MCQs in Rheumatology: Vasculitis

MCQs in Rheumatology:
Contributors: These MCQs were written by Dr Kar-Ping Kuet; and were reviewed
by Dr Marian Regan. The MCQs were edited by Dr A Abhishek who also facilitated
the review process.
Question 1
A 43 year old lady is referred urgently to the rheumatology outpatient clinic. She is
an ex-intravenous drug user. She has recently been diagnosed with hepatitis C and
is under the infectious disease team but is yet to receive treatment. Recently, she
developed pain of ankles and hands. She also has a palpable purpuric rash on her
shins. There is no objective synovitis. Urine dipstick is 1+ protein, 1+ blood. BP is
120/80 mmHg. FBC, U&E and LFTs are normal. CRP and ESR are elevated at 60
mg/l, and 45 mm/hr respectively. ANCA screen is negative (PR3 and MPO awaited),
ANA negative, and rheumatoid factor positive 11280. Blood culture – sterile at 48
Which of the following tests is most likely to help establish a diagnosis?
Anti-CCP antibody
Urine for M C & S
HIV test
Urine protein creatinine ratio
Question 2
A 64 year old lady presents with fever and painful lesions on her scalp. She also
complains of shortness of breath and chest x-ray shows patchy opacification
throughout. She has a history of asthma of over 20 years and previous surgery for
polyps. She recalls a similar presentation 3 years ago which was a presumed
pneumonia and responded promptly to prednisolone, and antibiotics. She has been
well in between otherwise. She also felt ‘viral’ prior to onset of symptoms.
Investigations show the following
Hb 10.9 g/dl
WCC 16.5 x103/ml
Plt 455 x105/ml
Neut 10.2 x103/ml
Eos 4.6 x103/ml
CRP 124 mg/l
UE and LFT are normal. ANCAs are negative.
What is the most likely diagnosis?
Atypical pneumonia
Granulomatous polyangitis with eosinophilia
Polymyalgia rheumatic with underlying pneumonia
Granulomatous polyangitis
Question 3
A 52 year old previously healthy non-smoker Caucasian man presents to accident
and emergency with shortness of breath, cough and haemoptysis of several weeks.
He complains of intermittent fatigue, and pains in his right ankle and knee. On
examination, his temperature is 36.80C, BP120/60 mm/Hg, pulse100 beats/min,
saturations 92% on room air and respiratory rate is 20/minute. He has synovitis of
his ankles, and left knee. There is a palpable purpuric rash on his lower limbs and
elbows, his left eye is blood shot, and he has one small oral ulcer.
Investigations are as follows
Hb 10.1 g/l (13.1-16.6), WCC 10.4 x10 (normal differential count), Plts 600, U&E
normal. CRP is 110 mg/l. Urine dipstick is normal. Chest X-ray shows a cavitating
mass in the right upper zone. Immunology is pending.
Which of the following is the most likely diagnosis?
Granulomatous polyangitis
Granulomatous polyangitis with eosinophilia
Lung malignancy with paraneoplastic vasculitis
Right upper lobe pneumonia due to S Aureus
Question 4
A 28 year old lady was diagnosed with Takayasu’s arteritis when she presented 3
years ago with a myocardial infarction and stroke. Her diagnosis was confirmed on
MR angiogram. Her inflammatory markers were normal on presentation. She is
known to have stenosis of both internal carotid arteries. Her disease is well
controlled on mycophenolate 1g BD and 5mg of prednisolone and she is
asymptomatic. Most recently her CRP was <6 mg/l and ESR was 8 mg/l. You wish
to reassess for disease activity.
Which of the following is the most appropriate investigation?
CT angiogram
Conventional angiography
High resolution ultrasonography
MR angiogram
Question 5
A 73 year old lady has presented to the medical assessment unit with a 3 month
history of fever, weight loss and feeling unwell. She denies any other symptoms.
These symptoms started after returning from a holiday in Greece, and she has no
other medical history. On examination she looks unwell. Temperature is 37.6°C, BP
120/79, HR 100 beats/min, RR 18/min. There is no rash, or synovitis of the joints.
Auscultation of chest is normal and heart sounds reveal no added sounds.
Abdominal examination is unremarkable.
Hb 8.9
WCC 9.8
Plt 677
MCV 79.9
Alb 28
LFTs normal
Ur 15.6 mmol/l
Ct 256 mmol/l
K 4.5 mmol/l
Na 135 mmol/l
Urine dipstick protein3+ blood 3+
pANCA is positive with elevated MPO 186
Chest x-ray is normal.
Blood cultures x3 no growth after prolonged cultures
Transthoracic echocardiogram no vegetation
CT of thorax abdomen and pelvis is normal
What is the most likely diagnosis
1. Granulomatous polyangitis
2. Granulomatous polyangitis with eosinophilia
3. Goodpasture syndrome
4. Microscopic polyangiitis
5. Polyarteritis nodosa
Question 6
A 56 year old lady presents with fever, night sweats, and weight loss for 12months.
She has raised inflammatory markers. PET CT scan has shown increased uptake in
the ascending, arch and descending aorta. She is diagnosed with a large vessel
vasculitis. She has been commenced on oral prednisolone and has responded well.
You now wish to initiate a steroid sparing agent as adjunctive therapy.
Which of the following agents do you consider to be the most appropriate?
Mycophenolate mofetil
Question 7
An 87 year old female with type 2 diabetes and hypertension presents to the
ophthalmology department with sudden loss of vision in her left eye. She also
reports jaw pain and malaise. Fundoscopy reveals pale oedematous disc. ESR is 66
mg/l and CRP is 129 mg/l. You are concerned she has giant cell arteritis and
arrange a temporal artery biopsy for her. She is concerned about commencing
prednisolone because she is diabetic.
Which of the following is the most appropriate initial treatment for her?
1. Methyl prednisolone 0.5 gm i.v. O.D. for 3 days
2. Prednisolone 40 mg/day tapered slowly
3. Prednisolone 60mg/day tapered slowly
4. Prednisolone 60mg/day tapered slowly, and methotrexate 7.5/week
increased slowly
5. Prednisolone 40mg/day tapered slowly, and methotrexate 7.5/week
increased slowly
Question 8
A 61 year old man with long standing RA is admitted to hospital with anaemia,
weight loss, weakness and rash. He is unable to give a clear history about the
duration of his symptoms. He suffers with hypertension, and his medications include
leflunomide, amlodipine, and tramadol. On examination he has synovitis of the MCP
and PIP joints with swan neck deformity of the right index finger. The left small toe is
ischaemic with palpable pedal pulses. He also has a left foot drop and a punched
out ulcer on the medial malleolus of this right leg. Systemic examination is otherwise
Hb 9.9 gm/dl (normocytic, normochromic)
WCC 10.3 x 103/ml
Platelets 543 x 103/ml
CRP 156 mg/l
ESR 100 mg/l
UE normal
LFTs normal
Rheumatoid factor 256
Anti-CCP>600 IU/ml
ANCA screen negative
Urine protein creatinine ratio 4.5mmol/gm
What is the most likely diagnosis?
Granulomatous polyangitis
Granulomatous polyangitis with eosinophilia
Peripheral vascular disease
Rheumatoid vasculitis
2. Cryoglobulins
This lady has hepatitis C which is not currently being treated. The most likely
diagnosis is cryoglobulinemia secondary to her underlying hepatitis. The treatment
for this would be to treat the underlying cause.
2. Granulomatous polyangitis with eosinophilia
This lady has Granulomatous polyangitis with eosinophilia. She has a history of
asthma, nasal polyps, complains of painful nodular scalp lesions and has lung
infiltration on x-ray. ANCAs are not always positive, but if positive hepatitis B status
should be checked.
1. Granulomatous polyangitis
Granulomatous polyangitis is a multi-system necrotizing granulomatous disease.
Clinical manifestations of WG can include ENT, lung, joints, skin and scleritis and
can affect any organ. It is always important to consider mimics of vasculitis including
malignancy and infection as these can present in similar ways.
PET-CT is useful in demonstrating disease activity especially in those individuals
with normal inflammatory markers where it is difficult to ascertain whether symptoms
are due to disease activity or damage.
4. Microscopic polyangiitis
The most likely diagnosis is microscopic polyangiitis since she has renal involvement
with positive pANCA and elevated MPO titres. The other diagnoses would not really
fit in with this presentation.
4. Methotrexate
This lady has a large vessel vasculitis. This condition usually requires long term
glucocorticoid use. There is a modest role for methotrexate as an adjunctive therapy
to steroids. See Eular recommendations for the management of large vessel
vasculitis. Ann Rheum Dis 2009; 68 (3) 310-23.
1. Methyl prednisolone 0.5 gm i.v. O.D. for 3 days
This history is very suggestive of giant cell arteritis (GCA). Even if the temporal
artery biopsy is negative this may well be due to skip lesions and the patient should
still be treated for GCA. Since this lady has already developed visual symptoms you
would give i.v methylprednisolone. There is a role for starting methotrexate with
corticosteroids in patients with relapsing –remitting GCA, but not at clinical
presentation. See BSR guidelines
5. Rheumatoid vasculitis
This gentleman has rheumatoid vasculitis. He presents with multi-organ - joints,
skin, renal and neurological - involvement. Management would consist of pulsed
methylprednisolone and i.v. cyclophosphamide. Rheumatoid vasculitis remains a
rare but potentially life threatening condition.