regurgitation

MRCP (21 decks)

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