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EXAM > Rheum 7.5 > Flashcards

Rheum 7.5 Flashcards

1
Q

2ry prevention of osteoporotic fractures in postmenopausal women?

A
  • start Rx in postmenopausal women with osteoporotic fragility fractures confirmed to have osteoporosis
  • vit D & calcium to all (unless confident they have adequate calcium intake and are it D replete)
  • 1st line alendronate (25% don’t tolerate due to upper GI problems -> offer risedronate/etodronate)
  • strontium ranelate & raloxifine recommended if pts can’t tolerate bisphosphonates
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2
Q

Osteoporosis management - guidelines for patients who don’t tolerate alendronate - what factors are taken into account?

A
  • age
  • T-score
  • parental Hx of hip fracture
  • etoh intake 4+units/day
  • rheumatoid arthritis
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3
Q

Criteria for starting strontium ranelate or raloxifene for osteoporosis?

A
  • only if any bisphosphonate not tolerated

- strict T-scores e.g. 60y.o. female T-score <3/5

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4
Q

Osteoporosis drugs licensed for prevention AND treatment of post-menopausal & glucocorticoid-induced osteoporosis?
What type of fractures do they reduce the risk of?

A
  • Bisphosphonates: alendronate, risedronate, etidronate
  • reduce risk of Vertebral And non-vertebral fractures
  • alendronate & risedronate superior to etidronate in preventing hip fractures
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5
Q

Mechanism of action of Raloxifene?
4 benefits in osteoporosis?
1 disadvantage?
1 risk?

A
  • selective oestrogen receptor modulator
  1. prevents bone loss
  2. reduces risk of vertebral fractures (not yet shown for non-vertebral fractures)
  3. increases bone density in spine & proximal femur
  4. may reduce risk of breast cancer

X may worsen menopausal Sx
X increased risk of thromboembolic events

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6
Q

How does strontium ranelate work in osteoporosis?

3 high risks?

A

‘dual action bone agent’

  • increases deposition of new bone by osteoblasts (promotes differentiation of pre-osteoblast to osteoblast)
  • reduces resorption of bone by inhibiting osteoclasts
  1. increased risk of cardiovascular events (any CVD or significant risk is a C/I)
  2. increased risk of thromboembolic events (do not use if Hx of VTE)
  3. may cause skin reactions e.g. Stevens Johnson syndrome
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7
Q

How does denosumab work in osteoporosis?

A
  • human mAb that inhibits RANK ligand -> inhibits maturation of osteoclasts
  • SC injection / 6m
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8
Q

What is Teriparatide?

Significance in osteoporosis?

A
  • recombinant form of parathyroid hormone

- v. effective at increasing bone mineral density but role inRx of osteoporosis yet to be defined

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9
Q

HRT in osteoporosis: benefits & risks?

A
  • reduces incidence of vertebral And non-vertebral fractures
  • NOT recommended as part of 1ry/2ry prevention of osteoporosis (only Ox for vasomotor Sx)
  • increased rates of cardiovascular disease & breast cancer
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10
Q

What is shown to significantly reduce hip fractures in nursing home patients?

A

hip protectors

issue of compliance

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11
Q

What is McArdle’s disease?
genetics?
Features?

A

Decreased muscle glycogenolysis
- autosomal recessive type V glycogen storage disease caused by myophosphorylase deficiency

  1. muscle pain & stiffness after exercise
  2. muscle cramps
  3. myoglobinuria
  4. low lactate levels during exercise
  • characteristically ass with a ‘2nd wind’ phenomenon
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12
Q

Limited cutaneous systemic sclerosis

  • subtype
  • distribution
  • Ab
  • common late complication affecting mortality?
A
  • face & distal limbs predominately
  • CREST: calcinosis, Raynauds, oesophageal dysmotility, sclerodactyly, telangiectasia
  • anti-centromere Ab
  • pulmonary hypertension
  • malabsorption (bacterial overgrowth of small bowel)
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13
Q

Diffuse cutaneous systemic sclerosis

  • distribution
  • Ab
  • common complications
A
  • trunk & proximal limbs
  • scl-70 Ab
  • HTN, lung fibrosis, renal involvement
  • poor Px
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14
Q

What is scleroderma?

A

hardened, sclerotic skin & CT without internal organ involvement

  • plaques = morphoea
  • linear = coup de sabre
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15
Q

Ab in systemic sclerosis?

A

ANA + 90%
RF + 30%
anti-scl-70 diffuse
anti-centromere Ab limited

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16
Q

Swelling over posterior aspect of elbow, may be ass with pain, warmth, erythema
- typically affecting middle-aged male pts

A

Olecranon bursitis

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17
Q

Elbow pain that may be worse when elbow resting on a firm surface or flexed for extended periods
Elbow pain initially intermittent tingling in 4th & 5th fingers
Later numbness in 4th & 5th finger with ass weakness

A

Cubital tunnel syndrome

due to ulnar n compression

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18
Q

Elbow pain as a result of overuse, usually 4-5cm distal to lateral epicondyle
Sx may be worsened by extending elbow & pronating forearm

A

Radial tunnel syndrome

- most commonly due to compression of posterior interosseous branch of radial nerve

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19
Q

Elbow pain aggravated by wrist flexion & pronation, +/- numbness/tingling in 4th & 5th finger due to ulnar nerve involvement
- pain & tenderness localised to medial epicondyle

A

Medial epicondylitis

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20
Q

Elbow pain worse on resisted wrist extension with elbow extended or supination of forearm with elbow extended

  • acute pain 6-12wks
  • episodes can last 6m-2yrs
  • pain & tenderness localised to lateral epicondyle
A

Lateral epicondylitis

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21
Q

What is Gout?

A
  • chronic hyperuricaemia (uric acid > 450) leading to micro crystal synovitis
  • caused by deposition of monosodium rate monohydrate in the synovium
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22
Q

Acute Rx of Gout?

A
  1. NSAIDs/Colchicine + PPI
  • Cont. max dose NSAID until 1-2d after Sx have settled
  • Colchicine has slower onset of action, dose can be ltd by diarrhoea
  • Oral steroids e.g. Pred 15mg OD can be considered if NSAIDs/Colchicine C/I
  • Intra-articular steroid injection also an option
  • If already on Allopurinol, continue
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23
Q

Indications for urate-lowering in gout

A
  1. 2+ acute attacks in 12 months
  2. tophi
  3. uric acid renal stones
  4. renal disease
  5. prophylaxis if on cytotoxics/diuretics

BSR now advocates: Offer urate-lowering therapy to all after their FIRST attack of gout

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24
Q

Lifestyle modifications for pts with gout

A
  1. Reduce etoh intake and avoid during an acute attack
  2. weight loss if appropriate
  3. avoid food high in purines e.g. liver, kidneys, seafood, oily fish, yeast
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25
Q

How does colchicine work?

A
  • inhibits microtubule polymerisation by binding to tubulin, interfering with mitosis
  • also inhibits neutrophil motility & activity
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26
Q

Urate-Lowering therapy in gout
1st line
2nd line
Aim of Rx?

A

1st. Allopurinol - wait at least 2wks after acute attack of gout
2nd. Febuxostat if allopurinol not tolerated or ineffective (also xanthine oxidase inhibitor)

Start with Allopurinol 100mg OD - Lower if reduced eGFR

  • titrate every few weeks to aim for serum uric acid < 300
  • consider covering with colchicine for approx 6months, or said if not tolerated
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27
Q

Antihypertensive that may benefit in gout?

Vitamin that may benefit?

A

Losartan - has a specific uricosuric action - therefore may be particularly suitable if coexistent hypertension
Increased vitamin C intake may help decrease serum uric acid levels?

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28
Q

Commonest causative organism in septic arthritis?

What to consider in young sexually active adults?

A

Staph aureus

Neisseria gonorrhoeae

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29
Q

Kocher criteria for Dx of septic arthritis?

A
  1. fever > 38.5
  2. non-weight bearing
  3. raised ESR
  4. raised WCC
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30
Q

Management of septic arthritis?

A

Aspirate synovial fluid
IV Abx 6-12weeks to cover Gram +ve cocci - Flucloxacillin or Clindamycin
Needle aspiration to decompress joint
Arthroscopic lavage may be required (i.e. if prosthetic)

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31
Q

Features of prolapsed lumbar disc/sciatica?

A
  • leg pain usually worse than back
  • pain often worse when sitting
  • usually dermatomal leg pain ass with neurological deficits
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32
Q

Prolapsed lumbar disc:

  • sensory loss over anterior thigh
  • weak quadriceps
  • reduced knee reflex
  • +ve femoral stretch test
A

L3 nerve root compression

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33
Q

Prolapsed lumbar disc:

  • sensory loss anterior aspect of knee
  • weak quadriceps
  • reduced knee reflex
  • +ve femoral stretch test
A

L4 nerve root compression

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34
Q

Prolapsed lumbar disc:

  • sensory loss dorsum foot
  • weakness in foot & big toe dorsiflexion
  • reflexes intact
  • +ve sciatic nerve stretch test
A

L5 nerve root compression (sciatica)

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35
Q

Prolapsed lumbar disc:

  • sensory loss posterolateral aspect of leg
  • weakness in plantar flexion of foot
  • reduced ankle reflex
  • +ve sciatic nerve stretch test
A

S1 nerve root compression (sciatica)

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36
Q

Management of prolapsed lumbar disc?

A
  • analgesia, PT, exercises, amitriptyline

- consider MRI if persists despite above

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37
Q

Mechanism of action of Azathioprine?

A

Metabolsied to active compound mercaptopurine - purine analogue that inhibits purine synthesis
- TPMT test before starting to look for pts prone to azathioprine toxicity

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38
Q

Adverse effects of Azathioprine?

When to use a lower dose of Azathioprine?

A
  1. bone marrow suppression
  2. nausea/vomiting
  3. pancreatitis
    - If prone to toxicity, or if on Allopurinol (significant interaction)
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39
Q

What is Behcet’s syndrome?

A

complex multi system disorder ass with presumed autoimmune-mediated inflammation of arteries & veins

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40
Q

Behcet’s syndrome

  • commoner in which people?
  • ass with which alleles?
A
  • eastern Mediterranean, men, 30% have a +ve FHx

- HLA B5(1), MICA6

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41
Q

Features of Behcet’s syndrome?

A 
classic of triad of:
1. genital ulcers
2. oral ulcers
3. anterior uveitis
also:
- thrombophlebitis, DVT
- arthritis
- neuro: e.g. aseptic meningitis
- GI: abdo pain, diarrhoea, colitis
- erythema nodosum

Ocular involvement most feared complication - uveitis, retinal vasculitis, iridocyclitis, chorioretinitis

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42
Q

Dx of Behcet’s syndrome?

A
  • clinical

- +ve pathergy test is suggestive (needle prick puncture site comes inflamed with small pustule forming)

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43
Q

Important RFs for osteoporosis?

A
  • age
  • female sex
  • low BMI
  • currently smoking
  • ETOH XS
  • RA
  • Hx of glucocorticoid use
  • Hx of parental hip#

Others: sendentary; premature menopause; Caucasians & Asians; endocrine disorders inc: hyperthyroid, hypogonadism, GH deficiency, hyperPTH, DM; myeloma, lymphoma; GI: IBD, malabsorption, gastrectomy, liver disease; CKD; osteogenesis imperfecta, homocystinuria

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44
Q

Meds that may worsen osteoporosis (other than glucocorticoids)

A
  • SSRIs
  • antiepileptics
  • PPIs
  • glitazones
  • long term heparin Rx
  • aromatase inhibitors
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45
Q

Ix for 2ry causes of osteoporosis - when to test?

A
  • to exclude diseases that mimic osteoporosis e.g. osteomalacia, myeloma
  • identify cause of osteoporosis & contributory factors
  • assess risk of subsequent #
  • select the most appropriate form of Rx
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46
Q

Ix recommended for 2ry cause of osteoporosis recommended by NOGG?

Others IF indicated?

A
  • Hx & Ex
  • FBC, U&Es for Cr, ESR/CRP, LFTs for liver transaminases & ALP & albumin, Ca & phosphate, TFTs
  • DEXA: bone densitometry

Others:

  • lateral XR of L&T spine/DXA-based vertebral imaging
  • myeloma screen
  • 25OHD, PTH
  • testosterone, SHBG, FSH, LH, prolactin
  • Cushing’s tests
  • coeliac tests
  • isotope bone scan
  • markers of bone turnover
  • urine calcium excretion
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47
Q

Causes of drug-induced lupus?

A
  • Procainamide
  • Hydralazine
  • Isoniazid
  • Penicillamine
  • Minocycline
  • Phenytoin
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48
Q

Features of drug-induced lupus?

- Abs that are positive?

A
  • arthralgia, myalgia
  • skin & chest involvement common e.g. malar rash, pleurisy
  • ANA 100% +ve, dsDNA Negative
  • anti-histone Ab 80-90%
  • anti-Ro, anti-Smith +ve in 5%
  • normal complement
  • renal & neuro involvement unusual
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49
Q

What is avascular necrosis?

A
  • death of bone tissue 2ry to loss of blood supply -> bone destruction & loss of joint function
  • most commonly affects epiphysis of long bones
  • initially aSx then leads to pain in affected joint
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50
Q

Causes of avascular necrosis?

A
  • long-term steroid use
  • chemo
  • etoh XS
  • trauma
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51
Q

Ix of avascular necrosis?

A

XR - often initially normal, may show osteopenia & micro fractures; increased density of femoral head if hip; collapse of articular surface may result in the crescent sign
MRI - Dx Ix of choice - more sensitive than radionuclide bone scanning

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52
Q

How do bisphosphonates work?

A
  • analogues of pyrophosphate, a molecule that decreases demineralisation in bone
  • they inhibit osteoclasts by reducing recruitment & promoting apoptosis
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53
Q

4 Clinical uses of bisphosphonates?

A
  1. prevention & Rx of osteoporosis
  2. hypercalcaemia
  3. Paget’s disease
  4. metastatic bony pain
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54
Q

5 Adverse effects of bisphosphonates?

A
  1. oesophageal: -itis, ulcers esp alendronate
  2. osteonecrosis of jaw
  3. inc risk atypical stress fractures of proximal femoral shaft in pts taking alendronate
  4. acute phase response: fever, myalgia, arthralgia
  5. hypocalcaemia (due to reduced calcium efflux from bone, usually insignificant)
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55
Q

Duration of bisphophonate Rx varies according to level of risk - can recommend stopping bisphosphonates at 5 years if what?

A
  1. < 75yrs
  2. femoral Tscore > -2.5
  3. low risk acc to FRAX/NOGG
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56
Q

What is Still’s disease?

A

systemic-onset juvenile idiopathic arthritis

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57
Q 
Adult Still's disease:
age?
bloods &amp; Ab?
features?
Rx?
A
  • 16-35yrs
  • raised ferritin, RF -ive, ANA -ive
    1. arthralgia
    2. rash: salmon-pink, maculopapular
    3. fever: usually daily in late afternoon/early evening with worsening of joint Sx
    4. lymphadenopathy
  • NSAIDs for fever, joint pain, serositis at least 1 wk before considering adding steroids
  • Steroids may help Sx but don’t improve prognosis
  • can consider MTX, IL-1 or anti-TNF if Sx persist
  • Anakinra competitively inhibits the action of IL-1 by binding to the IL-1 receptor and plasma levels correlate well with IL-1 in synovial fluid and presence of synovitis.
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58
Q

What type of vasculitis is temporal arteritis?

A

Large vessel

  • overlaps with PMR
  • skip lesions on histology
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59
Q

Features of temporal arteritis?

A
  • rapid/subacute onset < 1month, pt > 60yrs old
  • headache, jaw claudication
  • tender, palpable temporal artery
  • visual disturbance 2ry to anterior ischaemic optic neuropathy
  • 50% have features of PMR: aching, morning stiffness in proximal limb muscles (not weakness)
  • also lethargy, depression, low-grade fever, anorexia,, night sweats
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60
Q

Ix in temporal arteritis?

A
  • ESR>50 (<30 in 10%), CRP may be raised
  • temporal artery Bx: skip lesions
  • CK & EMG normal
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61
Q

1ry causes of gout?

A

Decreased renal excretion of uric acid (90%)

  1. drugs: diuretics
  2. ckd
  3. lead toxicity

Increased production of uric acid

  1. myelo/lymphoproliferative disorder
  2. cytotoxic drugs
  3. severe psoriasis

Lesch-Nyhan syndrome: HGPRTase deficiency, x-linked recessive (only boys affected)
- gout, renal failure, neuro deficits, LD, self-mutilation

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62
Q

What is antiphospholipid syndrome?

Other features?

A

acquired disorder with a predisposition to:

  1. venous AND arterial thromboses
  2. recurrent fetal loss
  3. thrombocytopenia

Other:

  • livedo reticularis
  • prolonged APTT
  • pre-eclampsia
  • pulmonary hypertension
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63
Q

Why is there a paradoxical rise in APTT in antiphospholipid syndrome?

A
  • ex-vivor reaction of lupus anticoagulant autoAb with phospholipids involved in the coagulation cascade
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64
Q

Associations of antiphospholipid syndrome?

A

SLE
other autoimmune disorders
lymphoproliferative disorders
phenothiazines (rare)

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65
Q

Management of antiphospholipid syndrome:
initial VTE?
recurrent VTE?
arterial thrombosis?

A
  • initial VTE: Warfarin 6m target INR 2-3
  • recurrent VTE: Warfarin lifelong target INR 3-4
  • arterial: Warfarin lifelong target INR 2-3
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66
Q

When to offer bone protection for risk of glucocorticoid-induced osteoporosis without doing a DEXA?

A
  1. If you envision a patient taking Prednisolone 7.5mg OD for 3months+
    e. g. newly Dx PMR
  2. Over 65 who has had a previous fragility fracture
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67
Q

Managing pts at risk of glucocorticoid-induced osteoporosis - offer bone mineral density scan in under 65s - Rx if T-score is
>0?
0 to -1.5
< -1.5

A

T > 0 Reassure
T 0 to -1.5 Repeat DEXA in 1-3yrs
T < -1.5 Offer bone protection

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68
Q

What is pseudogout?

A

Microcrystal synovitis caused by deposition of calcium pyrophosphate dehydrate in the synovium

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69
Q

Risk factors for pseudo gout?

A
  • hyperPTH, hypothyroid, acromegaly
  • haemochromatosis, Wilsons disease
  • low Mg, low phosphate
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70
Q

Features of pseudo gout:
which joints?
on aspirate?
xray?

A
  • knee, wrist, shuolders
  • weakly-positive birefringent rhomboid shaped crystals
  • chondrocalcinosis
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71
Q

Rx of pseudo gout?

A
  • aspirate joint fluid to exclude septic arthritis
  • NSAIDs or
  • intra-articular/IM/PO steroids, as for gout
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72
Q

Osteomyelitis/Discitis - commonest cause?
commonest cause in patients with sickle-cell/Hbopathies?
Ix of choice?

A

Staph aureus
Salmonella
MRI sensitivity 90-100%

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73
Q

Rx of osteomyelitis?

A

Flucloxacillin 6 weeks

Clindamycin if allergic

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74
Q

Predisposing conditions for osteomyelitis?

A
  • DM
  • sickle cell
  • IVDU
  • etoh XS
  • immunosuppression e.g. HIV/meds
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75
Q

What is Marfan’s syndrome?

A
  • autosomal dominant CT disorder
  • defect in FBN1 gene on chr 15 that codes for protein fibrillin-1
  • affects 1/3000
  • leads to a defect of the glycoprotein structure which usually wraps around elastin
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76
Q

Features of Marfan’s syndrome?

A
  • tall stature, arm span:height > 1.05, scoliosis > 20degrees
  • high-arched palate, arachnodactyly, pectus excavatum, pes planus
  • heart: 90% aortic sinuses dilation which may lead to aneurysm, aortic dissection, A regurgitation, MVP 75%
  • lungs: repeated pneumothoraces
  • eyes: upward lens dislocation (superotemporal ectopia lentis_, blue sclera, myopia
  • dural ectasia = ballooning of the dural sac at the lumbosacral level - may cause back pain with neuro problems e.g. bladder & bowel dysfunction
  • life expectancy 40-50yrs improved by echo monitoring & beta blocker/ACE-I therapy
  • aortic dissection etc leading cause of death
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77
Q

Extra-articular complications of rheumatoid arthritis?

  • systemic
  • resp
  • cardio
  • neuro
  • ocular
  • haem
  • other
A
  • fatigue, depression, increased risk of infections, osteoporosis
  • fibrosis, effusion, nodules, MTX pneumonitis, pleurisy
  • IHD, pericarditis, conduction defects
  • carpal tunnel, mononeuritis, neuropathy
  • sicca, epi/scleritis, corneal ulcers, kerattis, steroid cataract, chloroquine retinopathy
  • normocytic anaemia, high platelets
  • Felty’s (splenomegaly, low WCC), amyloid
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78
Q

What is discoid lupus erythematous?

A
  • follicular keratin plugs, autoimmune

- benign, younger females, rarely progressive to SLE

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79
Q

Features of discoid lupus:
rash?
distribution?
heling?

A
  • photosensitive, erythematous, papular rash, sometimes scaly
  • face, neck, ears & scalp
  • lesions heal with atrophy, scarring & pigmentation
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80
Q

Management of discoid lupus

A

Cons: avoid sun exposure
1st topical steroid cream
2nd oral anti-malarials e.g. hydroxychloroquine

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81
Q

Ab Immunology of SLE

  • most specific?
  • also highly specific?
  • most sensitive?
  • other?
A
  • anti-Smith most specific (sens 30%)
  • anti-dsDNA (sens 30%)
  • ANA 99% +ve
  • RF 20% +ve
  • also anti-U1 RNP, SS-A (anti-Ro), & SS-B (anti-La)
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82
Q

Monitoring blood Ix of SLE?
inflam markers
complement
Ab

A
  • ESR (CRP usually normal during active disease therefore if raised consider underlying infection)
  • C3, C4a & C4b are low during active disease (formation of complexes leads to consumption of complement)
  • anti-dsDNA titres can be used for disease monitoring if present
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83
Q

What is T score

What is Z score

A

T: based on bone mass of young female population
Z: adjusted for age & sex (&ethnic factors?)

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84
Q

T score > -1.0?
-1.0 to -2.5?
< -2.5

A

normal
osteopaenia
osteoporosis

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85
Q

Most common site of hand OA?

A

trapeziometacarpal joint = base of thumb

86
Q

Management of osteoarthritis?

A

Cons:

  • weight loss, local muscle strengthening exercises, general aerobic fitness
  • supports, braces, TENS, shock-absorbing insoles/shoes

Med 1st: paracetamol
+ topical NSADs if OA knee/hand
2nd: oral NSAIDs/COX-2 inhib, opioids, capsaicin cream, intra-articular steroids + PPI

Surg: joint replacement

87
Q

Glucosamine in OA…
what is it?
role?

A

= normal constituent of glycosaminoglycans in cartilage & synovial fluid

  • may give some short-term Sx benefits in OA knee
  • but not recommended by NICE - studies mixed
88
Q

What is dermatomyositis?

What is polymyositis?

A
  • inflammatory disorder causing symmetrical, proximal muscle weakness & characteristic skin lesions
  • may be idiopathic or ass. with CT disorders, or underlying malignancy (e.g. ovarian, breast, lung)

polymyositis = variant of disease where skin manifestations are not prominent

89
Q

Skin features of dermatomyositis?

Other features?

A
  1. photosensitive
  2. macular rash over back & shoulder
  3. periorbital heliotrope rash
  4. Gottron’s papules - roughened red papillose over extensor surfaces of fingers
  5. nail fold capillary dilatation
  • Proximal muscle weakness +/- tenderness
  • Raynaud’s
  • resp muscle weakness
  • ILD e.g. fibrosing alveolitis or organising pneumonia
  • dysphagia, dysphonia
90
Q

Ix in dermatomyositis (Ab)?

A
  • majority ANA +ve
  • about 25% anti-Mi-2 +ve
  • muscle Bx: Perimysial inflammation of lymphocytes & parafascicular atrophy
91
Q

What is anti-synthetase syndrome?

A

Autoimmune disease ass with ILD, dermatomyositis, polymyositis

  • proximal myopathy
  • mechanic hands/ Raynaud’s
  • ILD
92
Q

What is mixed connective tissue disease = Sharp’s syndrome?

A

Rare, heterogenous, multi-system autoimmune disorder

  • features of SLE, systemic sclerosis, myositis may all be present
  • M:F 1:3
  • presents age 30-40s, may present in children
93
Q

What Ab is mixed CT disease associated with?

A

anti-U1 ribonucleoprotein (RNP) antibodies

94
Q

3 main features at presentation of mixed connective tissue disease?

Other clinically important?
derm
GI
resp
haem
cardiac
renal
neuropsych
A
  1. 90% Raynaud’s (often 1st)
  2. polyarthralgia/arthritis, myalgia
  3. dactylitis (sausage fingers)
  • photosensitive rash, scleroderma-like changes, alopecia
  • oesophageal dysfunction
  • pleuritis, pulm HTN, ILD
  • anaemia, lymphadenopathy, splenomegaly, rarely TTP
  • pericarditis, pericardial effusion, acc coronary artery disease
  • glomerulonephritis (tends to be milder than SLE)
  • seizures, mood disturbance
95
Q 
Ix in mixed CT disease?
1st
bloods
Ab
organ-specific
A

1st exclude other CT disease/vasculitis

  • anaemia, leucopenia, thrombocytopenia, renal impairment, raised ESR/CRP
  • ANA usually +ve
  • anti-dsDNA & scleroderma Ab -ive
  • Anti-U1 RNP (extractable nuclear Ag, ENA) MUST be +ve
  • ecg, echo, CT chest, MRI brain
96
Q

Management of mixed CT disease?

A
  • stop smoking, exercise
  • DMARDs/immunosuppression
  • calcium channel blockers for Raynaud’s
  • PPIs for reflux
  • endothelin r antagonists/ prostacyclin analogues for pulmonary HTN

1/3 long-term remit, 1/3 chronic Sx, 1/3 severe systemic involvement & premature death

97
Q

What is osteomalacia?

A

Decreased mineral content of bone, but normal bony tissue
= rickets if when growing
= osteomalacia if after epiphysis fusion

98
Q

Types of osteomalacia?

A
  • vit D deficiency
  • renal failure
  • drug-induced e.g. anticonvulsants, phenytoin
  • vit D resistant - inherited
  • liver disease e.g. cirrhosis
99
Q

Features of osteomalacia?

A

rickets - knock-knee, bow leg, features of hypocalcaemia

osteomalacia - bone pain, fractures, muscle tenderness, proximal myopathy

100
Q

Ix for osteomalacia:

  • bloods
  • imaging

Rx

A
  • low 25(OH) it D
  • raised ALP
  • low Ca, phosphate
    children XR: cupped, ragged metaphysical surfaces
    adults XR: translucent bands (LOOSERS ZONES/pseudofractures)

Rx: calcium with vit D

101
Q

What is Leflunomide?

A

= DMARD used in RA with a very long half-life

102
Q

Contra-indications of Leflunomide?

Caution

A

Pregnancy - Must be on effective contraception during Rx and at least 2 YEARS after Rx in women, 3yrs after Rx in men (monitor with plasma conc)
- caution if pre-existing lung & liver disease

103
Q

Adverse effects of Leflunomide?
Monitoring?
Stopping?

A
  • GI esp diarrhoea
  • HTN
  • weight loss, anorexia
  • peripheral neuropathy
  • myelosuppression
  • pneumonitis

Monitor FBC, LFT & BP

Leflunomide has a v long wash-out period of up to a year which requires co-administration of cholestyramine

104
Q

Which groups should take vitamin D supplementation?

A
  • pregnant & breastfeeding 10 mcg vit D
  • children aged 6m-5yrs (formula milk is fortified with it D so if taking >500ml/day, don;t need to take a supplement)
  • adults >65yrs
  • ‘people not exposed to much sun’
105
Q

When may testing for vitamin D deficiency be appropriate?

A
  • pts with bone diseases that may be improved with vit D Rx e.g. known osteomalacia, Paget’s
  • pts with bone diseases, prior to specific Rx where correcting vitamin deficiency is appropriate e.g. prior to IV zolendronate/denosumab
  • pts with MSK Sx that could be attributed to vit D deficiency e.g. bony pain

Pts with osteoporosis shouldn’t be tested - they should always be given calcium/vit D
People at higher risk should be treated anyway

106
Q

What is ankylosing spondylitis?

What are its main features?

A
  • HLA-B27 ass spondyloarthropathy
  • males aged 20-30
  • typically young man who presents with lower back pain & stiffness of insidious onset
  • stiffness worse in AM, improves with exercise
  • may experience night pain which improves on getting up
107
Q

Clinical features in ankylosing spondylitis?

A
  • reduced lateral flexion
  • reduced anterior flexion (Schobers test)
  • reduced chest expansion
108
Q

Other features of ankylosing spondylitis (8A’s)?

A 
Apical fibrosis
Anterior uveitis
Aortic regurgitation
Achilles tendonitis
AV node block
Amyloidosis
peripheral Arthritis
And cauda equina syndrome
109
Q

What is Reactive arthritis?

A

HLA-B27 ass seronegative spondyloarthropathy of arthritis that develops following an infection where the organism canNOT be recovered from the joint

includes Reiter's syndrome:
1. urethritis
2. conjunctivitis
3. arthritis
following dysentery (1:1) during WWII, or STI (men 10:1)
110
Q

Management of reactive arthritis

A

Sx: analgesia, NSAIDs, intra-articular steroids
Med if persistent: sulfasalazine, MTX
Sx rarely last >12m

111
Q

Pregnancy risk in SLE?

Strongly ass with which Ab?

A
  • risk of maternal autoAb crossing placenta
  • leads to neonatal lupus
  • complications inc congenital heart block
  • anti-Ro (SSA) Ab
112
Q 
Extractable nuclear antigens are specific nuclear Ag usually associated with being ANA positive
What are the following ass with?
anti-Ro
anti-La
anti-Jo1
Anti-scl-70
anti-centromere
A 
anti-Ro - Sjogren's, SLE, congenital heart block
anti-La - Sjogren's
anti-Jo 1 - polymyositis
anti-scl-70 - diffuse sclerosis
anti-centromere - limited sclerosis
113
Q

What is Ehler-Danlos syndrome?

A
  • autosomal dominant CT disorder
  • affects type III collagen
  • elastic tissue -> joint hyper mobility & inc elasticity of skin
114
Q

Features & complications of Ehlers-Danlos?

A
  • elastic, fragile skin with easy bruising
  • joint hyper mobility - rec joint dislocation
  • aortic regurg, MVP, aortic dissection
  • SAH
  • angioid retinal streaks
115
Q

What are the common features seronegative spondyloarthropathies?

4 main spondyloarthropathies?

A
  • RF negative
  • ass with HLA-B27
  • peripheral arthritis usually asymmetrical
  • sacroiliitis
  • enthesopathy: Achilles tendonitis, plantar fasciitis
  • extra-articular: uveitis, upper zone lung fibrosis, amyloid, aortic regurg
  1. ank spond
  2. psoriatic arthritis
  3. Reiter’s syndrome inc reactive arthritis
  4. enteropathic arthritis (ass with IBD)
116
Q

Adverse effect of hydroxychloroquine?

Monitoring?

A

Bull’s eye retinopathy - may lead to severe & permanent visual loss

  • more common than previously thought
  • RCOphthal suggest colour retinal photography & spectral domain optical coherence tomography scanning of the macula
  • monitoring: ask about visual Sx & monitor visual acuity annually using standard reading chart
117
Q

Drug causes of Gout?

A
  • diuretics: thiazide, furosemide
  • ciclosporin
  • etoh
  • cytotoxics
  • pyrazinamide
  • aspirin high dose
118
Q

Rheumatoid factor is usually what kind of circulating Ab?

How is it detected?Significance of high titre levels?

A

IgM to the Fc portion of pt’s own IgG

  • Rose-Waaler test: sheep red cell agglutination
  • Latex agglutination test less specific

High titre levels ass with severe progressive disease (but NOT a marker of disease activity)

119
Q

What is pseudoxanthoma elasticum?

Features?

A
  • inherited usually autosomal recessive, characterised by abnormality in elastic fibres
  • retinal angioid streaks
  • ‘plucked chicken skin’ appearance of small yellow papillose on neck, antecubital fossa, axillae
  • heart: MVP, increased risk IHD
  • GI haemorrhage
120
Q 
What is carpal tunnel syndrome?
Hx features?
Ex features?
Causes?
Electrophysiology?
Rx?
A
  • median nerve compression
  • pain, pins & needles in thumb, index & middle finger; Sx may ascend proximally; pt shakes hand for relief
  • weakness of thumb abduction (abductor policies brevis)
  • wasting of thenar eminence
  • Tinel’s
  • Phalen’s
  • idiopathic, pregnancy, oedema, RA, lunate #, obesity
  • motor & sensory: prolongation of the action potential
  • Rx with corticosteroid injection, wrist splints at night, surgical decompression (flexor retinaculum division)
121
Q 
Rheumatoid arthritis:
prevalence?
F:M?
peak onset?
HLA association?
A

1%
F:M 3:1
HLA-DR4 (esp Felty’s)

122
Q

Poor prognostic features of rheumatoid arthritis?

A
  • RF +ve, anti-CCP Ab, HLA DR4
  • insidious onset, poor functional status at presentation
  • early erosions on X-ray
  • extra-articular features
123
Q

What is polyarteritis nodosa?

Who is it associated with?

A

Vasculitis affecting medium-sized vessels with necrotising inflammation leading to aneurysm formation
- more common in middle-aged men & ass with hep B infection

124
Q

Features of polyarteritis nodosa?

  • systemic
  • cardiac
  • neuro
  • skin
  • uro/renal/GU
  • Ab & blds
A
  • fever, malaise, arthralgia, weight loss
  • HTN
  • mononeuritis multiplex, sensorimotor polyneuropathy
  • livedo reticularis
  • haematuria, renal failure, testicular pain
  • p-ANCA 20% in those with ‘classic’ PAN, hep B serology +ve in 30%
125
Q

What is polymyositis?
how is it mediated?
what are it’s associations?
typical patients?

A

Inflammatory disorder causing symmetrical, proximal muscle weakness

  • T-cell mediated cytotoxic process directed against muscle fibres
  • idiopathic/ass with CT disorders
  • ass with malignancy
  • dermatomyositis = variant where skin manifestations are prominent e.g. a purple (heliotrope) rash on cheeks & eyelids
  • middle-aged, F:M 3:1
126
Q

Features of polymyositis?

A
  • proximal muscle weakness +/- tenderness
  • Raynaud’s
  • resp muscle weakness
  • ILD (major RF for premature death)
  • dysphagia, dysphonia
127
Q

Ix in polymyositis:
muscle enzymes?
Ab?
other Ix?

A
  • raised CK, also LDH, aldodase, AST & ALT can be raised
  • anti-Jo-1 Ab seen in pattern ass with lung involvement, Raynaud’s & fever - it is a predictor of ILD at Dx
  • EMG
  • muscle Bx: endomysial lymphocytic infiltrates that invade nonnecrotic muscle fibres
128
Q

What is familial Mediterranean fever?

A

= Recurrent polyserositis

  • autosomal recessive
  • usually presents in 2nd decade
  • more common in Turkish/Armenian/Arabic
129
Q

Features of familial Mediterranean fever?

Rx?

A
  • attacks usually last 1-3days
  • fever, abdo pain (peritonitis)
  • pleurisy, pericarditis
  • arthritis, erysipeloid rash on lower limbs

Colchicine can help

130
Q

Factors with Raynaud’s suggesting underlying CT disease?

A
  • onset after 40yrs
  • unilateral Sx
  • rash
  • autoAb
  • digital ulcers, calcinosis
  • features to suggest RA/SLE
  • v rarely chillblains
131
Q

2ry causes of Raynaud’s?

A
  • CT disorders: scleroderma (commonest), RA, SLE
  • leukaemia
  • type I cryoglobulinaemia, cold agglutinins
  • use of vibrating tools
  • cervical rib
  • OCP, ergot
132
Q

Rx of Raynaud’s?

A

1st Ca++ ch bl e.g. nifedipine

2nd IV prostacyclin infusions: effects may last several weeks

133
Q

ACR 1990 criteria for Dx of giant cell arteritis?

A

3 of 5:

  1. age > 50
  2. new onset localised headache
  3. temporal artery tenderness or decreased pulsation
  4. ESR > 50
  5. temporal artery Bx positive
134
Q

Management of GCS:

  • uncomplicated?
  • complicated (with visual involvement &/or jaw claudication)
A
  • High-dose Pred 40-60mg OD until Sx & Ix normalise
  • IV methylpred 500-1000mg 3/7 before starting PO Pred
  • bone protection & PPI
  • should be dramatic response
  • Urgent ophthal R/V - Same day if visual Sx - irreversible
135
Q

What is de quervain’s tenosynovitis?

A

sheath containing extensor pollicis brevis & abductor pollicis longus tendons becomes inflamed
- typically affects females 30-50yrs

136
Q

Features of de quervain’s tenosynovitis?

A
  • radial side wrist pain
  • tenderness over radial styloid process
  • thumb abduction against resistance is painful
  • Finkelstein’s test: with thumb flexed across palm of hand, pain is reproduced by movement of the wrist into flexion & ulnar deviation
137
Q

Management of de quervain’s tenosynovitis?

A
  • analgesia
  • steroid injection
  • immobilisation with thumb splint may help
  • sometimes surgical Rx required
138
Q

What is cytoplasmic ANCA’s most common target?
can it be used to monitor disease activity?
ass. diseases?

A
  • serine proteinase 3 (PR3)
  • some correlation between cANCA levels & disease activity
  • Wegener’s/GPA +ve >90%
  • microscopic polyangitis +ve 40%
139
Q

What is the most common target of perinuclear ANCA?

  • can it be used to monitor disease activity?
  • ass. diseases?
A
  • MPO: myeloperoxidase commonest target
  • canNOT use level with disease activity
  • immune crescenteric glomerulonephritis +ve 80%
  • microscopic polyangitis +ve 50-75%
  • Churg-Strauss syndrome +ve 60%
  • 1ry sclerosing cholangitis +ve 60-80%
  • granulomatosis with polyangitis +ve 25%
  • (also IBD UC>Crohns, CT disorders, autoimmune hepatitis)
140
Q

5 types of psoriatic arthropathy? drasa

A
  • DIPJ disease
  • Rheumatoid-like polyarthritis (30-40%, commonest)
  • Arthritis mutilans
  • Sacroilitis
  • Asymmetrical oligoarthritis typically affecting hands & feet (20-30%)
141
Q

Rx of psoriatic arthropathy?

A
  • treat as RA but better prognosis
  • often precedes skin lesions
  • M:F 1:1
  • 10-20% of pts with skin lesions develop an arthropathy
142
Q

What is chronic fatigue syndrome?

Epidemiology ?

A
  • Dx after at least 4months of disabling fatigue affecting mental & physical function >50% of the time in the absence of other disease which may explain Sx
  • more common in females
  • past psych Hx not been shown to be a RF
143
Q

Features of chronic fatigue syndrome except fatigue?

A
  • Sleep affected
  • muscle & joint pains
  • headaches, malaise, ‘flu-like Sx’
  • sore throat, painful LNs without enlargement
  • dizziness, palpitations
  • physical/mental exertion makes Sx worse
  • cognitive dysfunction
144
Q

Ix before Dx chronic fatigue syndrome

A
  • FBC, U&E, LFT, CRP, ESR

- TFT, glucose, calcium, CK, coeliac screen, urinalysis

145
Q

Rx of chronic fatigue syndrome

A
  • CBT (NNT=2)
  • graded exercise therapy (formal programme)
  • ‘pacing’ - organising activities to avoid tiring
  • low-dose amitriptyline may be useful for poor sleep
  • pain clinic referral if pain predominant
146
Q

Adhesive capsulitis/frozen shoulder:
association?
features?
management?

A
  • diabetes mellitus (20%)
  • EXTERNAL rotation affected > IR/abduction
  • active & passive movement affected
  • typically a painful freezing phase, an adhesive phase, a recovery phase
  • bilateral in upto 20% of pts
  • episode typically lasts 6m-2yrs
  • no single intervention shown to improve outcome in long-term
  • Rx options inc: NSAIDs, PT, oral & intra-articular steroids
147
Q

Shoulder pain ass with popping/swelling/clicking/grinding & +ve scarf test…?

A

Acromioclavicular degeneration

148
Q

Shoulder pain on overhead activities with painful arc of abduction +/- popping/snapping/grinding?

A

Subacromial impingement

149
Q

Xray features of ankylosing spondylitis?

A
  • subchondral erosions
  • sclerosis
  • squaring of lumbar vertebrae
150
Q

What is Paget’s disease?

A
  • increased uncontrolled bone turnover
  • disorder of osteoclasts with excessive osteoclast resorption followed by increased osteoblastic activity
  • 5% UK prevalece, Sx in 1/20
151
Q

Predisposing factors of Paget’s disease?

A
  • increasing age
  • male sex
  • northern latitude
  • FHx
152
Q

Clinical features of Paget’s disease:
Sx?
blds?
xr?

A
  • bony pain
  • if untreated: bowing of tibia, bossing of skull
  • raised ALP+++, Ca & phosphate normal
  • skull XR: thickened vault, osteoporosis circumscripta
153
Q

Paget’s disease:
indications for Rx?
Rx?

A
  • bone pain, skull/long bone deformity, fracture, periarticular Paget’s
  • bisphosphonate (PO risedronate or IV zoledronate)
  • calcitonin less commonly used now
154
Q

Complications of Paget’s disease?

A
  • deafness (cranial nerve entrapment)
  • bone sarcoma (1% if affected >10yrs)
  • fractures, skull thickening
  • high-output cardiac failure
155
Q

What is the most specific Ab in lupus?

A

anti-Smith

156
Q

What Ab can be used to correlate with disease activity in lupus?

A

anti-dsDNA

157
Q

Most sensitive Ab in lupus?

A

ANA

158
Q

What happens to RA Sx in pregnancy?

After delivery?

A
  • they tend to improve in pregnancy

- but flare after delivery

159
Q 
When to stop methotrexate in pregnancy?
Is leflunomide safe in pregnancy?
Which drugs are considered safe?
what about steroids?
what about NSAIDs?
A
  • stop mtx at least 3 months before conception
  • leflunomide is safe
  • sulfasalazine & hydroxychloroquine considered safe in pregnancy
  • low-dose steroids can be used in pregnancy to help control Sx
  • NSAIDs can be used until 32/40 but then stopped - due to risk of early closure of ductus arteriosus
  • pts need obstetric anaesthetist referral due to risk of atlanto-axial subluxation
160
Q

Most specific autoAb for dermatomyositis?

A

anti-Mi-2

161
Q

Most common autoAb in dermatomyositis?

A

ANA

162
Q

What is Sjogren’s syndrome?

A
  • an autoimmune disorder affecting exocrine glands in dry mucosal surfaces
  • 1ry or 2ry to e.g. RA (develops 10yrs after initial onset)
  • F:M 9:1
163
Q

Marked increase of WHAT in Sjogren’s syndrome?

A

lymphoid malignancy (40-60X)

164
Q

Features of Sjogren’s syndrome?

A
  • dry eyes (sicca), dry mouth dry vagina
  • arthralgia, myalgia, Raynaud’s
  • recurrent parotitis
  • sensory polyneuropathy
  • renal tubular acidosis (usually subclinical)
165
Q

Most sensitive Ab in Sjogren’s?

A

RF (like 100%)

ANA 70%

166
Q

Histology in Sjogren’s syndrome?

What is Schirmer’s test?

A
  • focal lymphocytic infiltration

- filter paper near conjunctival sac to measure tear formation

167
Q

Bloods in Sjogren’s syndrome except for Ab?

A
  • low C4

- hypergammaglobulinaemia

168
Q

Rx of Sjogren’s syndrome?

A
  • artificial salive & tears

- PILOCARPINE may stimulate saliva production

169
Q

Xray changes in rheumatoid arthritis?

A
  • loss of joint space
  • juxta-articular osteoporosis
  • soft-tissue swelling
  • periarticular erosions
  • subluxation
170
Q

What is tumour necrosis factor?

What cells secrete TNF?

A
  • pro-inflammatory cytokine

- secreted by macrophages

171
Q 
What are the effects of TNF?
Immune
Endothelial
Systemic
RA
TNF-alpha
A

Immune system effects:

  • activates macrophages & neutrophils
  • costimulator for T cell activation
  • key mediator of body’s response to Gram -ve septicaemia
  • similar properties to IL-1
  • anti-tumour effect e.g. phospholipase activation

Endothelial effects:
- increases expression of selecting & increased production of platelet-activating factor, IL-1 & prostaglandins

Promoties proliferation of fibroblasts & their production of protease & collagenase

Systemic effects: pyrexia, increased acute phase proteins, disordered metabolism leading to cachexia

RA pathogenesis - TNF blockers can help

TNF-alpha: binds to p55 & p75 - these receptors can induce apoptosis; also causes activation of NFkB

172
Q

TNF blockers - adverse effects?
infliximab
etanercept
adalimumab

A
  • reactivation of latent TB, demyelination
    infliximab: mAb, IV
    etanercept: fusion protein that mimics inhibitory effects of naturally occurring soluble TNF r’s, SC
    adalimumab: mAb, SC
173
Q

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

A

Lupus anticoagulant

174
Q

anti-ribonuclear protein (anti-RNP)

think what?

A

mixed CT disease

175
Q

Cautions of sulfasalazine (dmard):
condition?
other drugs?

A
  • gp6d deficiency

- aspirin or sulphonamides allergy (X-sensitivity)

176
Q

Adverse effects of sulfasalazine?

A 
oligospermia
Steven-Johnsons syndrome
pneumonitis, lung fibrosis
myelosuppression, Heinz body anaemia, megaloblastic anaemia
may colour tears/stains CL
177
Q

What is osteopetrosis?
blds?
Rx?

A

= marble bone disease

  • rare disorder of defective osteoclast function -> failure of normal bone resorption
  • results in dense, thick bones prone to fracture
  • bone pains & neuropathies common
  • normal calcium, phosphate, ALP
  • stem cell Tx & IFN-gamma have been used for Rx
178
Q

What is fibromyalgia?
Features?
Dx?
Rx?

A

syndrome of widespread pain throughout body with tender points at specific sites, cause known
- women, 30-50yrs

  • chronic pain at multiple sites
  • lethargy
  • cognitive impairment ‘fibro fog’
  • sleep disturbance, headaches, dizziness common
  • 11/18 points tender, but Dx clinical
  • explanation
  • aerobic exercise - strongest evidence base
  • CBT
  • meds: pregabalin, duloxetine, amitriptyline
179
Q

Hip pain exacerbated by exercise, relieved by rest
1st sign reduction in internal rotation
RFs inc age, obesity, previous joint problems

A

Osteoarthritis

180
Q

Hip pain worse 1st thing in AM, with systemic features & raised inflammatory markers?

A

Inflammatory arthritis

181
Q

Hip pain with positive femoral nerve stretch test?

A

Referred lumbar spine pain

182
Q

Hip pain due to repeated movement of the fibroelastic iliotibial band with pain & tenderness over lateral side of thigh, most common in women aged 5-70yrs

A

Greater trochanteric pain syndrome = trochanteric bursitis

183
Q

Hip pain caused by compression of lateral cutaneous nerve of thigh - typically a burning sensation over anterolateral aspect of thigh

A

Meralgia paraesthetica

184
Q

Gradual/sudden onset hip pain that may follow high dose steroid therapy or previous hip fracture/dislocation?

A

Avascular necrosis

185
Q

Hip pain common in pregnancy with radiation to groins & medial thighs - waddling gait may be seen

A

Pubic symphysis dysfunction - ligament laxity increases in response to hormonal changes of pregnancy

186
Q

Uncommon condition of hip pain sometimes seen in 3rd trimester, & going pain ass with limited range of movement in hip - may be unable to weight bear - ESR may be elevated

A

Transient idiopathic osteoporosis

187
Q

Xray features of ankylosing spondylitis?

A
  • Sacroilitis: subchondral erosions, sclerosis
  • Squaring of lumbar vertebrae
  • Syndesmophytes: due to ossification of outer fibres of annulus fibrosus-> dagger sign
  • ‘bamboo spine’: late & uncommon
  • CXR: apical fibrosis
188
Q

Management of ankylosing spondylitis?

A

Cons: exercise, physio
Med 1st: NSAIDs
- DMARDs only if there’s peripheral joint involvement
- Anti-TNF therapy if persistently high disease activity despite conventional treatments

189
Q

What do TNF-inhibitors improve in ankylosing spondylitis?

what will not be improved?

A
  • quality of life
  • spinal mobility
  • extra-articular features
  • early morning stiffness
  • does NOT help with radiological progression… which correlates with spinal mobility & overall physical function
190
Q

Mechanism of action of methotrexate?

Indications?

A
  • antimetabolite that inhibits dihydrofolate reductase (essential for purine & pyrimidine synthesis)
  1. inflammatory arthritis esp RA
  2. psoriasis
  3. some chemo e.g. ALL
191
Q

Adverse effects of methotrexate?

Monitoring?

A 
mucositis
pneumonitis
myelosuppression
pulmonary fibrosis
liver cirrhosis

(inc risk of marrow aplasia if co-prescribed with trimethoprim/cotrimoxazole)

192
Q

Radiological features of gout?

A
  • joint effusion early
  • well-defined ‘punched-out’ erosions with sclerotic margins in a junta-articular distribution, often with overhanging edges
  • relative preservation of joint space intimate disease
  • eccentric erosions
  • no periarticular osteopaenia
  • soft tissue top may be seen
193
Q

What is osteogenesis imperfecta?

A

= brittle bone disease

  • disorders of collagen metabolism resulting in bone fragility & fractures
  • commonest & mildest form = type 1
  • autosomal dominant
  • abnormality in type 1 collagen due to decreased synthesis of pro-alpha 1/2 collagen polypeptides
194
Q

Features of osteogenesis imperfecta?

A
  • childhood presentation
  • fractures following minor trauma
  • blue sclera
  • deafness 2ry to otosclerosis
  • dental imperfections are common
195
Q

Cause of acute/chronic lower back pain worse in the morning & on standing - typically worse on back extension

A

Facet joint pain

196
Q

Gradual onset lower back pain +/- UL/BL leg pain, numbness, & weakness worse on walking

  • resolves when sits down
  • ‘aching’/’crawling’
  • relieved by sitting down, leaning forwards & crouching down
  • often normal clinical exam
  • MRI to confirm Dx
A

spinal stenosis

197
Q

‘Double contour’ sign on joint aspiration?

A

GOUT
= hyperechoic, irregular band over the superficial margin of the joint cartilage, produced by deposition of monosodium urate crystals on the surface of the hyaline cartilage, which increases the interface of the cartilage surface, reaching a thickness similar to the subchondral bone

198
Q

Adult Xray: translucent bands (LOOSERS ZONES/pseudofractures) - Dx?

A

Osteomalacia

199
Q

arthralgia, myositis and Raynaud’s

what test to confirm Dx?

A

Anti-RNP

mixed CT disease

200
Q

Muscle Bx: Inflammatory infiltrates and inclusions within muscle fibres

A

Inclusion body myositis

201
Q

Non-painful non-erosive arthropathy in lupus

A

Jaccoud’s

202
Q

Polymyalgia rheumatica

  • histology?
  • features?
  • Ix?
  • Rx?
A
  • vasculitis with giant cells, ‘skip’ lesions of affected artery, muscle bed arteries affected most
  • pt>60, rapid onset <1month, aching & morning stiffness in proximal muscles
  • also mild polyarthralgia, lethargy, depression, low-grade fever, anorexia, night sweats
  • ESR>40, reduced CD8+ T cells
  • CK & EMG normal
  • Pred 15 OD
203
Q

Dx Ix for PAN?

A
  • renal angiography
204
Q

Middle-aged man with fever, lethargy, neuropathy, testicular pain, renal dysfunction & livedo reticularis - Dx?

A

Polyarteritis nodosa

205
Q

What to do if new oral ulceration starts whilst on MTX?

A

Stop MTX

D/w rheum

206
Q

What drug can precipitate a scleroderma renal crisis? Sep if already has HTN

A

Steroids !

207
Q

Which gout Rx has a severe interaction with Azathioprine leading to BM suppression?

A

Allopurinol

208
Q

1st lien antihypertensive in scleroderma

A

ACE-I

209
Q

Diabetic amyotrophy = proximal diabetic neuropathy

- features?

A
  • pain 1st eg in hips/buttocks

- then weakness

210
Q

Cutaneous manifestations of systemic sclerosis, grade 4 hypertensive retinopathy & heart failure

  • Dx?
  • Rx?
A

scleroderma renal crisis

- give ACE-I

EXAM (21 decks)

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