MRCP rheumatology test
questions and answers 2024
ANCA
There are two main types of anti-neutrophil cytoplasmic antibodies
(ANCA):
1. cytoplasmic (cANCA) and 2. perinuclear (pANCA)
***Remember
cANCA - Wegener's granulomatosis
pANCA - Churg-Strauss syndrome + others*
cANCA
most common target serine proteinase 3 (PR3)
some correlation between cANCA levels and disease activity
Wegener's granulomatosis, positive in > 90%
microscopic polyangiitis, positive in 40%
pANCA
most common target is *myeloperoxidase (MPO)
cannot use level of pANCA to monitor disease activity
associated with immune crescentic glomerulonephritis (positive in c.
80% of patients)
microscopic polyangiitis, positive in 50-75%
Churg-Strauss syndrome, positive in 60%
primary sclerosing cholangitis, positive in 60-80%
Wegener's granulomatosis, positive in 25%
Other causes of positive ANCA (usually pANCA)
inflammatory bowel disease (UC > Crohn's)
connective tissue disorders: RA, SLE, Sjogren's
autoimmune hepatitis
Ankylosing Spondylitis (AS)
Ankylosing spondylitis - x-ray findings: subchondral erosions,
sclerosis and squaring of lumbar vertebrae
Key features of the pain include:
,- Early morning stiffness of more than 30 minutes Alternating
buttock pain
- Waking in the second half of the night
- Pain easing with non-steroidal anti-inflammatory drugs (NSAIDs)
- Pain which is worse with rest and eases with exercise
Clinical examination
reduced lateral flexion
reduced forward flexion - Schober's test -
loss of lumbar lordosis
accentuated thoracic kyphosis
**Plain x-ray of the sacroiliac joints is the most useful investigation
in establishing the diagnosis.
MRI:
More sensitive than x-ray
NSAIDs are the first-line treatment
the disease-modifying drugs which are used to treat rheumatoid
arthritis (such as sulphasalazine) are only really useful if there is
peripheral joint involvement
70 y female co Painful swollen left knee
X-ray
Some loss of joint space
Linear calcification of the articular cartilage
Pseudogout is a form of microcrystal synovitis caused by the
deposition of calcium pyrophosphate dihydrate in the synovium
Risk factors
hyperparathyroidism
hypothyroidism
haemochromatosis
acromegaly
,low magnesium, low phosphate
Wilson's disease
Features
knee, wrist and shoulders most commonly affected
joint aspiration: weakly-positively birefringent rhomboid shaped
crystals
x-ray: chondrocalcinosis
Management
aspiration of joint fluid, to exclude septic arthritis
NSAIDs or intra-articular, intra-muscular or oral steroids as for gout
hydroxychloroquine.
Which is the most important to monitor?
Visual activities
Hydroxychloroquine - may result in a severe and permanent
retinopathy
leflunomide.
Which is the most important to monitor?
Leflunomide may cause hypertension
Painful knee with swelling. An ultrasound: effusion and states the
'double contour' sign was observed. What is the most likely
diagnosis?
Gout
What is the most important investigation to monitor in marfan's
syndrome ?
Marfan's syndrome
autosomal dominant connective tissue disorder.
Features
tall stature with arm span to height ratio > 1.05
high-arched palate
arachnodactyly
, pectus excavatum
pes planus
scoliosis of > 20 degrees
Heart: dilation of the aortic sinuses (seen in 90%) which may lead to
aortic aneurysm, aortic dissection, aortic regurgitation, mitral valve
prolapse (75%),
lungs: repeated pneumothoraces
eyes: upwards lens dislocation (superotemporal ectopia lentis) seen
in 50% of patients, blue sclera, myopia
dural ectasia (ballooning of the dural sac at the lumbosacral level)
affects 60% of patients
, It may cause lower back pain associated with neurological
problems such as bladder and bowel dysfunction.
Diagnosis
Unfortunately DNA testing for fibrillin gene mutations, whilst
helpful, cannot exclude a diagnosis of Marfan because a number of
mutations exist (at least 130).
Hence diagnosis is made on the major and minor features
associated with the syndrome.
Prognosis & treatment :
The life expectancy of patients used to be around 40-50 years.
With the advent of **regular echocardiography monitoring and beta-
blocker/ACE-inhibitor therapy this has improved significantly over
recent years.
Treatment with β-blockers reduces the rate of aortic dilatation and
the risk of rupture
Aortic dissection and other cardiovascular problems remain the
leading cause of death however.
Pregnancy is associated with increased risk of aortic rupture
Which of the following findings is not typical in a patient with
antiphospholipid syndrome?
Prolonged APTT
Thrombocytosis
Recurrent venous thrombosis
Recurrent arterial thrombosis
Livedo reticularis
Antiphospholipid syndrome: arterial/venous thrombosis,
miscarriage, livedo reticularis
questions and answers 2024
ANCA
There are two main types of anti-neutrophil cytoplasmic antibodies
(ANCA):
1. cytoplasmic (cANCA) and 2. perinuclear (pANCA)
***Remember
cANCA - Wegener's granulomatosis
pANCA - Churg-Strauss syndrome + others*
cANCA
most common target serine proteinase 3 (PR3)
some correlation between cANCA levels and disease activity
Wegener's granulomatosis, positive in > 90%
microscopic polyangiitis, positive in 40%
pANCA
most common target is *myeloperoxidase (MPO)
cannot use level of pANCA to monitor disease activity
associated with immune crescentic glomerulonephritis (positive in c.
80% of patients)
microscopic polyangiitis, positive in 50-75%
Churg-Strauss syndrome, positive in 60%
primary sclerosing cholangitis, positive in 60-80%
Wegener's granulomatosis, positive in 25%
Other causes of positive ANCA (usually pANCA)
inflammatory bowel disease (UC > Crohn's)
connective tissue disorders: RA, SLE, Sjogren's
autoimmune hepatitis
Ankylosing Spondylitis (AS)
Ankylosing spondylitis - x-ray findings: subchondral erosions,
sclerosis and squaring of lumbar vertebrae
Key features of the pain include:
,- Early morning stiffness of more than 30 minutes Alternating
buttock pain
- Waking in the second half of the night
- Pain easing with non-steroidal anti-inflammatory drugs (NSAIDs)
- Pain which is worse with rest and eases with exercise
Clinical examination
reduced lateral flexion
reduced forward flexion - Schober's test -
loss of lumbar lordosis
accentuated thoracic kyphosis
**Plain x-ray of the sacroiliac joints is the most useful investigation
in establishing the diagnosis.
MRI:
More sensitive than x-ray
NSAIDs are the first-line treatment
the disease-modifying drugs which are used to treat rheumatoid
arthritis (such as sulphasalazine) are only really useful if there is
peripheral joint involvement
70 y female co Painful swollen left knee
X-ray
Some loss of joint space
Linear calcification of the articular cartilage
Pseudogout is a form of microcrystal synovitis caused by the
deposition of calcium pyrophosphate dihydrate in the synovium
Risk factors
hyperparathyroidism
hypothyroidism
haemochromatosis
acromegaly
,low magnesium, low phosphate
Wilson's disease
Features
knee, wrist and shoulders most commonly affected
joint aspiration: weakly-positively birefringent rhomboid shaped
crystals
x-ray: chondrocalcinosis
Management
aspiration of joint fluid, to exclude septic arthritis
NSAIDs or intra-articular, intra-muscular or oral steroids as for gout
hydroxychloroquine.
Which is the most important to monitor?
Visual activities
Hydroxychloroquine - may result in a severe and permanent
retinopathy
leflunomide.
Which is the most important to monitor?
Leflunomide may cause hypertension
Painful knee with swelling. An ultrasound: effusion and states the
'double contour' sign was observed. What is the most likely
diagnosis?
Gout
What is the most important investigation to monitor in marfan's
syndrome ?
Marfan's syndrome
autosomal dominant connective tissue disorder.
Features
tall stature with arm span to height ratio > 1.05
high-arched palate
arachnodactyly
, pectus excavatum
pes planus
scoliosis of > 20 degrees
Heart: dilation of the aortic sinuses (seen in 90%) which may lead to
aortic aneurysm, aortic dissection, aortic regurgitation, mitral valve
prolapse (75%),
lungs: repeated pneumothoraces
eyes: upwards lens dislocation (superotemporal ectopia lentis) seen
in 50% of patients, blue sclera, myopia
dural ectasia (ballooning of the dural sac at the lumbosacral level)
affects 60% of patients
, It may cause lower back pain associated with neurological
problems such as bladder and bowel dysfunction.
Diagnosis
Unfortunately DNA testing for fibrillin gene mutations, whilst
helpful, cannot exclude a diagnosis of Marfan because a number of
mutations exist (at least 130).
Hence diagnosis is made on the major and minor features
associated with the syndrome.
Prognosis & treatment :
The life expectancy of patients used to be around 40-50 years.
With the advent of **regular echocardiography monitoring and beta-
blocker/ACE-inhibitor therapy this has improved significantly over
recent years.
Treatment with β-blockers reduces the rate of aortic dilatation and
the risk of rupture
Aortic dissection and other cardiovascular problems remain the
leading cause of death however.
Pregnancy is associated with increased risk of aortic rupture
Which of the following findings is not typical in a patient with
antiphospholipid syndrome?
Prolonged APTT
Thrombocytosis
Recurrent venous thrombosis
Recurrent arterial thrombosis
Livedo reticularis
Antiphospholipid syndrome: arterial/venous thrombosis,
miscarriage, livedo reticularis
