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MRCP part 1 > Rheumatology > Flashcards

Rheumatology Flashcards

1
Q

what is systemic sclerosis

A

Systemic sclerosis is a condition of unknown aetiology characterised by hardened, sclerotic skin and other connective tissues. It is four times more common in females.

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2
Q

Limited cutaneous systemic sclerosis

A

Raynaud’s may be first sign
scleroderma affects face and distal limbs predominately
associated with anti-centromere antibodies
a subtype of limited systemic sclerosis is CREST syndrome: Calcinosis, Raynaud’s phenomenon, oEsophageal dysmotility, Sclerodactyly, Telangiectasia

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3
Q

What is CREST

A 
Calcinosis
Raynaud's phenomenon
oEsophageal dysmotility 
Sclerodactyly
Telangiectasia
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4
Q

Diffuse cutaneous systemic sclerosis

A

scleroderma affects trunk and proximal limbs predominately
associated with scl-70 antibodies
the most common cause of death is now respiratory involvement, which is seen in around 80%: interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH)
other complications include renal disease and hypertension
poor prognosis

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5
Q

Scleroderma (without internal organ involvement)

A

tightening and fibrosis of skin

may be manifest as plaques (morphoea) or linear

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6
Q

Systemic sclerosis antibodies

A

ANA positive in 90%
RF positive in 30%
anti-scl-70 antibodies associated with diffuse cutaneous systemic sclerosis
anti-centromere antibodies associated with limited cutaneous systemic sclerosis

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7
Q

What is pseudogout?

A

Pseudogout is a form of microcrystal synovitis caused by the deposition of calcium pyrophosphate dihydrate crystals in the synovium. For this reason, it is now more correctly termed acute calcium pyrophosphate crystal deposition disease.

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8
Q

Risk factors for pseudogout

A 
Pseudogout is strongly associated with increasing age. Patients who develop pseudogout at a younger age (e.g. < 60 years) usually have some underlying risk factor, such as:
haemochromatosis
hyperparathyroidism
low magnesium, low phosphate
acromegaly, Wilson's disease
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9
Q

Features of pseudogout

A

knee, wrist and shoulders most commonly affected
joint aspiration: weakly-positively birefringent rhomboid-shaped crystals
x-ray: chondrocalcinosis
in the knee this can be seen as linear calcifications of the meniscus and articular cartilage

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10
Q

Management of pseudogout

A

aspiration of joint fluid, to exclude septic arthritis

NSAIDs or intra-articular, intra-muscular or oral steroids as for gout

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11
Q

Features of drug induced lupus

A

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

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12
Q

Most common causes of drug induced lupus

A

procainamide

hydralazine

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13
Q

Least common causes of drug induced lupus

A

isoniazid
minocycline
phenytoin

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14
Q

What is polyarteritis nodosa

A

Polyarteritis nodosa (PAN) is a vasculitis affecting medium-sized arteries with necrotizing inflammation leading to aneurysm formation. PAN is more common in middle-aged men and is associated with hepatitis B infection.

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15
Q

Features of polyarteritis nodosa

A

fever, malaise, arthralgia
weight loss
hypertension
mononeuritis multiplex, sensorimotor polyneuropathy
testicular pain
livedo reticularis
haematuria, renal failure
perinuclear-antineutrophil cytoplasmic antibodies (ANCA) are found in around 20% of patients with ‘classic’ PAN
hepatitis B serology positive in 30% of patients

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16
Q

SLE antibodies

A

99% are ANA positive
this high sensitivity makes it a useful rule out test, but it has low specificity
20% are rheumatoid factor positive
anti-dsDNA: highly specific (> 99%), but less sensitive (70%)
anti-Smith: highly specific (> 99%), sensitivity (30%)
also: anti-U1 RNP, SS-A (anti-Ro) and SS-B (anti-La)

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17
Q

SLE monitoring investigations

A

Inflammatory markers
ESR is generally used
during active disease the CRP may be normal - a raised CRP may indicate underlying infection
complement levels (C3, C4) are low during active disease (formation of complexes leads to consumption of complement)
anti-dsDNA titres can be used for disease monitoring (but note not present in all patients)

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18
Q

Mechanism of action of methotrexate

A

Methotrexate is an antimetabolite that inhibits dihydrofolate reductase, an enzyme essential for the synthesis of purines and pyrimidines. It is considered an ‘important’ drug as whilst it can be very effective in controlling disease the side-effects may be potentially life-threatening - careful prescribing and close monitoring is essential.

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19
Q

Indications for Methotrexate

A

nflammatory arthritis, especially rheumatoid arthritis
psoriasis
some chemotherapy acute lymphoblastic leukaemia

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20
Q

Adverse effects of methotrexate

A 
mucositis
myelosuppression
pneumonitis
pulmonary fibrosis
liver fibrosis
hepatotoxicity
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21
Q

Pregnancy and methotrexate

A

women should avoid pregnancy for at least 6 months after treatment has stopped
the BNF also advises that men using methotrexate need to use effective contraception for at least 6 months after treatment

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22
Q

Prescribing methotrexate

A

methotrexate is a drug with a high potential for patient harm. It is therefore important that you are familiar with guidelines relating to its use
methotrexate is taken weekly, rather than daily
FBC, U&E and LFTs need to be regularly monitored. The Committee on Safety of Medicines recommend ‘FBC and renal and LFTs before starting treatment and repeated weekly until therapy stabilised, thereafter patients should be monitored every 2-3 months’
folic acid 5mg once weekly should be co-prescribed, taken more than 24 hours after methotrexate dose
the starting dose of methotrexate is 7.5 mg weekly (source: BNF)
only one strength of methotrexate tablet should be prescribed (usually 2.5 mg)

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23
Q

Methotrexate interactions

A

avoid prescribing trimethoprim or co-trimoxazole concurrently - increases risk of marrow aplasia
high-dose aspirin increases the risk of methotrexate toxicity secondary to reduced excretion

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24
Q

What is Behcet’s syndrome

A

Behcet’s syndrome is a complex multisystem disorder associated with presumed autoimmune-mediated inflammation of the arteries and veins. The precise aetiology has yet to be elucidated however. The classic triad of symptoms are oral ulcers, genital ulcers and anterior uveitis

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25
Q

Epidemiology of Behcet’s syndrome

A

more common in the eastern Mediterranean (e.g. Turkey)
more common in men (complicated gender distribution which varies according to country. Overall, Behcet’s is considered to be more common and more severe in men)
tends to affect young adults (e.g. 20 - 40 years old)
associated with HLA B51- more specifically HLA B51, a split antigen of HLA B5
around 30% of patients have a positive family history

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26
Q

Features of Behcet’s syndrome

A

classically: 1) oral ulcers 2) genital ulcers 3) anterior uveitis
thrombophlebitis and deep vein thrombosis
arthritis
neurological involvement (e.g. aseptic meningitis)
GI: abdo pain, diarrhoea, colitis
erythema nodosum

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27
Q

Diagnosis of Behcet’s syndrome

A

no definitive test
diagnosis based on clinical findings
positive pathergy test is suggestive (puncture site following needle prick becomes inflamed with small pustule forming)

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28
Q

Causes of Osteomalacia

A 
vitamin D deficiency
malabsorption
lack of sunlight
diet
chronic kidney disease
drug induced e.g. anticonvulsants
inherited: hypophosphatemic rickets (previously called vitamin D-resistant rickets)
liver disease: e.g. cirrhosis
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29
Q

Features of Osteomalacia

A

bone pain
bone/muscle tenderness
fractures: especially femoral neck
proximal myopathy: may lead to a waddling gait

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30
Q

Investigations of osteomalacia

A

bloods
low vitamin D levels
low calcium, phosphate (in around 30%)
raised alkaline phosphatase (in 95-100% of patients)
x-ray
translucent bands (Looser’s zones or pseudofractures

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31
Q

Treatment of osteomalacia

A

vitamin D supplmentation
a loading dose is often needed initially
calcium supplementation if dietary calcium is inadequate

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32
Q

Bone profile

osteoporosis

A

Normal Ca
Normal phosphate
Normal ALP
Normal PTH

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33
Q

Bone Profile

Osteomalacia

A

Decreased Ca
Decreased phosphate
Increased ALP
Increased PTH

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34
Q

Bone Profile

Primary hyperparathyroidism (→ osteitis fibrosa cystica)

A

IncreasedCa
Decreased phosphate
Increased ALP
Increased PTH

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35
Q

Bone Profile

Chronic kidney disease (→ secondary hyperparathyroidism)

A

Decreased Ca
Increased phosphate
Increased ALP
Increased PTH

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36
Q

Bone profile

Paget’s disease

A

Normal Ca
Normal phosphate
Increased ALP
Normal PTH

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37
Q

Bone Profile

Osteopetrosis

A

Normal Ca
Normal phosphate
Normal ALP
Normal PTH

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38
Q

What is Sjogren’s syndrome

A

autoimmune disorder affecting exocrine glands resulting in dry mucosal surfaces. It may be primary (PSS) or secondary to rheumatoid arthritis or other connective tissue disorders, where it usually develops around 10 years after the initial onset. Sjogren’s syndrome is much more common in females (ratio 9:1). There is a marked increased risk of lymphoid malignancy (40-60 fold).

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39
Q

Features of Sjogren’s syndrome

A 
dry eyes: keratoconjunctivitis sicca
dry mouth
vaginal dryness
arthralgia
Raynaud's, myalgia
sensory polyneuropathy
recurrent episodes of parotitis
renal tubular acidosis (usually subclinical)
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40
Q

investigations for Sjogren’s syndrome

A

rheumatoid factor (RF) positive in nearly 50% of patients
ANA positive in 70%
anti-Ro (SSA) antibodies in 70% of patients with PSS
anti-La (SSB) antibodies in 30% of patients with PSS
Schirmer’s test: filter paper near conjunctival sac to measure tear formation
histology: focal lymphocytic infiltration
also: hypergammaglobulinaemia, low C4

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41
Q

Management of Sjogren’s syndrome

A

artificial saliva and tears

pilocarpine may stimulate saliva production

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42
Q

What is polymyositis

A

inflammatory disorder causing symmetrical, proximal muscle weakness
thought to be a T-cell mediated cytotoxic process directed against muscle fibres
may be idiopathic or associated with connective tissue disorders
associated with malignancy
dermatomyositis is a variant of the disease where skin manifestations are prominent, for example a purple (heliotrope) rash on the cheeks and eyelids
typically affects middle-aged, female:male 3:1

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43
Q

Features of polymyositis

A 
proximal muscle weakness +/- tenderness
Raynaud's
respiratory muscle weakness
interstitial lung disease: e.g. fibrosing alveolitis or organising pneumonia
dysphagia, dysphonia
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44
Q

Investigations for polymyositis

A

elevated creatine kinase
other muscle enzymes (lactate dehydrogenase (LDH), aldolase, AST and ALT) are also elevated in 85-95% of patients
EMG
muscle biopsy
anti-synthetase antibodies
anti-Jo-1 antibodies are seen in pattern of disease associated with lung involvement, Raynaud’s and fever

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45
Q

Investigations of dermatomysositis

A

elevated creatine kinase
EMG
muscle biopsy
ANA positive in 60%
anti-Mi-2 antibodies are highly specific for dermatomyositis, but are only seen in around 25% of patients
anti-Jo-1 antibodies are not commonly seen in dermatomyositis - they are more common in polymyositis where they are seen in a pattern of disease associated with lung involvement, Raynaud’s and fever

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46
Q

Management of dermatomyositis

A

prednisolone

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47
Q

Methotrexate side effects

A

Myelosuppression
Liver cirrhosis
Pneumonitis

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48
Q

Sulfasalazine side effects

A

Rashes
Oligospermia
Heinz body anaemia
Interstitial lung disease

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49
Q

Leflunomide side effects

A

Liver impairment
Interstitial lung disease
Hypertension

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50
Q

Hydroxychloroquine side effects

A

Retinopathy

Corneal deposits

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51
Q

Prednisolone side effects

A 
Cushingoid features
Osteoporosis
Impaired glucose tolerance
Hypertension
Cataracts
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52
Q

Gold side effects

A

Proteinuria

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53
Q

Penicillamine side effects

A

Proteinuria

Exacerbation of myasthenia gravis

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54
Q

Etanercept side effects

A

Demyelination

Reactivation of tuberculosis

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55
Q

Infliximab side effects

A

Reactivation of tuberculosis

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56
Q

Adalimumab side effects

A

Reactivation of TB

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57
Q

Rituximab side effects

A

Infusion reactions are common

58
Q

NSAIDs (e.g. naproxen, ibuprofen) side effects

A

Bronchospasm in asthmatics

Dyspepsia/ gastric ulceration

59
Q

Examples of ANCA associated vasculitis

A

granulomatosis with polyangiitis
eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)
microscopic polyangiitis

60
Q

Common features of ANCA associated vasculitis

A 
renal impairment
caused by immune complex glomerulonephritis → raised creatinine, haematuria and proteinuria
respiratory symptoms
dyspnoea
haemoptysiis
systemic symptoms
fatigue
weight loss
fever
vasculitic rash: present only in a minority of patients
ear, nose and throat symptoms
sinusitis
61
Q

First line investigations for ANCA associated vasculitis

A

urinalysis for haematuria and proteinuria
bloods:
urea and creatinine for renal impairment
full blood count: normocytic anaemia and thrombocytosis may be seen
CRP: raised
ANCA testing (see below)
chest x-ray: nodular, fibrotic or infiltrative lesions may be seen

62
Q

cANCA target

A

Serine proteinase 3 (PR3)

63
Q

cANCA in disease- levels

A

+ve in Granulomatosis with polyangiitis (90%)
Low in eosinophilic granulomatosis with polyangiitis
40% of microscopic polyangiitis
some relationship between cANCA levels and disease management.

64
Q

pANCA target

A

myeloperoxidase (MPO)

65
Q

pANCA in disease - levels

A

+ve in Granulomatosis with polyangiitis (25%)
+ve in eosinophilic granulomatosis with polyangiitis (50%)
75% of microscopic polyangiitis

66
Q

Diseases associated with pANCA

A

Ulcerative colitis (70%)
Primary sclerosing cholangitis (70%)
Anti-GBM disease (25%)
Crohn’s disease (20%)

67
Q

pANCA monitoring

A

Can’t be used for disease monitoring

68
Q

Haematogenous osteomyelitis

A

results from bacteraemia
is usually monomicrobial
most common form in children
vertebral osteomyelitis is the most common form of haematogenous osteomyelitis in adults
risk factors include: sickle cell anaemia, intravenous drug user, immunosuppression due to either medication or HIV, infective endocarditis

69
Q

non-haematogenous osteomyelitis

A

results from the contiguous spread of infection from adjacent soft tissues to the bone or from direct injury/trauma to bone
is often polymicrobial
most common form in adults
risk factors include: diabetic foot ulcers/pressure sores, diabetes mellitus, peripheral arterial disease

70
Q

Microbiology of osteomyelitis

A

Staph. aureus is the most common cause except in patients with sickle-cell anaemia where Salmonella species predominate

71
Q

Management of osteomyelitis

A

flucloxacillin for 6 weeks

Clindamycin if pen allergic

72
Q

ANA antibodies

A

SLE
diffuse cutaneous systemic sclerosis
Limited cutaneous systemic sclerosis
Sjogren’s syndrome

73
Q

Anti-Jo-1 antibodies

A

polymyositis

74
Q

Anti-cyclic citrullinated peptide antibody

A

Rheumatic Athritis

75
Q

What is Raynaud’s disease

A

Raynaud’s phenomenon is characterised by an exaggerated vasoconstrictive response of the digital arteries and cutaneous arteriole to the cold or emotional stress. It may be primary (Raynaud’s disease) or secondary (Raynaud’s phenomenon).

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

76
Q

Secondary causes of Raynaud’s disease

A 
connective tissue disorders
scleroderma (most common)
rheumatoid arthritis
systemic lupus erythematosus
leukaemia
type I cryoglobulinaemia, cold agglutinins
use of vibrating tools
drugs: oral contraceptive pill, ergot
cervical rib
77
Q

Factors suggesting underlying connective tissue disease in Raynaud’s

A 
onset after 40 years
unilateral symptoms
rashes
presence of autoantibodies
features which may suggest rheumatoid arthritis or SLE, for example arthritis or recurrent miscarriages
digital ulcers, calcinosis
very rarely: chilblains
78
Q

Management of Raynaud’s

A

all patients with suspected secondary Raynaud’s phenomenon should be referred to secondary care

first-line: calcium channel blockers e.g. nifedipine
IV prostacyclin (epoprostenol) infusions: effects may last several weeks/months
79
Q

What is discoid lupus erythematous?

A

benign disorder generally seen in younger females. It very rarely progresses to systemic lupus erythematosus (in less than 5% of cases). Discoid lupus erythematosus is characterised by follicular keratin plugs and is thought to be autoimmune in aetiology

80
Q

Features of discoid lupus erythematous

A

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

81
Q

Management of discoid lupus erythematous

A

topical steroid cream
oral antimalarials may be used second-line e.g. hydroxychloroquine
avoid sun exposure

82
Q

Osteopetrosis

A

also known as marble bone disease
rare disorder of defective osteoclast function resulting in failure of normal bone resorption
results in dense, thick bones that are prone to fracture
bone pains and neuropathies are common.
calcium, phosphate and ALP are normal
stem cell transplant and interferon-gamma have been used for treatment

83
Q

Eye symptoms of Marfan’s

A

upwards lens dislocation (superotemporal ectopia lentis), blue sclera, myopia

84
Q

Development Dysplasia of Hip

A

Often picked up on newborn examination
Barlow’s test, Ortolani’s test are positive
Unequal skin folds/leg length

85
Q

Transient synovitis (irritable hip)

A

Typical age group = 2-10 years
Acute hip pain associated with viral infection
Commonest cause of hip pain in children

86
Q

Perthe’s disease

Epidemiology

A

Perthes disease is a degenerative condition affecting the hip joints of children, typically between the ages of 4-8 years. It is due to avascular necrosis of the femoral head

Perthes disease is 5 times more common in boys. Around 10% of cases are bilateral

87
Q

Perthes disease

Features

A

hip pain: develops progressively over a few weeks
limp
stiffness and reduced range of hip movement
x-ray: early changes include widening of joint space, later changes include decreased femoral head size/flattening

88
Q

Slipped upper femoral epiphysis

A

Typical age group = 10-15 years
More common in obese children and boys
Displacement of the femoral head epiphysis postero-inferiorly
Bilateral slip in 20% of cases
May present acutely following trauma or more commonly with chronic, persistent symptoms

89
Q

Slipped upper femoral epiphysis

Features

A

knee or distal thigh pain is common

loss of internal rotation of the leg in flexion

90
Q

Juvenile idiopathic arthritis (JIA)

A

Preferred to the older term juvenile chronic arthritis, describes arthritis occurring in someone who is less than 16 years old that lasts for more than three months. Pauciarticular JIA refers to cases where 4 or less joints are affected. It accounts for around 60% of cases of JIA

91
Q

Juvenile idiopathic arthritis (JIA)

Features

A

joint pain and swelling: usually medium sized joints e.g. knees, ankles, elbows
limp
ANA may be positive in JIA - associated with anterior uveitis

92
Q

Septic Arthritis

A

Acute hip pain associated with systemic upset e.g. pyrexia. Inability/severe limitation of affected joint

93
Q

What is pagets disease of the bone

A

Paget’s disease is a disease of increased but uncontrolled bone turnover. It is thought to be primarily a disorder of osteoclasts, with excessive osteoclastic resorption followed by increased osteoblastic activity. Paget’s disease is common (UK prevalence 5%) but symptomatic in only 1 in 20 patients. The skull, spine/pelvis, and long bones of the lower extremities are most commonly affected.

94
Q

Pre-disposing factors of pagets disease

A

increasing age
male sex
northern latitude
family history

95
Q

Clinical features of Pagets disease

A 
older male with bone pain and an isolated raised ALP
bone pain (e.g. pelvis, lumbar spine, femur)
classical, untreated features: bowing of tibia, bossing of skull
raised alkaline phosphatase (ALP) - calcium* and phosphate are typically normal
other markers of bone turnover include: procollagen type I N-terminal propeptide (PINP), serum C-telopeptide (CTx), urinary N-telopeptide (NTx), and urinary hydroxyproline
skull x-ray: thickened vault, osteoporosis circumscripta
96
Q

Treatment of Pagets disease of the bone

(indicated with bone pain, skull/long bone deformity, fracture or peri-articular Pagets

A

bisphosphonate (either oral risedronate or IV zoledronate)

calcitonin is less commonly used now

97
Q

Complications of Pagets disease of the bone

A 
deafness (cranial nerve entrapment)
bone sarcoma (1% if affected for > 10 years)
fractures
skull thickening
high-output cardiac failure
98
Q

Polymyositis

A

inflammatory disorder causing symmetrical, proximal muscle weakness
thought to be a T-cell mediated cytotoxic process directed against muscle fibres
may be idiopathic or associated with connective tissue disorders
associated with malignancy
dermatomyositis is a variant of the disease where skin manifestations are prominent, for example a purple (heliotrope) rash on the cheeks and eyelids
typically affects middle-aged, female:male 3:1

99
Q

Features of polymyositis

A 
proximal muscle weakness +/- tenderness
Raynaud's
respiratory muscle weakness
interstitial lung disease: e.g. fibrosing alveolitis or organising pneumonia
dysphagia, dysphonia
100
Q

Investigations of polymyositis

A

elevated creatine kinase
other muscle enzymes (lactate dehydrogenase (LDH), aldolase, AST and ALT) are also elevated in 85-95% of patients
EMG
muscle biopsy
anti-synthetase antibodies
anti-Jo-1 antibodies are seen in pattern of disease associated with lung involvement, Raynaud’s and fever

101
Q

Pre-disposing factors for Pseudogout

A

haemochromatosis
hyperparathyroidism
low magnesium, low phosphate
acromegaly, Wilson’s disease

102
Q

Features of pseudogout

A

knee, wrist and shoulders most commonly affected
joint aspiration: weakly-positively birefringent rhomboid-shaped crystals
x-ray: chondrocalcinosis
in the knee this can be seen as linear calcifications of the meniscus and articular cartilage

103
Q

Management of pseudogout

A

aspiration of joint fluid, to exclude septic arthritis

NSAIDs or intra-articular, intra-muscular or oral steroids as for gout

104
Q

What is Schirmer’s test

A

filter paper near conjunctival sac to measure tear formation

105
Q

SLE antibodies

A 
ANA 
Anti-dsDNA
Anti-Smith
Rheumatoid factor
anti-U1 RNP, SS-A (anti-Ro) and SS-B (anti-La)
106
Q

Dermatomyositis antibodies

A

ANA

Anti- Mi-2

107
Q

Sjogren’s antibodies

A

Rheumatoid factor
ANA
Anti-Ro (SSB)
Anti- La

108
Q

Raloxifene

A

has been shown to prevent bone loss and to reduce the risk of vertebral fractures, but has not yet been shown to reduce the risk of non-vertebral fractures
has been shown to increase bone density in the spine and proximal femur
may worsen menopausal symptoms
increased risk of thromboembolic events
may decrease risk of breast cancer

109
Q

what is stronium ranelate

A

‘dual action bone agent’ - increases deposition of new bone by osteoblasts (promotes differentiation of pre-osteoblast to osteoblast) and reduces the resorption of bone by inhibiting osteoclasts
concerns regarding the safety profile of strontium have been raised recently. It should only be prescribed by a specialist in secondary care
due to these concerns the European Medicines Agency in 2014 said it should only be used by people for whom there are no other treatments for osteoporosis
increased risk of cardiovascular events: any history of cardiovascular disease or significant risk of cardiovascular disease is a contraindication
increased risk of thromboembolic events: a Drug Safety Update in 2012 recommended it is not used in patients with a history of venous thromboembolism
may cause serious skin reactions such as Stevens Johnson syndrome

110
Q

Mechanism of action for etanercept

A

acts as a decoy receptor for TNF-a

may reactivate TB

111
Q

Mechanism of action for infliximab

A

Binds to TNF-a and prevents it binding to TNF receptors

May reactivate TB

112
Q

Management of raynaurd’s

A 
all patients with suspected secondary Raynaud's phenomenon should be referred to secondary care
first-line: calcium channel blockers e.g. nifedipine
IV prostacyclin (epoprostenol) infusions: effects may last several weeks/months
113
Q

What is McArdle’s disease

A

autosomal recessive type V glycogen storage disease
caused by myophosphorylase deficiency
this causes decreased muscle glycogenolysis

114
Q

Features of McArdle’s

A

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

115
Q

What is azathioprine

A

Azathioprine is metabolised to the active compound mercaptopurine, a purine analogue that inhibits purine synthesis. A thiopurine methyltransferase (TPMT) test may be needed to look for individuals prone to azathioprine toxicity.

116
Q

Adverse effects of azathioprine

A

bone marrow depression
nausea/vomiting
pancreatitis
increased risk of non-melanoma skin cancer

A significant interaction may occur with allopurinol and hence lower doses of azathioprine should be used.

Azathioprine is generally considered safe to use in pregnancy.

117
Q

Chondromalacia patellae

A

Softening of the cartilage of the patella
Common in teenage girls
Characteristically anterior knee pain on walking up and down stairs and rising from prolonged sitting
Usually responds to physiotherapy

118
Q

Osgood-Schlatter disease

tibial apophysitis

A

Seen in sporty teenagers

Pain, tenderness and swelling over the tibial tubercle

119
Q

Osteochondritis dissecans

A

Pain after exercise

Intermittent swelling and locking

120
Q

Patellar subluxation

A

Medial knee pain due to lateral subluxation of the patella

Knee may give way

121
Q

Patellar tendonitis

A

More common in athletic teenage boys
Chronic anterior knee pain that worsens after running
Tender below the patella on examination

122
Q

mechanism of action: cholchicine

A

Inhibits microtubule polymerization by binding to tubulin, interfering with mitosis. Also inhibits neutrophil motility and activity

123
Q

genetic cause of marfans

A

a defect in the FBN1 gene on chromosome 15 that codes for the protein fibrillin-1

124
Q

hypoxanthine-guanine phosphoribosyl transferase (HGPRTase) deficiency

A

Lesch-Nyhan syndrome

125
Q

Lesch-Nyhan syndrome

A

x-linked recessive therefore only seen in boys

features: gout, renal failure, neurological deficits, learning difficulties, self-mutilation

126
Q

Giant cell tumours of the bone - radiological features

A

double/soap bubble’ x-ray appearance around epiphysis of femur
tumour of multinucleated giant cells within a fibrous stroma
peak incidence: 20-40 years
occurs most frequently in the epiphyses of long bones

127
Q

Langerhans cell histocytosis

A

child, bone pain in skull/proximal femur, cutaneous nodules, recurrent otitis media/mastoiditis

128
Q

osteoma

A

benign ‘overgrowth’ of bone, most typically occuring on the skull
associated with Gardner’s syndrome (a variant of familial adenomatous polyposis, FAP)

129
Q

osteochondroma

A

most common benign bone tumour
more in males, usually diagnosed in patients aged < 20 years
cartilage-capped bony projection on the external surface of a bone

130
Q

Osteosarcoma

A

most common primary malignant bone tumour
seen mainly in children and adolescents
occurs most frequently in the metaphyseal region of long bones prior to epiphyseal closure, with 40% occuring in the femur, 20% in the tibia, and 10% in the humerus
x-ray shows Codman triangle (from periosteal elevation) and ‘sunburst’ pattern
mutation of the Rb gene significantly increases risk of osteosarcoma (hence association with retinoblastoma)
other predisposing factors include Paget’s disease of the bone and radiotherapy

131
Q

Ewing’s sarcoma

A

small round blue cell tumour
seen mainly in children and adolescents
occurs most frequently in the pelvis and long bones. Tends to cause severe pain
associated with t(11;22) translocation which results in an EWS-FLI1 gene product
x-ray shows ‘onion skin’ appearance

132
Q

chondrosarcoma

A

malignant tumour of cartilage
most commonly affects the axial skeleton
more common in middle-age

133
Q

syndesmophytes

A

due to ossification of outer fibers of annulus fibrosus in ankylosing spondylitis

134
Q

Familial Mediterranean Fever Diagnosis

A

Tel-Hashomer diagnostic criteria
recurrent episodes of serositis (peritonitis, synovitis, pericarditis) in association with fever and prompt response to colchicine.

135
Q

Features of Familial Mediterranean Fever

A 
pyrexia
abdominal pain (due to peritonitis)
pleurisy
pericarditis
arthritis
erysipeloid rash on lower limbs
136
Q

Methotrexate interactions

A

Trimethoprim and co-trimoxazole - increase risk of bone marrow suppression
High dose aspirin - increases the risk of methotrexate toxicity

137
Q

Paget’s disease

A 
Raised ALP 
Normal serum calcium 
Increased uncontrolled bone turn over 
Lytic lesions and thickened skull volt 
Advanced - can cause hearing loss
138
Q

Markers of bone turnover in Pagets disease

A

procollagen type I N-terminal propeptide (PINP), serum C-telopeptide (CTx), urinary N-telopeptide (NTx), and urinary hydroxyproline

139
Q

Features of drug induced lupus

A

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

140
Q

SLE in pregnancy

A

risk of maternal autoantibodies crossing the placenta
leads to a condition termed neonatal lupus erythematosus
neonatal complications include congenital heart block
strongly associated with anti-Ro (SSA) antibodies- causes peadiatric heart block

141
Q

DMARD in pregnancy

A

Azathioprine

MRCP part 1 (15 decks)

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