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MRCP > Rheumatology > Flashcards

Rheumatology Flashcards

1
Q

Which HLA is associated with Behcets Syndrome?

A

HLA B51

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2
Q

Which age group is temporal arteritis most common in?

A

Only >50s

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3
Q

How does temporal arteritis present?

A

Headache
Temporal artery (palpable) + scalp tenderness
Jaw claudication (pain after 10-15th bite)
Amaurosis fugax/sudden blindness
Around 50% have symptoms of PMR also

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4
Q

Investigations for temporal arteritis?

A

Bloods - raised ESR/CRP
Temporal artery biopsy - may be normal d/t skip lesions
Temporal artery US - halo sign, as sensitive as biopsy but often not available

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5
Q

Management of temporal arteritis?

A

Steroids @ 40mg
If visual symptoms, @ 60mg or IV methylprednisolone
Treatment often continues for 1-2 years
Urgent ophthalmology review
There should be a dramatic response, if not consider other Dx

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6
Q

What is the biggest cause of morbidity in temporal arteritis?

A

Long-term steroid treatment so consider bisphosphonates/PPI

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7
Q

Demographic for PMR?

A

Age >50yrs, often women

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8
Q

Symptoms of PMR?

A

Subacute onset (<2 weeks) of bilateral aching -> Worse in morning, improves with activity
Tenderness and morning stiffness in shoulders and proximal limb muscles (NO weakness) +/- mild polyarthritis
Tenosynovitis
Low grade fever + fatigue + WL
Carpel tunnel syndrome (10%)
Temporal arteritis symptoms

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9
Q

Investigations for PMR?

A

Raised CRP +/- ESR
ALP raised in 30%
CK is normal

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10
Q

Management of PMR?

A

Prednisolone 15mg
Dramatic response
Continue for >2yrs + bisphosphonates/PPI

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11
Q

What is the pathophysiology of dermato/polymyositis?

A

capillary obliteration causes ischaemia and muscle infarction

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12
Q

Symptoms of dermato/polymyositis?

A

Progressive, symmetrical, proximal muscle weakness
Their bulk is greater than expected from weakness – important sign

Skin features (only in Dermatomyositis)
Photosensitive
Macular rash over back and shoulders – shawl distribution
Heliotrope rash – periorbital
Gottron’s sign – roughened red papules over extensor surfaces of fingers

Other - Raynaud’s, ILD, resp muscle weakness

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13
Q

Investigations for dermato/polymyositis?

A

Autoantibodies - o Anti-Jo1 (poly), anti-Mi-2 (derm), ANA (most sensitive, not specific)
Raised serum muscle enzymes - CK, aldolase
Muscle biopsy – shows muscle necrosis, phagocytosis of muscle fibres + inflam infiltrates
Screen for malignancy

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14
Q

Management of dermato/polymyositis?

A

Systemic steroids - prednisolone
Immunosuppressive drugs – methotrexate
<10% of cases are fatal

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15
Q

What is Langerhans cell histiocytosis?

A

Langerhans cell histiocytosis is a rare condition associated with the abnormal proliferation of histiocytes (phagocytic cell). It typically presents in childhood with bony lesions

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16
Q

Features of Langerhans cell histiocytosis?

A

bone pain, typically in the skull or proximal femur, cutaneous nodules, recurrent otitis media/mastoiditis

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17
Q

Diagnosis of Langerhans cell histiocytosis?

A

Electronmicroscopy - tennis racket-shaped Birbeck granules

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18
Q

Is RA more common in M or F?

A

Females >2:1

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19
Q

Which HLA is RA associated with?

A

HLA DR4 (main), also HLA DR1

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20
Q

What are the RFs for RA?

A

smoking + genetics (combination is v significant)

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21
Q

Joint presentation of RA?

A

Symmetrical, swollen, painful and stiff small joints of the hands, wrist and feet
• Worse in the morning
• Larger joints may become involved
• Does not affect DIP joints or spinal involvement
Fatigue, more likely to get carpel tunnel

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22
Q

Signs of deformity in RA?

A

Boutonniere deformity
Ulnar deviation of MCP joints
Swan-neck deformity of fingers

Z deformity of the thumb

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23
Q

Extra-articular presentation of RA?

A

Splenomegaly (+low WCC and RA = Felty’s syndrome)
Nodules - elbow, lung, heart valve, achilles tendon
Eye - episcleritis/scleritis
Lung - fibrosis, pleuritis, pleural effusion

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24
Q

What is RA a significant RF for?

A

CVS disease - many of RA patients are on NSAIDs (increased risk), more likely to be inactive (increased risk)

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25
Q

What investigation should you do prior to surgery for RA?

A

X-ray of cervical spine to check for atlantoaxial subluxation - impt for intubation

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26
Q

Autoantibodies for RA?

A

RhF +ve 70% (not specific, cheaper test than anti-CCP)
Anti-CCP 98% (also +ve up to 10yrs before getting RA)
Worse prognosis with +ve RhF and anti-CCP

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27
Q

XR findings in RA?

A

SOLE
Soft tissue swelling
Osteopaenia
Loss of joint space
Erosions

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28
Q

What advice should you give to women taken methotrexate who want to conceive?

A

methotrexate is not safe in pregnancy and needs to be stopped at least 6 months before conception

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29
Q

Which RA medications are safe in pregnancy?

A

sulfasalazine, azathioprine and hydroxychloroquine

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30
Q

If a patient has an allergy to aspirin, which DMARD medication should be avoided?

A

Sulfasalazine
- should also be cautious giving sulfasalazine to patients with G6PD

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31
Q

Which HLA is ank spon associated with?

A

HLA B27

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32
Q

Symptoms of ank spon?

A

Backache and stiffness

  • worse after inactivity
  • begins in lumbar region -> thoracic
  • may develop question mark posture in longterm
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33
Q

Signs of ank spon?

A

Schober’s test - <5cm increase
Reduced lateral flexion
Chest expansion <5cm

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34
Q

7A’s of extra-articular features of ank spon?

A
  • Atlanto-axial subluxation (C1 + C2 move out of shape)
  • Anterior uveitis
  • Apical lung fibrosis
  • Aortic regurgitation
  • Amyloidosis
  • Achilles tendonitis
  • Autoimmune bowel disease
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35
Q

Investigations in ank spon?

A

Raised ESR/CRP
Hypergammaglobulinaemia
XR:
Sacroiliac joints - erosion, sclerosis
Squaring of the lumbar spine - give bamboo spine (becomes osteoporotic and risk of microfractures on movement)
Syndesmophytes

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36
Q

Scoring systems in ank spon?

A

BASDAI (disease activity index)
BASFI (functional index)
Scores need to be >4 on 2 occasions 3 months apart despite NSAIDS

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37
Q

Management of ank spon?

A

General - patient education, stop smoking, exercise, PT
Therapy - NSAIDS for 3 months, local steroids (systemic steroids won’t help spine but may help surrounding features), Anti-TNF therapy

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38
Q

Which category of medication will not help in ank spon?

A

DMARDs

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39
Q

Male to Female ratio for Gout?

A

M:F 4:1

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40
Q

Cause of Gout?

A

Deposition of monosodium urate crystals in and near joints

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41
Q

Causes of increased crystal deposition in Gout?

A

Increased production – hereditary, leukaemia/lymphomas (tumour lysis syndrome), cytotoxic drugs, increased dietary purines
Impaired excretion - diuretics, CKD, alcohol excess (beer)

-> Diuretics may make your body fluid more concentrated (so higher conc of uric acid), some diuretics also impair the excretion of uric acid

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42
Q

What can precipitate Gout flare ups?

A

Trauma, surgery, starvation, infection or diuretics

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43
Q

RFs for Gout?

A

Male, meat + seafood (high in purines), alcohol, diuretics, obesity, HTN, coronary heart disease, DM, CKD, heart failure

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44
Q

Presentation of Gout?

A

Acute pain, swollen, hot, erythematous joint, reaching a peak after 6-12hrs
Often with fever + malaise
Primary site – MTP joint
Other sites – knee, midtarsal joints, wrists, ankles, small hand joints

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45
Q

What type of crystals will you see on polarised light microscopy following aspiration of a joint with Gout vs Pseudogout?

A

Crystals:
Strongly negatively birefringent needles = gout
Rhomboid positively birefringent = pseudogout

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46
Q

What happens to the urate levels in your body in Gout?

A

Serum urate - may be raised/normal, useful to see response to Tx
Urine uric acid - may be raised

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47
Q

XR findings in chronic Gout?

A

punched-out lesions may be seen, juxta-articular erosions

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48
Q

USS/CT/MRI findings in Gout?

A

can identify urate deposition, structural joint damage and joint inflammation

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49
Q

Management of Gout?

A

Untreated, resolves over 5-15 days
With treatment, lasts about 3-5 days

General - RICE, continue prophlaxis therapy
NSAIDs - 1st line
Colchicine - if NSAIDs poorly tolerated/HF/on anticoagulation
Steroids - useful if NSAIDs + Colchicine are CI

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50
Q

Prevention of Gout?

A

urate lowering therapy: allopurinol (do not start until after acute attack) or febuxostat (more potent and expensive)
Also lifestyle modifications

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51
Q

What is the main SE of colchicine?

A

Diarrhoea

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52
Q

Most common demographic in SLE?

A

F:M 9:1, commoner in Afro-Caribbean + Asians, age 20-40yrs

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53
Q

What may trigger SLE?

A

Infection, injury, stress, EBV, drug induced

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54
Q

Which drugs may cause drug-induced SLE and which Ab is it associated with?

A

SHIPP (Sulfonamide (abx), hydralazine (HF), isoniazid (TB), phenytoin (seizures), procainamide (arrhythmia))

Anti-histone Abs in 100%

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55
Q

Symptoms of SLE?

A

Discoid rash
Oral ulcers (usually painless)
Photosensitivity
ANA (>95%)
Malar rash (butterfly rash – occurs in 50%)
Immunological phenomenon (anti-smith, anti-dsDNA, anti-phospholipid, Ro, La)
NEurological (altered mental state, seizures, stroke, headache, depression)
Renal (persistent proteinuria/cell casts)
Arthralgia/non-erosive arthritis (occurs in 90%, involves 2+ peripheral joints)
Serositis (pleuritis/pericarditis)
Haematological (pancytopaenia)

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56
Q

Investigations for SLE?

A

3 best tests

1) Anti-dsDNA antibody titres
2) Complement: low C3+C4
3) raised ESR

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57
Q

Management of SLE?

A

Severe flares -> req urgent IV cyclophosphamide and high-dose prednisolone

Maintenance -> NSAIDs and hydroxychloroquine, low-dose steroids may be used if chronic

Cutaneous symptoms -> Topical steroids, suncream
Biologicals -> Target B cells with rituximab/belimumab
Lupus nephritis -> immunosuppression; steroids + cyclophosphamide/mycophenolate

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58
Q

Symptoms of antiphospholipid syndrome?

A

Antiphospholipid Abs cause CLOTS:
Coagulation defect (arterial and venous thrombosis)
Livedo reticularis
Obstetric (recurrent miscarriages)
Thrombocytopaenia (decreased platelets)

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59
Q

Investigations of antiphospholipid syndrome?

A

Measure anti-phospholipid antibodies
o Lupus anticoagulant Ab
o Anticardiolipin Ab
Causes a paradoxical rise in APTT (measures the speed of clotting)

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60
Q

Who would you screen for anti-phospholipid syndrome?

A

<50 with stroke + woman with 3+ pregnancy losses before 10 weeks

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61
Q

Management of anti-phospholipid syndrome?

A

Healthy lifestyle advice

One VTE/arterial thrombosis: Warfarin lifelong (aim INR 2-3)
Recurrent VTE (whilst on warfarin): Add in aspirin (aim INR 3-4)

Seek advice in pregnancy

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62
Q

Which features of SLE are less common in drug-induced lupus?

A

Renal and nervous system involvement

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63
Q

What are the classical features of raynauds disease vs phenomenon?

A

Raynaud’s disease typically presents in young women (e.g. 30 years old) with bilateral symptoms

Raynaud’s phenomenon (secondary causes):
connective tissue disorders
scleroderma (most common)
RA, SLE, leukaemia
type I cryoglobulinaemia, cold agglutinins
use of vibrating tools
drugs: oCP, ergot
cervical rib

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64
Q

How should you manage Raynaud’s?

A

Suspected secondary Raynaud’s -> referred to secondary care
first-line: CCB e.g. nifedipine
IV prostacyclin (epoprostenol) infusions: effects may last several weeks/months

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65
Q

What percentage of patients with psoriasis develop psoriatic arthropathy?

A

10-20%
All patients with psoriasis should be offered an annual assessment

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66
Q

How can you differentiate between psoriatic arthritis and RA?

A

Psoriatic: DIP joints, no RF, no skin nodules
RA: MCP joints, RF in 70%, Skin nodules

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67
Q

What are the features of psoriatic arthritis?

A

Stiffness, swelling and pain in joints, ligaments and tendons
Arthritis is relapsing and remitting
Can be symmetrical polyarthritis/asymmetrical oligoarthritis, usually in hands and feet
Usually preceded by the rash by a few years
Nail changes in 80% – pitting, yellowing, onycholysis
Dactylitis

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68
Q

XR changes in psoriatic arthritis?

A

Unusual combination of coexistence of erosive changes and new bone formation
Periostitis
‘pencil-in-cup’ appearance

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69
Q

How do you treat psoriatic arthritis?

A

Same as for RA
• NSAIDs – to relieve MSK symptoms
• Corticosteroid injections
• DMARDs at an early stage – e.g. Methotrexate
• TNF-alpha inhibitors

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70
Q

What is the most common causative organism in septic arthritis in young sexually active people? vs most common organism overall?

A

Neisseria gonorrhoeae
overall: Staphylococcus aureus

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71
Q

Treatment of septic arthritis?

A

IV flucloxacillin or clindamycin if penicillin allergic
For 6-12 weeks

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72
Q

How many people with reactive arthritis have HLA association?

A

HLA B27, 75%

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73
Q

What is the demographic for patients with reactive arthritis?

A

Young white adults

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74
Q

What causes reactive arthritis?

A

Occurs 1-4 weeks after either:
• GU infection from STI (e.g. chlamydia)
• GI infection (campylobacter, salmonella, shigella or Yersinia)
It may be chronic or relapsing

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75
Q

Which joints are affected in reactive arthritis and what are the other symptoms?

A
  • Asymmetrical
  • Lower extremities
  • Oligoarthritic (usually no more than 6 joints)
    “I knee’d to pee”
    I = conjunctivitis/anterior uveitis
    Knee’d = arthritis
    Pee = urethritis
    (organism is not present in arthrocentesis of joint)
May also see these skin changes:
keratoderma blenorrhagica(waxy yellow/brown papules on palms and soles)
Circinate balanitis (painless vesicles on the coronal margin of the prepuce)
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76
Q

Management of reactive arthritis?

A

No specific cure
Splint joints
NSAIDs or intra-articular steroid injections
Consider sulfasalazine or methotrexate if symptoms >6 months
Symptoms rarely last >12 months

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77
Q

Name a SE caused by hydroxychloroquine?

A

Bull’s-eye retinopathy

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78
Q

What is the pathophysiology of osteoporosis?

A

Skeletal disease characterised by:

  • Low bone mass
  • Microarchitectural deterioration of bone tissue
  • > increase in bone fragility and susceptibility to fracture
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79
Q

Name of treatments that can be used for osteoporosis?

A

Bisphosphonates, HRT, Denosumab, Teriparatide, Ca + vit D

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80
Q

MOA/SEs of bisphosphonates?

A

MOA - decrease osteoclast activity, acts on the cholesterol synthesis pathway - inhibits the enzyme ‘Farnesyl Pyrophosphate Synthase’
SEs - GI irritation (alendronate), sit up and take half an hour before food
Osteonecrosis of the jaw (rare)

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81
Q

HRT, MOA/SEs?

A

MOA - Decrease osteoclast activity
Benefits – reduce fractures, reduce risk of CC, prevent menopausal symptoms
Negatives – increase breast cancer risk, stroke, CVS disease, VTE

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82
Q

MOA/SEs of Denosumab?

A

MAb to RANK ligand, decreases osteoclast
Negative – rebound increase of bone turnover when stopped

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83
Q

MOA/CIs of Teriparatide?

A

Increases osteoblast activity (ANABOLIC), PTH analogue
Reduced risk of fractures by >50%
Increases bone density and improves trabecular structure
CI – Hx of bone cancer/mets

84
Q

What are the FRAX and DEXA score?

A

FRAX - assess risk over next 10 years, if >10% warrants a DEXA scan
DEXA – T>-1 = normal, T-1 to -2.5 = osteopaenia, T-2.5 = osteoporosis
o T score – compares your BMD to that of a young person with peak BMD
o Z score – compares your BMD to an average person of the same age, sex + ethnicity

85
Q

RFs for osteoporosis?

A

SHATTERED
Steroids
Hyperthyroidism/hyperparathyroidism
Alcohol + smoking
Thin BMI <22
Testosterone deficiency
Early menopause
Renal/liver failure
Erosive bone disorders (Inflammatory cytokines increase bone resorption: RA, spondyloarthritis, SLE, IBD)
Dietary Ca deficiency

86
Q

Management of patients with Osteopaenia?

A

DEXA T score between -1 and -2.5
- Diet (adequate Calcium + Vit D)
- Regular weight-bearing exercises - dancing/skipping/gym
- Reduce drinking + stop smoking
Rescan in 3 years
Management of patients at risk of corticosteroid-induced osteoporosis

87
Q

What is the classic triad in Behcets disease and what other symptoms may occur?

A

AI inflammation of vessels
Oral ulcers, genital ulcers, anterior uveitis
thrombophlebitis, DVT, arthritis, aseptic meningitis, erythema nodosum

88
Q

What demographic does Behcet’s disease most commonly occur in?

A

Young men from eastern Med (Turkey), FH

89
Q

What simple test can be used to assist diagnosis of Behcet’s disease?

A

Positive pathergy test is suggestive (the puncture site of a needle prick becomes inflamed with small pustules forming)

90
Q

Management of Behcet’s disease?

A

Topical steroids – for ulcers + uveitis
Systemic steroids
Steroid-sparing drugs – e.g. azathioprine

91
Q

What is McArdle’s Disease?

A

AR disease - inability to release glucose from glycogen
Characterised by reversible exercise intolerance, fatigue and acute crisis (severe fatigue and acute muscle cramps)
‘Second wind’ phenomenon

92
Q

Investigations for McArdle’s Disease?

A

Clinical
Elevated CK
Myoglobinuria in urine

93
Q

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

A

Macrophages

94
Q

What is Rugger Jersey spine and when is it seen?

A

Prominent endplate densities at multiple vertebral levels to produce an alternating sclerotic-lucent-sclerotic appearance
Hyperparathyroidism

95
Q

What causes Marfans Syndrome?

A

Autosomal dominant connective tissue disorder.
Caused by a defect in the FBN1 gene on chromosome 15 that codes for the protein fibrillin-1
(think FBI/MI5)

96
Q

What is included in the Major/Minor criteria for Marfans syndrome?

A

Major criteria (<2):
Lens dislocation
Aortic dissection or dilatation (risk rises in pregnancy)
Dural ectasia (dilation)
Skeletal features: arachnodactyly
(long spider fingers), armspan > height,
pectus deformity, scholiosis

Minor signs:
MV prolapse, joint hypermobility, high-arched palate

97
Q

Management of Marfans Syndrome?

A

B-blockers to slow dilation of the aortic root
Annual echos

98
Q

Pathophysiology of Ehler Danlos Syndrome?

A

Autosomal dominant CTD – affects T3 collagen

99
Q

Presentation of Ehler Danlos?

A

Presentation:
Joint hypermobility – recurrent dislocation
Elastic, fragile skin
Easy bruising
Aortic regurgitation, mitral valve prolapse, aortic dissection
SAH
Retinal streaks

100
Q

Fibromyalgia presentation?

A

9 pairs of tender points on the body – if tender in 11/18 of these points, Dx is more likely
More commonly on neck, shoulders, spine

101
Q

Management of fibromyalgia?

A

Explanation
Aerobic exercise
CBT
Medication: pregabalin, duloxetine, amitriptyline, replace vit D

102
Q

What is the most common pathogen if sickle-cell patents develop osteomyelitis?

A

Salmonella
In everyone else: Staph aureus

103
Q

How to treat osteomyelitis?

A

Flucloxacillin for 6 weeks
Clindamycin if allergic

104
Q

What do you need to test before commencing azathioprine?

A

Thiopurine methyltransferase (TPMT) deficiency is present in about 1 in 200 people and predisposes to azathioprine toxicity (pancytopaenia)

105
Q

What three categories of people should be offered bisphosphonates?

A

Patients >65 yrs or those who’ve previously had a fragility fracture
Patients <65 yrs with osteoporosis Dx from DEXA scan
Patients taking/anticipated to be taking steroids at 7.5mg daily for >3m

106
Q

Management of RA?

A

DMARDs - should start within 3/12 of persistent symptoms, can take 6-10wks to work (hydroxychloroquine, methotrexate - try alone first, sulfasalazine)
Steroids - can be used as a bridging agent waiting for DMARDs to work
Biological agents - TNF-a inhibitors (first-line when no response to 2xDMARDs), then B cell depletion (rituximab), IL1/6 inhibitors (tocilizumab), disruption of T cell function

107
Q

When should you stop biological treatment in RA?

A

When someone becomes unwell

108
Q

What should you check before starting immunosuppressants?

A

Hep B, HIV, TB

109
Q

How should you assess response to treatment in RA?

A

NICE recommends using a combination of CRP and disease activity (using a composite score such as DAS28) to assess response to treatment

110
Q

What symptoms do you get in De Quervain’s tenosynovitis?

A

Pain on the radial side of the wrist/tenderness over the radial styloid process

111
Q

How to manage De Quervain’s tenosynovitis?

A

analgesia
steroid injection
immobilisation with a thumb splint (spica) may be effective
surgical treatment is sometimes required

112
Q

What is discoid lupus erythematous?

A

Only the rash:
erythematous, raised rash, sometimes scaly,
may be photosensitive,
more common on face, neck, ears and scalp,
lesions heal with atrophy, scarring (may cause scarring alopecia), and pigmentation

113
Q

Treatment of discoid lupus erythematous?

A

topical steroid cream
Hydroxychloroquine (use this DMARD is SLE also)
avoid sun exposure

Very rarely progresses to SLE (5%)

114
Q

Which sex is more associated with a poor prognosis in RA?

A

Female

115
Q

Which medications may worsen osteoporosis (other than glucocorticoids)?

A

SSRIs
antiepileptics
proton pump inhibitors
glitazones
long term heparin therapy
aromatase inhibitors e.g. anastrozole

116
Q

What is Pseudoxanthoma elasticum?

A

An inherited condition (usually AR) characterised by an abnormality in elastic fibres

117
Q

What are the symptoms 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
gastrointestinal haemorrhage

MAGIC

118
Q

What is the most worrying condition that can occur in patients with Pseudoxanthoma elasticum?

A

Cardiac problems: IHD, mitral valve prolapse
Progressive narrowing of small/medium vessels from changes in the elastic properties of vessels

119
Q

What is the most common infective organism in an iliopsoas abscess?

A

Staph aureus

120
Q

What can cause a secondary iliopsoas abscess?

A

Crohn’s (commonest cause in this category)
Diverticulitis, colorectal cancer
UTI, GU cancers
Vertebral osteomyelitis
Femoral catheter, lithotripsy
Endocarditis
intravenous drug use

121
Q

Symptoms of an iliopsoas abscess?

A

Fever
Back/flank pain
Limp
Weight loss

122
Q

How to treat an iliopsoas abscess?

A

Antibiotics + percutaneous drainage -> successful in around 90% of cases
Surgery is indicated if:
1. Failure of percutaneous drainage
2. Presence of an another intra-abdominal pathology which requires surgery

123
Q

Symptoms of limited cutaneous systemic sclerosis?

A

Calcinosis (calcium deposits in the skin)
Raynaud’s (long Hx)
oEsophageal and gut dysmotility
Sclerodactyly (swollen tight digits)
Telangiectasia

124
Q

Which Abs is most associated with limited cutaneous systemic sclerosis?

A

Anti-centromere Abs 70-80%

125
Q

What is the difference between limited and diffuse cutaneous systemic sclerosis?

A

Limited - limited to the skin on the hands/face/feet
Diffuse - diffuse skin involvement and early organ fibrosis (lung, heart, GI, renal)

126
Q

Which Abs is most associated with diffuse cutaneous systemic sclerosis?

A

Antitopoisomerase-1 (anti-Scl70) Abs in 40% and anti-RNA polymerase in 20%

127
Q

Management of systemic sclerosis?

A

Currently no cure
Can be managed with steroids initially
Immunosuppression – IV cyclophosphamide (for organ involv or progressive skin disease)
Treat specific features of the disease e.g. oesophageal reflux
Monitor BP and renal function and echos
ACE-i/ARBs to decrease risk of renal crisis
Tx Raynauds -> hand warmers, battery-powered gloves, CCB/ACEi (vasodilators)

128
Q

What is the most common cause of death in diffuse cutaneous systemic sclerosis?

A

interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH)
Other complications include renal disease and HTN

129
Q

Which medication should not be prescribed alongside methotrexate?

A

Trimethoprim - can cause bone marrow suppression and severe pancytopaenia
High-dose aspirin increases the risk of methotrexate toxicity secondary to reduced excretion

130
Q

How often do you take methotrexate?

A

Weekly

131
Q

What should you take alongside methotrexate?

A

Folic acid, at least 24hrs after methotrexate

132
Q

How to treat methotrexate toxicity?

A

Folinic acid

133
Q

What is Paget’s disease?

A

Increased bone turnover from increased numbers of osteoclasts and resulting osteoblasts, resulting in:

  • Disorganised remodelling (bone = weaker, bulkier, less compact and more vascular)
  • Bone enlargement
  • Deformity
  • Weakness
134
Q

What causes Paget’s disease of the bone and who is more at risk?

A

both genetic and environmental factors (e.g. infections)
Over 40s, male, commoner in temperate climates (lacking extremes in temperatures)

135
Q

What are the symptoms of Paget’s disease?

A

Commonly asymptomatic (~70%)
Deep ‘boring’ bone pain
Deformity or bone enlargement ->Commonly in the pelvis, lumbar spine, skull, femur and tibia

136
Q

What would you see on an initial blood profile and urine in Paget’s?

A

raised ALP, Ca2+ and PO43- normal
Urinary hydroxyproline

137
Q

What would you see on XR in Paget’s disease?

A
  • Localised enlargement of bone
  • Osteolysis (bone degradation) and sclerosis (hardening)
  • Specific findings in Paget’s:
    o Classic ‘blade of grass’ lesion between healthy and diseased long bones
    o ‘Cotton wool’ pattern in the skull
138
Q

Complications of Paget’s Disease?

A
  • Pathological fractures
  • Osteoarthritis
  • Nerve compression from bone overgrowth e.g. CN8 (deafness/tinnitus), spinal nerves
  • Hypercalcaemia (Vomiting, polyuria, polydipsia)
  • Osteosarcoma
139
Q

Management of Paget’s Disease?

A

Analgesia (NSAIDs/paracetamol)
If not enough, bisphosphonates
May need surgery for bone deformity, osteoarthritis, fractures and nerve compression
Monitoring by ALP d/t risk of osteosarcoma

140
Q

What is Von Gierke Disease?

A

T1 glycogen storage disease - unable to break down glycogen into glucose
Usually presents in first few months of life with complications of hypoglycaemia

141
Q

What causes osteomalacia?

A

Vitamin D deficiency

142
Q

What does the initial blood work look like in osteomalacia?

A

Low Ca2+, low PO43-, raised ALP, raised PTH

143
Q

What might you see on XR in osteomalacia?

A
  • Loss of cortical (hard) bone
  • Partial fractures
  • Pseudofractures: ‘Looser’s zones’ – linear areas of low density surrounded by sclerotic borders
144
Q

How to treat osteomalacia?

A

Vitamin D
Monitor calcium levels (at risk of hypercalcaemia)

145
Q

What is carpel tunnel syndrome?

A

compression of median nerve in the carpal tunnel

146
Q

Symptoms of carpel tunnel syndrome?

A

Pain/parasthesia in thumb, index + middle finger
Weakness of thumb abduction, wasting of thenar eminence
Tinel + Phalen’s test positive

147
Q

Causes of carpel tunnel syndrome?

A

idiopathic
pregnancy
oedema e.g. heart failure
lunate fracture
rheumatoid arthritis
RF: obesity

148
Q

Management of carpel tunnel syndrome?

A 
corticosteroid injection 
wrist splints at night 
surgical decompression (flexor retinaculum division)
149
Q

What is osteopetrosis?

A

Opposite of osteoporosis
also known as marble bone disease, is a rare condition characterised by bone hardening due to the malfunction of osteoclasts which don’t absorb the bone correctly
results in dense, thick bones that are prone to fracture

150
Q

What are the symptoms and blood work for osteopetrosis?

A

bone pains and neuropathies are common,
calcium, phosphate and ALP are normal

151
Q

Which drugs can cause gout?

A

diuretics: thiazides, furosemide
ciclosporin
alcohol
cytotoxic agents
pyrazinamide
aspirin

CAT FLAP

152
Q

What can cause/precipitate a flare of pseudo gout?

A

dehydration, intercurrent illness, haemochromatosis (high iron), hyperparathyroidism, long-term steroids

153
Q

What would you use to diagnosis pseudogout and what would it show?

A

XR - linear opacification of articular cartilage (collection of chondrocalcinosis)
Joint aspiration - raised WCC, predominantly neutrophils, weakly positive birefringent crystals, joint fluid often looks purulent and SA must be excluded

154
Q

Management of pseudogout?

A

No specific treatment
Acute attacks: cool packs, rest, aspiration
Mx: intra-articular steroids, NSAIDs/colchicine

155
Q

Male to female ratio of systemic sclerosis?

A

4x more common in females

156
Q

What other non-specific antibodies are present in systemic sclerosis?

A

ANA positive in 90%
RF positive in 30%

157
Q

The presence of anti-Ro antibodies in SLE can lead to what defects in a foetus?

A

heart block

158
Q

What is Still’s disease?

A

The adult form of systemic juvenile idiopathic arthritis
A rare inflammatory disorder than can affect the whole body

159
Q

What are the symptoms of Still’s disease?

A

triad of joint pain, spiking fevers (typically in early eve daily), and a pink (salmon) bumpy rash is very characteristic of adult-onset Still’s disease

160
Q

Blood test result in Still’s disease?

A

Elevated serum ferritin

161
Q

Management of Still’s disease?

A

NSAIDs for first few weeks
Steroids if not managed effectively
Mtx, IL-1 or anti-TNF

162
Q

What is frozen shoulder?

A

Aetiology not fully understood
Known as adhesive capsulitis

163
Q

What is frozen shoulder associated with?

A

Diabetes (20%)

164
Q

Features of frozen shoulder?

A

External rotation is affected - both active + passive
Bilateral in up to 20%

165
Q

How long does frozen shoulder typically last?

A

Between 6 months - 2yrs

166
Q

Treatment of frozen shoulder?

A

NSAIDs, physio, PO or IA steroids

167
Q

Demographic of osteoarthritis?

A

More common in females, >50yrs, obesity, DDH

168
Q

Symptoms of osteoarthritis?

A

Pain worse at end of day
Most commonly in hip/knee
Crepitus, joint instability

Heberden’s nodes (DIPs), bouchard’s nodes (PIPs)
Most commonly affects the CMC/DIP joints
Squaring of the thumb

169
Q

XR findings for osteoarthritis?

A

LOSS
Loss of joint space
Osteophytes
Subchondral cysts
Subarticular sclerosis

170
Q

Management of osteoarthritis?

A

Exercise, WL, physio
Analgesia - paracetamol, NSAIDs, IA injections
Joint replacement surgery

171
Q

Causes of avascular necrosis of the hip?

A

long-term steroid use, chemotherapy, alcohol, trauma

172
Q

What might you see on XR, if at all, in avascular necrosis of the hip?

A

Crescent sign (shows osteonecrosis along the edge of the femur head)

173
Q

Management in avascular necrosis of the hip?

A

Joint replacement

174
Q

In ank spon, what will TNF-inhibitors not have an effect on?

A

Radiological progression of disease

175
Q

Which drug may cause a significant interaction with azathioprine and constitute a never event?

A

Allopurinol

176
Q

Is Azathioprine safe in pregnancy?

A

Yes

177
Q

What is the cause of De Quervain’s tendinosis and who gets it?

A

De Quervain’s tendinosis may be caused by overuse. It also is associated with pregnancy and rheumatoid disease. It is most common in middle-aged women.

178
Q

Poor prognostic factors for RA?

A

RF + anti-CCP +ve
Poor functional BL
XR - early erosions
HLA DR4
Extra-articular features
Slow onset

179
Q

What is a simplistic general rule to remember whether conditions are AR or AD?

A

dominant = structural, recessive = metabolic

180
Q

Examples of large cell vasculitis?

A

Giant cell arteritis, Takayasu’s arteritis

181
Q

Examples of medium cell vasculitis?

A

Polyarteritis nodosa, Kawasaki disease

182
Q

Examples of small cell vasculitis?

A
  • ANCA +ve vasculitis – p-ANCA associated microscopic polyangiitis, glomerulonephritis and Churg-Strauss syndrome, and c-ANCA associated granulomatosis with polyangiitis
  • ANCA -ve vasculitis – HSP, Goodpasture’s syndrome and cryoglobulinemia
183
Q

Who gets PAN?

A

Hep B
Middle aged men

184
Q

What are the features of PAN?

A

Fever, malaise, arthralgia
WL
HTN
Mononeuritis multiplex – sensorimotor polyneuropathy
Testicular pain
Livedo reticularis
Renal failure + haematuria

185
Q

Which autoAb is positive in PAN and how likely is this?

A

pANCA in 20%

186
Q

What is the most common target of pANCA?

A

Myeloperoxidase (MPO)

187
Q

What is the most common target of cANCA?

A

Serine proteinase 3 (PR3)

188
Q

Which autoAb is specific for mixed connective tissue disease and what are the symptoms?

A

anti-RNP
R Raynauds
N swollen hands with No synovitis
P Pain in muscle and joints

189
Q

Which drug is associated with atypical stress fractures of the proximal femoral shaft?

A

Bisphosphonates (essential because a reduction in osteoclast activity means bones last longer and are therefore more prone to fractures)

190
Q

Which nerve passes through the cubital tunnel?

A

Ulnar nerve

191
Q

Poor prognostic factor in polymyositis?

A

Anti-Jo Abs, because this is nearly always associated with ILD which gives a poorer prognosis

192
Q

What happens to the hair follicles in discoid lupus?

A

Characterised by follicular keratin plugs (keratin forms over the top of the follicle)

193
Q

Which cytokines is the most important in the pathophysiology of rheumatoid arthritis?

A

Tumour necrosis factor (TNF)

194
Q

What is the mechanism of action of azathioprine?

A

azathio’purine’ = purine synthesis inhibition

195
Q

What is familial mediterranean fever?

A

Familial Mediterranean Fever (FMF, also known as recurrent polyserositis) is an autosomal recessive disorder causing autoinflammation which typically presents by the second decade

196
Q

What is the mode of inheritance of FMF?

A

AR

197
Q

How long do attacks of FMF usually last and what symptoms do you get?

A

attacks typically last 1-3 days
pyrexia
abdominal pain (due to peritonitis)
pleurisy
pericarditis
arthritis
erysipeloid rash on lower limbs

198
Q

How to treat FMF?

A

Colchicine

199
Q

What is meralgia paraesthetica?

A

paraesthesia or anaesthesia in the distribution of the lateral femoral cutaneous nerve (LFCN)

200
Q

What can cause meralgia paraesthetica?

A

Obesity
Pregnancy
Tense ascites
Trauma
Iatrogenic - e.g. surgery
Various sports

201
Q

How to manage meralgia paraesthetica?

A

Injection of the nerve with local anaesthetic

202
Q

What type of immunoglobulin is RF and what type of immunoglobulin in the body does it react to?

A

Rheumatoid factor (RF) is a circulating antibody (usually IgM) that reacts with the Fc portion of the patients own IgG

203
Q

What is the gender divide in psoriatic arthritis?

A

EQUAL, hallelujah

204
Q

What is radial tunnel syndrome?

A

Thought to be compression of the posterior interosseous branch of the radial nerve

205
Q

What are the symptoms of radial tunnel syndrome?

A

pain tends to be around 4-5 cm distal to the lateral epicondyle
symptoms may be worsened by extending the elbow and pronating the forearm

MRCP (14 decks)

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