MCQs in Rheumatology:
Miscellaneous conditions
Contributors: These MCQs were written by Dr Fran Rees, Dr Kar-Ping Kuet and Dr
Michelle Hui; and were reviewed by Dr Neal Hopkinson, Dr Marian Regan, Prof Andy
Hassell, and Prof Nigel Arden. The MCQs were edited by Dr A Abhishek who also
facilitated the review process.
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Question 1
A 19-year old man presents with acute swelling of both ankles. On examination,
there is positive stress test (pain at extreme movements) at the tibiotalar and
subtalar joints with pitting oedema of the shins associated with painful raised red
patches.
What is the most appropriate investigation?
1.
2.
3.
4.
5.
Aspiration of ankles
Chest x-ray
Skin biopsy
Urethral swab
X-ray of ankles
Question 2
A four year old child is referred to accident and emergency unwell with fever and not
feeding for 5 days. On examination he has fever of 40°C, BP of 140/90, HR 100
bpm, RR 20/min. He has palpable cervical lymph nodes, a red tongue and a truncal
rash with desquamation of the hands and feet.
What is the most likely diagnosis?
1.
2.
3.
4.
5.
Infectious mononucleosis
Kawasaki disease
Measles
Parvovirus infection
Systemic lupus erythematosus
Question 3
A 60-year-old man is admitted with fever, arthralgia and rash. He has been generally
unwell for past 2 weeks. On examination, his temperature is 38.2 oC. He has tender,
erythematous plaques over his hands, arms and legs. There is no synovitis.
Investigations show a neutrophilia (15x109/l), a raised C-reactive protein (89mg/L),
and a punch biopsy of the lesion shows neutrophilic infiltration of the dermis.
What is the initial treatment of choice?
1. Colchicine
2. Dapsone
3. Ibuprofen
4. Indomethacin
5. Prednisolone
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Question 4
A 19-year-old Greek man presents with an 8 week history of intermittent attacks of
left sided chest pain and fever. During the attacks he is unable to deep breathe or lie
flat. His temperature has been recorded up to 39 degrees. In addition, he has
developed generalised arthralgia and has noticed intermittent swelling of his right
knee.
On examination, temperature is 37.8 degrees. He has a left-sided pleural rub and a
swollen right knee, which is aspirated.
Initial investigations
Joint aspirate No organisms, no crystals.
C-reactive protein 110mg/L
Hb 13.5g/dL
WCC 19.8x109/l
Plts 175 x109/l
Blood cultures negative
Which gene is most likely to be mutated?
1. CIASI
2. CINCA
3. MEFV
4. MVK
5. TNFRFSFIA
Question 5
A 55 year old man with long standing Rheumatoid arthritis, and bronchiectasis, is
seen in clinic with weakness, fatigue, weight loss and diarrhoea. He is currently
taking Methotrexate 15mg weekly. He has recently been diagnosed with heart
failure, and has developed peripheral neuropathy over the last few years. He has
hepato-splenomegaly, and the urine dipstick in clinic shows 2+ proteinuria.
Recent blood tests show a normal FBC, U&E. The CRP and ESR are 12 mg/l and 65
mm/hr. The serum albumin is 27g/l (35-55), and total protein is 90g/l (60-84).
Which of the following investigations would you choose next?
1.
2.
3.
4.
5.
Abdominal subcutaneous fat biopsy
Liver biopsy
Rectal biopsy
Radiolabelled- SAP scan
Stool sample
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Question 6
An 18 year old Turkish man presents with an 8 week history of intermittent fevers
associated with severe abdominal pain. He has generalised arthralgia and has
noticed a rash on his legs. On examination, the temperature is 37.8 oC. His abdomen
is soft, but there is generalised tenderness. He has a swollen right knee, which is
aspirated. He has a confluent erythematous macular rash on his legs. Joint aspirate
No organisms, no crystals.
What is the most useful long-term treatment?
1.
2.
3.
4.
5.
Colchicine
Methotrexate
Naproxen
Prednisolone
Sulfasalazine
Question 7
A 72 year old man with long standing Rheumatoid arthritis is seen in clinic with
weakness, fatigue, weight loss and diarrhoea. He is currently taking Methotrexate
15mg weekly. On examination he has hepato-splenomegaly and peripheral oedema.
Initial investigations
Hb 10.5g/dL
WCC 4.3 x109/l
Plts 135 x109/l
MCV 100fL
U&E and LFTs normal.
Urine dipstick Protein 3+ , Blood Albumin 27g/L (35-55)
Total protein 90g/L (60-84).
Serum electrophoresis no free light chains.
What is the most likely diagnosis?
1.
2.
3.
4.
5.
Amyloidosis
Felty’s syndrome
Methotrexate toxicity
Neuroendocrine tumour
Sarcoidosis
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Question 8
A 59 year old lady presented to the medical admission unit with arthralgia, sore
throat and an intermittent temperature for 10 days. On further questioning she has
had similar symptoms previously. She has no other significant past medical history.
Examination reveals synovitis of the small joints of the hands and a faint macular
papular rash was noted in the A&E but has completely resolved now. Her
temperature is 37.50C but was 390C in A&E. Chest X-ray is normal as is urine
dipstick. Throat swabs, blood cultures, urine dipstick, and a 2D echocardiogram are
normal.
Hb 9.6 g/dl(11-14.7)
MCV 76.4 fl(80-98.1)
Plts 777 x10/l(140-370)
WCC 18.6 x10/l(3.5-9.5)
Neu 16..0 x19/l(1.7-6.5)
ESR 100 mg/l
CRP120 mg/l
ALT 44 iu/l
AST 56 iu/l
Which one of the following investigations would you do next?
1.
2.
3.
4.
5.
ACE
Anti-nuclear-antibody
Anti- DNase titre
Ferritin
Viral throat swab
Question 9
A 55 year old man is referred to your clinic with skin thickening. There is no history of
Raynaud’s and he has no problems with swallowing. He is ANA negative. On
examination, he has thickening of the skin of both arms, but sparing of the hands. On
lifting his arm, the veins appeared sunken.
What is the most likely histological finding on biopsy of the involved skin?
1. Normal epidermis, thickened and sclerosed reticular dermis with
dermal expansion extending into subcutaneous tissue. Few adnexal
structures seen. No cellular proliferation, minimal inflammatory
component. (Morphoea)
2. Normal epidermis and dermis. The deep fascia and lower
subcutaneous tissues are oedematous, thickened and infiltrated with
lymphocytes, plasma cells, histiocytes and eosinophils. (Eosinophilic
fasciitis)
3. The dermis contains an increase in fibroblasts, collagen and interstitial
mucin. The fibroblasts are irregularly arranged, and the collagen has a
whorled pattern. A sparse perivascular lymphocytic infiltrate is present.
(Scleromyxoedma)
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4. There is a normal epidermis with subjacent pigment incontinence in the
papillary dermis. The reticular dermis is replaced by a thick band-like
area of collagen. There is a decrease in adnexal structures. There is
sparse mild superficial perivascular lymphocytic infiltrate. (Limited
cutaneous systemic sclerosis)
5. Whorled, haphazardly arranged collagen bundles are seen in
association with an increased number of spindled fibroblasts. There are
scattered collections of multinucleated giant cells. There is no other
inflammatory infiltrate. (Nephrogenic systemic fibrosis)
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Answers:
Q1.
2. Chest x-ray
The triad of erythema nodosum, synovitis and bilateral hilar lymphadenopathy is very
suggestive of Löfgren's syndrome (Sarcoidosis). This usually carries a good
prognosis.
Q2.
2. Kawasaki disease
This child has presented with Kawasaki disease.
The diagnosis requires the
following fever for >5days and 4/ 5 of the following conjunctivitis, rash,
lymphadenopathy, strawberry tongue and desquamation of the hands and feet. The
most important first line management is high dose aspirin.
Q3.
5. Prednisolone
This patient has features consistent with Sweet’s syndrome, an acute febrile
neutrophilic dermatosis. Prednisolone is the initial treatment of choice
Q4.
3. MEFV
This patient has a periodic fever. He is of Greek origin which suggests Familial
Mediterranean fever. This is associated with the MEFV gene mutation. CIASI is a
gene mutation associated with the Cryopyrin-associated periodic syndromes
(CAPS). CINCA is a subtype of CAPS. MVK mutation is associated with Hyper-IgD
syndrome. TNFRFSFIA mutation is found in TNF receptor associated periodic
syndrome (TRAPS).
Q5.
1.Biopsy of abdominal fat
This patient has symptoms and signs of peripheral neuropathy, hepatosplenomegaly, and congestive cardiac failure. He has raised total protein with low
albumin suggesting the presence of a circulating protein. With a background of RA
he is most likely to have developed reactive serum AA amyloidosis.
The quickest test to confirm your diagnosis would be either rectal or abdominal
subcutaneous fat biopsy stained for congo red. An abdominal subcutaneous fat
biopsy is preferred as it is a more straightforward procedure than a rectal biopsy.
However, in some centres rectal biopsy may be the preferred investigation.
Q6.
1.Colchicine
This patient has a periodic fever. He is of Turkish origin which suggests Familial
Mediterranean fever. The most useful long-term treatment is colchicine as it can
prevent the onset of Amyloidosis.
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Q7.
5.Amyloidosis
This patient has symptoms and signs of malabsorption, nephrotic syndrome and
hepatosplenomegaly. He has raised total protein with low albumin suggesting a
circulating protein. With a background of RA he is most likely to have developed
reactive serum AA amyloidosis.
Q8.
4. Ferritin
Adult onset Still’s disease (AOSD) is the most likely differential diagnosis. Typical
features of AOSD include a quotidian fever often associated with a rash when the
fever is present (salmon coloured, macular popular and often confused as a drug
reaction). Patients often have sore throat. Other features include deranged
transaminase, synovitis of small joints and myalgia.
Pleuritis, pericarditis,
lymphadenopathy and splenomegaly have been reported. The results of serum
ferritin should be interpreted with caution, as it is an acute phase reactant, and likely
to be elevated in those with any acute illness, but typically in those with AOSD the
serum ferritin levels are extremely elevated. The pronounced neutrophilia makes a
viral illness unlikely. Anti-DNAse antibody is positive in those with previous
streptococcal infection, and not in those with streptococcal sore throat currently.
There are no other features to suggest SLE, or ANCA associated vasculitis.
Q9.
2.Normal epidermis and dermis. The deep fascia and lower
subcutaneous tissues are oedematous, thickened and infiltrated with
lymphocytes, plasma cells, histiocytes and eosinophils.
Eosinophilic fasciitis is a scleroderma mimic. It is associated with venous guttering.
Characteristic histological features are described in 2 above. Option 1 describes
morphoea, 3 describes scleromyxoedma, 4 describes limited cutaneous systemic
sclerosis, and 5 describes nephrogenic systemic fibrosis. For further details and
pictures, please refer to http//dermatlas.med.jhmi.edu/derm/.
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