.
I. Proximal myopathy
J. Photosensitive rash.
Correct Answer: EF
Comment
Fibromyalgia is a diagnosis of exclusion. All blood
tests should be normal. Depression, irritable bowel
syndrome, unexplained headache and unexplained
chest pain are common in these patients.
6. A 26-year-old man with recurrent chest
infections is diagnosed as having common
variable immunodeficiency (CVID). From the
following, select the two most common
infectious agents responsible for disease in
this condition.
A S pneumoniae
.
B H influenzae
.
C E coli
.
D M tuberculosis
.
E. Enteroviruses
F. P Carinii
G Aspergillosis
.
H Nocardia
.
I. T gondii
J. L
monocytogenes
.
Correct Answer: AB
Comment
Ninety-five per cent of patients with CVID present
with recurrent sinopulmonary infections,
occurring as a result of encapsulated bacterial
infection, most of which are due to S pneumoniae
and H influenzae.
CVID patients are also predisposed to chronic
enteroviral infection, but fortunately this
complication is rare.
7. A 55-year-old woman presents with a 3month history of a painful swollen knee.
Examination shows restricted, painful
movement and a moderate sized effusion.
Synovial fluid is aspirated for diagnostic
purposes. Which two of the following findings
in the synovial fluid would be most compatible
with a final diagnosis of osteoarthritis?
Correct Answer: HI
Comment
This patient has presented with the pulmonaryrenal syndrome, which is most commonly caused
by the small vessel vasculitides (Wegeners
granulomatosis and microscopic polyangiitis (MP))
although Goodpastures disease, lupus and
cryoglobulinaemic vasculitis should also be included
in the differential diagnosis. ANCA positivity in this
patient group has a positive predictive value >90%
for diagnosing small vessel vasculitis. Although
specificity to MPO is more commonly associated with
microscopic polyangiitis, 25% of patients with
Wegners will also have a positive MPO. It is therefore
not possible to differentiate these two diseases,
based on ANCA specificity, nor is it important as
immunosuppressive treatment is indicated
regardless. A diagnosis of lupus is highly unlikely with
a negative ANA and >90% of patients with
cryoglobulinaemic vasculitis will have a low C4 as a
result of classical pathway activation.
10. A 70-year-old woman presents with
inflammatory joint pain, fatigue and a dry
mouth. She is found to have marked
hypergammaglobulinaemia and a raised
erythrocyte sedimentation rate with a normal
C-reactive protein.
What is the most likely diagnosis?
A Rheumatoid arthritis
.
B Pyrophosphate
. arthropathy
C Primary Sjgren's
. syndrome
D Systemic lupus
. erythematosus
E. Fibromyalgia
Correct Answer: C
Comment
Sicca symptoms, a raised erythrocyte sedimentation
rate but normal C-reactive protein (CRP) and
hypergammaglobulinaemia are classic features of
primary Sjgren's syndrome. This syndrome tends
to begin in the fifth and sixth decades compared with
lupus, which typically begins between the second
and fourth decades. This patient could have
rheumatoid arthritis but this is unlikely since the CRP
is normal. Blood tests are usually normal in patients
with primary fibromyalgia.
11. An 80-year-old woman is admitted having
tripped over a mat. Physical examination is
apparently unremarkable. She is unable to
weight bear and the Orthopaedic team have
discharged her with a normal pelvic
radiograph. She has been transferred to a
medical ward as she is still not able to walk.
What investigation(s) would you consider that
Comment
In a patient presenting with a monoarthritis, the most
important diagnosis to consider/exclude is a septic
arthritis. This is best diagnosed by microscopy and
culture of the synovial fluid. Radiography will be
helpful in determining if the patient has pre-existing
osteoarthritis or chondrocalcinosis. A raised white
cell count might suggest infection. A raised Creactive protein would indicate inflammation or
infection. This patient has mild heart failure and so
may be taking bendroflumethiazide or loop diuretics,
which may cause hyperuricaemia and gout and
hence a monoarthritis.
A Ankylosing spondylitis
.
B Pseudogout
.
C Systemic lupus erythematosus
. (SLE)
D Primary generalized
. osteoarthritis
E. Reactive arthritis
F. Gout
G Rheumatoid arthritis
.
H Psoriatic arthropathy
.
I. Systemic sclerosis
J. Rheumatic fever.
Correct Answer: CG
Comment
The most likely cause of these symptoms is
rheumatoid arthritis, but SLE requires careful
consideration.
Rheumatoid arthritis can present as an acute
monoarthritis, an acute polyarthritis, a subacute
insidious polyarthritis, or with a polymyalgic
presentation (particularly in the elderly, when it
needs to be differentiated from polymyalgia
rheumatica).
Remember that acute synovitis in early rheumatoid
arthritis is associated with very little fixed joint
deformity and no extra-articular features. This
contrasts with the deforming arthritis that is
characteristic of chronic disease.
17. A 30-year-old woman presents with a 3month history of recurrent episodes of
urticaria. There are no clues in the history to
suggest a possible trigger. During each episode
she is tired but otherwise well and clinical
examination is unremarkable. What is the most
likely diagnosis?
A C1 inhibitor deficiency
.
B Urticarial vasculitis
.
C Food allergy
.
D Systemic mastocytosis
.
E. Chronic idiopathic
urticaria.
Correct Answer: E
Comment
The cardinal presenting feature of C1 inhibitor
deficiency is angioedema.
E. Hepatitis C antibodies
F. Extractable nuclear antibodies
(ENA)
G Endomysial antibodies
.
H Thyroid antibodies
.
I. Rheumatoid factor
J. Serum creatinine.
Correct Answer: GH
Comment
This womans symptoms are suggestive of
cryoglobulinaemia and the low C4 would be
compatible with complement consumption due to
mixed or polyclonal cryoglobulins. Cryglobulins are
proteins that precipitate at temperatures lower
than 37C. This results in sludging of the circulation
in the extremities, and sometimes vasculitis,
especially of skin and kidneys. The commonest
causes are hepatitis C and Sjrgrens syndrome.
C myeloma
.
D osteoporosis
.
E. osteoarthritis.
Correct Answer: A
Comment
The history is typical of a patient with mechanical
back pain, best treated by encouraging
mobilization, simple analgaesia, a graded
rehabilitative exercise programme and treatment of
depression (if present).
Red flag symptoms, requiring urgent
investigation to exclude sinister pathology, include:
progressive pain
night pain
systemic symptoms
recent trauma.
28. A 25-year-old man presents with a 3-week
history of haemoptysis and testicular pain. The
only significant findings on examination are
testicular tenderness and left-sided
episcleritis. The results of initial investigations
are as follows: serum CRP 124 g/l (nr <8), urine
diabetes mellitus,
hemochromatosis,
Wilson's disease,
hypothyroidism,
hyperparathyroidism,
hypomagnesemia,
hypophosphatasia.
31. A 30-year-old man has a swollen wrist, low
back pain with early morning stiffness lasting 2
hours and mouth ulcers. He does not have a
rash. What is the most likely diagnosis?
A Rheumatoid arthritis (RA)
.
B Psoriatic arthritis
.
C Ankylosing spondylitis
.
D Gout
.
E. systemic lupus erythematosus
(SLE)
Correct Answer: C
Comment
The history is suggestive of a seronegative
spondyloarthropathy.
.
D Rheumatoid arthritis
.
E. Sjogren's disease.
Correct Answer: C
Comment
Raynauds phenomenon is common, occurring in
4-15% of the general population and in the majority
of cases is not associated with connective tissue
disease. However, the detection of an anticentromere nuclear staining pattern has a high
specificity for limited systemic sclerosis,
especially in the context of dysphagia which is a
feature of the disease.
33. An 18-year-old student with known asthma
and peanut allergy collapses following a meal
in the hospital canteen. Strenuous attempts at
resuscitation are tragically unsuccessful. It is
thought that the most likely cause of his
collapse was an anaphylactic reaction to
hidden' nuts contained in the meal.
Which of the following mast cell-derived
mediators is it most useful to measure to
confirm the clinical suspicion of anaphylaxis?
A Mast cell - derived growth
. factor
B Chymase
.
C Interleukin-6
.
D Tryptase
.
E. Transforming growth factor
Correct Answer: D
Comment
Amongst the array of mediators released by mast
cells during anaphylaxis, tryptase has proven to be
a reliable marker of mast cell degranulation on
account of its stability, relatively long half-life and
ease of measurement. Consequently, an elevated
tryptase in the correct clinical context is a useful
surrogate marker of an anaphylactic/anaphylactoid
reaction.
34. A 60-year-old woman develops acute,
severe low thoracic back pain. A radiograph
shows a vertebral crush fracture. Which two of
the following reduce an individual's risk for
developing osteoporosis?
A Early menarche
.
B Early menopause
.
C Smoking
.
D High alcohol intake
.
E. Prolonged treatment with
corticosteroids
F. Rheumatoid arthritis
G Crohn's disease
.
H Asthma
.
I. Frequent walks
J. Obesity.
Correct Answer: AI
Comment
The most likely cause of a vertebral crush factor in a
woman of this age is obviously osteoporosis, but
local vertebral pathology - in particular secondary
tumours or myeloma - would need to be considered.
It is most likely that the cause of osteoporosis would
be postmenopausal in this woman, but many medical
factors influence the likelihood of this condition.
The following tests should be considered in a
patient presenting with an osteoporotic
fracture (although not all need to be performed in
all cases): full blood count, renal / liver / bone
chemistry, immunoglobulins and serum / urine
electrophoresis (to exclude myeloma), thyroid
function, testosterone (in men), investigations for
Cushing's syndrome.
.
D radial pulse of 40 beats per minute
.
E. persistent cough, haemoptysis and weight
loss of 12 kg in the last three months
Correct Answer: C
Comment
This patient is not able to stand up from a chair
unaided. This indicates proximal muscle
weakness. The skin rash and the raised levels of
muscle enzyme point towards an inflammatory
myopathy, namely dermatomyosytis (DM).
DM is easily recognized and diagnosed by a
characteristic rash:
.
C Low-dose aspirin alone
.
D Low-dose aspirin combined with low
. molecular weight heparin
E. Intravenous immunoglobulin.
Correct Answer: D
Comment
Of the above treatment options, low-dose aspirin
combined with low molecular weight heparin offers
her the best chance of having a successful
pregnancy.
40. You are called to A&E to review a 28-yearold woman with pleuritic chest pain. Which of
the following statements concerning this
patient are true?
A Low white count is consistent with systemic
. lupus erythematosus (SLE)
B Low white count is not consistent with
. tuberculosis.
C The presence of anticardiolipin antibodies
. IgG 17 GPLU/ml (NR <14), IgM 21 MPLU/ml
(NR<10) suggests that she may have a
pulmonary embolus.
D The presence of antinuclear antibodies at a
. titre of 1/80 suggests that she may have a
connective tissue disease.
B Antinuclear antibodies
.
C Antiglomerular basement membrane
. antibodies
D Antimyeloperoxidase antibodies
.
E. Cryoglobulins
Correct Answer: E
Comment
The combination of a markedly low C4 (with
normal C3), elevated rheumatoid factor,
elevated serum IgM on a background of active
urinary sediment and a history of blood
transfusion is highly suggestive of hepatitis Cassociated cryoglobulinaemic vasculitis. Of the
investigations listed, cryoglobulins are the single
most important test in establishing a definitive
diagnosis in this patient.
43. A patient with psoriatic arthritis has active
joints and troublesome plaque psoriasis. Which
of the following will improve both the joint and
skin problems?
A Sulphasalazine
.
B Hydroxychloroqui
. ne
C Gold
.
D Methotrexate
.
E. Penicillamine.
Correct Answer: D
Comment
Hydroxychloroquine can exacerbate psoriasis.
Sulphasalazine tends to only improve joint symptoms
and not improve the psoriasis. Gold and
penicillamine are not commonly used to treat this
condition.
44. A 45-year-old woman developed Raynaud's
2 years ago. She now complains of
breathlessness and skin tightness affecting her
fingers. A high-resolution CT scan shows
evidence of pulmonary fibrosis.
Which of the following tests is most likely to be
positive?
A Anticentromere antibody
.
B Anti-double-stranded DNA
. antibody
C Anti-Ro antibody
.
D
. Anti-Scl-70 antibody
E. Anti-Jo-1 antibody
Correct Answer: D
Comment
This patient has clinical symptoms suggestive of
diffuse cutaneous systemic sclerosis. Pulmonary
fibrosis and anti-Scl-70 are more common in patients
with diffuse disease. Anticentromere antibody is
associated with limited cutaneous systemic sclerosis.
Anti-double-stranded DNA antibody is associated
with systemic lupus erythematosus. Anti-Ro antibody
is associated with lupus and primary Sjgren's
syndrome. Anti-Jo-1 is associated with polymyositis,
particularly in patients with inflammatory lung
disease.
case).
Involvement of the base of the thumb is also
pathognomonic of nodal osteoarthritis, giving the
thumb base a characteristically square appearance.
46. A 35-year-old man is referred to you for
investigation of recurrent infection. He has had
frequent respiratory tract infections for the
past 5 years, requiring 4-5 courses of
antibiotics each winter. A month previously he
was admitted with pneumococcal pneumonia.
Two months prior to that he had sinus surgery.
However, this did not improve his recurrent
sinusitis. Which of the following is not in the
differential diagnosis?
A Antibody deficiency
.
B HIV infection
.
C Bronchiectasis secondary to recurrent
. infection
D Complement C6 deficiency
.
E. Smoking 5 cigarettes per day.
Correct Answer: D
Comment
Antibody deficiency is typically associated with
respiratory tract infections. Ask about diarrhoea and
bacterial skin infections which are also common. Take
a careful drug history and bear in mind the possibility
D Neutrophils
.
E. Platlets
F. IL-10
G IFN-gamma
.
H Mannan-binding. protein
I. C4
J. IL-2.
Correct Answer: AG
Comment
Disseminated BCG infection is uncommon and
suggests an underlying immune deficiency. The
immune response to mycobacterial infection is
characterised by granulomas composed of T-cells
and macrophages, whose development
critically involves interferon gamma, IL-12 and
TNF. Patients with inherited deficiencies of IL-12 and
IFN-gamma are predisposed to disseminated BCG
infection and tuberculosis is a recognised risk in
patients treated with anti-TNF therapy.
48. A 65-year-old man is admitted as an
emergency with a very hot, swollen left knee.
On examination he is unwell and pyrexial. He
has marked loss of range of movement,
secondary to pain. You have aspirated his knee
Correct Answer: BF
Comment
This patient has suffered an anaphylactic reaction
to bee venom. A rise in serum tryptase (if taken
within 6 hours) can help in identifying cases of
anaphylaxis where there is diagnostic uncertainty.
However, in this patient the classical features and
temporal relationship to a bee sting establish the
diagnosis clinically. He should be tested for venom
specific IgE by both skin prick testing and RAST; if
either are positive his anaphylactic risk for a further
wasp sting can be dramatically lowered by venom
immunotherapy
51. A 50-year-old woman presents to casualty
with a 2-year history of recurrent angioedema.
Her clinical history fails to reveal an underlying
trigger. Her serum complement profile is as
follows: serum C3 1.2 g/l (ref range 0.75-1.65),
serum C4 <0.02 g/l (ref range 0.2-0.6). The
most likely diagnosis is:
A food allergy
.
B drug allergy
.
C Idiopathic
. angioedema
D C1 inhibitor
. deficiency
E. venom allergy.
Correct Answer: D
Comment
Uncontrolled activation of the classical complement
pathway leading to a marked reduction in serum C4
levels is a hallmark of C1 inhibitor deficiency. In
the absence of a family history, it is possible that this
represents a new mutation. Alternatively, the
possibility of acquired C1 inhibitor deficiency due to
lymphoproliferative disease should be considered.
52. A 77-year-old man presents with persistent
head ache and progressive deafness. On
examination the patient has frontal bossing of
the forehead and conductive deafness, more
severe in the right ear. His serum alkaline
phosphatase is significantly raised at 870 u/L.
Which of the following statements is most
accurate about this disease?
A It usually affects a single bone.
.
B The skull is the most commonly affected
. bone.
C Bone pain is the most common presenting
. feature.
D Hearing loss is often due to involvement of
. the middle ear ossicles leading to conductive
deafness.
affect outcome
Correct Answer: C
Comment
Persistently high alcohol consumption is a
common cause of poor response to allopurinol,
although the underlying mechanism of this is unclear.
B, D and E are plausible answers, but are less
important in practice. Most adults will respond to
allopurinol 300 mg daily, although a small proportion
will require 600 or even 900 mg daily. The aim of
treatment should be to suppress the serum urate
level to the lower end of the normal range or just
below.
58. A 68-year-old woman complained of
progressive pain in her left hip after slipping
on ice and sustaining a sub-capital fracture of
the left femoral neck hip. Following recovery
from a hemiarthroplasty, bone mineral density
(BMD) measurement of the opposite femur
confirmed the diagnosis of osteoporosis with a
T score of -2.67. She did not wish to take
oestrogen replacement therapy and was given
alendronate 70 mg weekly. Which of the
following statements is true?
A Normally the peak bone mass in a woman is
. achieved just before the menopause.
B Plain radiographs are very sensitive in
. identifying minor reduction in bone mineral
density and hence a very useful screening
test.
C The T score compares a patients BMD with
G Cardiomyopathy
.
H Chronic renal
. failure
I. Optic atrophy
J. Sacroileitis.
Correct Answer: BF
Comment
CVID patients suffer from recurrent pulmonary
infections, and resultant repeated structural lung
damage can progress to bronchiectasis.
Gastrointestinal symptoms are a common problem in
CVID with a significant proportion being attributed to
a celiac like enteropathy.
62. A 40-year-old woman presents with a 6month history of a purpuric rash on her legs,
non-specific joint pains and vague ill health.
Initial investigations reveal a positive
rheumatoid factor. The GP makes a tentative
diagnosis of rheumatoid arthritis and refers
her to hospital. Results of further
investigations are as follows: Urinalysis: red
cell casts, protein+, ANA 1/80, anti-DNA
negative, anti-ENA negative, serum C3 1.02 g/l
(NR 0.75-1.65), C4 <0.02 g/l (0.20-0.65), CRP
<5mg/l (NR <5), creatinine 145 (NR 50-140).
Which is the most likely diagnosis?
A Systemic lupus erythematosus
. (SLE)
B Rheumatoid arthritis
.
C Mixed cryoglobulinaemia
.
D Primary complement deficiency
.
E. C1 inhibitor deficiency.
Correct Answer: C
Comment
This lady has a hypocomplementaemic
glomerulonephritis (note active urine sediment) and
a purpuric rash accompanied by a positive
rheumatoid factor and an isolated positive ANA.
The combination of renal and skin involvement in this
setting with a markedly low C4 suggests mixed
cryoglobulinaemia. Her serology does not support
SLE.
The key investigations which would help confirm the
diagnosis would be a warm clotted sample for
cryoglobulins and a renal biopsy.
63. A 69-year-old man with rheumatoid
arthritis presents with increased
breathlessness and nose bleeds. Which of the
following treatments for rheumatoid arthritis
are unlikely to cause bone marrow
suppression?
A Gold
.
B Azathioprine
.
C Non-steroidal anti-inflammatory drugs
. (NSAIDs)
D Methotrexate
.
E. Sulphasalazine
F. Infliximab
G Leflunomide
.
H Cyclophosphamide
.
I. Penicillamine
J. Hydroxychloroquine
Correct Answer: CF
Comment
Many of the treatments for rheumatoid arthritis
cause bone marrow suppression. NSAIDs may be
used early in the disease and cause anaemia via
gastrointestinal ulceration. Infliximab has been
associated with multiple sclerosis-like demyelination,
the development of tuberculosis and worsening heart
failure.
64. A 70-year-old female has a 17-year history
of rheumatoid arthritis. She presents with
.
H Reactive arthritis
.
I. Torn medial meniscus
J. Spontaneous
haemarthrosis.
Correct Answer: DH
Comment
Reactive arthritis, consequent upon either genital
chlamydia trachomatis or enteric infection, is the
most likely diagnosis. He is a little younger than
many patients presenting with gout, but this is not a
rare diagnosis in this age group, particularly if
alcohol intake is high. The important diagnosis to
exclude is septic arthritis, but this is a rare cause of
monoarthritis in this age group. Aspiration of synovial
fluid is the key diagnostic tool to differentiate
between these diagnoses.
69. A 65-year-old woman with primary
Sjgren's syndrome, which was diagnosed 1
year previously, is noted to have a persistently
elevated erythrocyte sedimentation rate (ESR)
of 90-100 mm/hour and a normal C-reactive
protein (CRP). At a routine outpatient visit it is
noted that her sicca symptoms are still
troublesome, but there are no other
abnormalities on clinical examination. The
following investigations are performed:
haemoglobin 12.5 g/dL (normal range 12-16),
white cell count 6.4 x 109/L (normal range 411), platelet count 320 x 109/L (normal range
150-400), ESR 98 mm/hour, CRP <6 mg/dL
(normal <6), serum IgG 42 g/L (normal range 613), IgA 8.4 g/L (normal range 0.8-4.0) and IgM
3.6 g/L (normal range 0.4-2.0), and serum
electrophoresis shows polyclonal
hypergammaglobulinaemia.
Her persistently elevated ESR is best explained
by which of the following?
A Development of lymphoma
.
B Poorly controlled sicca symptoms
.
C Positive antinuclear antibody
.
D Positive antibodies to Ro and La
. antigens
E. Polyclonal
hypergammaglobulinaemia
Correct Answer: E
Comment
Together with fibrinogen, serum
immunoglobulins are the major driving force
causing an elevation in erythrocyte
sedimentation rate (ESR). Any disorder associated
with a persistently elevated polyclonal
hypergammaglobulinaemia, such as primary
Sjgren's syndrome, is likely to be linked with a
diseases.
Antibodies to Sm antigen are highly specific for
a diagnosis of SLE (>99%). However, only about
25% of patients with SLE have anti-Sm antibodies.
Anti-DNA antibodies are diagnostic of SLE
(specificity >99%). However, only 60% of patients
with SLE will have these antibodies. Therefore,
absence of anti-DNA or anti-Sm antibodies should not
exclude SLE as a diagnosis. Anti-Ro/SS-A antibodies
are found in 30% of patients with SLE. Anti-histone
antibodies are identified in small proportion of SLE
patients. They are more often seen with druginduced lupus.
D Gout
.
E. systemic lupus erythematosus
(SLE)
Correct Answer: C
Comment
The history is suggestive of a seronegative
spondyloarthropathy.
RA is classically a peripheral, symmetrical
polyarthritis.
Gout can affect the wrist but is unusual in a
young person.
Patients with SLE can experience mouth ulcers but
SLE is uncommon in young males.
39. A 25-year-old woman with a history of 3second trimester fetal losses is planning a
fourth pregnancy. She has evidence of the
primary anti-phospholipid syndrome (strongly
positive cardiolipin antibody, positive lupus
anticoagulant but no evidence of lupus). Which
of the following treatment regimens offer her
the best chance of having a successful
pregnancy?
A Steroids alone
.
B Steroids combined with low-dose aspirin
.
C Low-dose aspirin alone
.
D Low-dose aspirin combined with low
. molecular weight heparin
E. Intravenous immunoglobulin.
Correct Answer: D
Comment
Of the above treatment options, low-dose aspirin
combined with low molecular weight heparin offers
her the best chance of having a successful
pregnancy.
40. You are called to A&E to review a 28-yearold woman with pleuritic chest pain. Which of
G Ankylosing spondylitis
.
H Rheumatoid arthritis
.
I. Reactive arthritis
J. SAPHO syndrome.
Correct Answer: BE
Comment
Swelling of the distal interphalangeal joints really
only occurs in nodal osteoarthritis and one of the
forms of psoriatic arthritis, which is usually easily
distinguished by nail involvement (not present in this
case).
Involvement of the base of the thumb is also
pathognomonic of nodal osteoarthritis, giving the
thumb base a characteristically square appearance.
46. A 35-year-old man is referred to you for
investigation of recurrent infection. He has had
frequent respiratory tract infections for the
past 5 years, requiring 4-5 courses of
antibiotics each winter. A month previously he
was admitted with pneumococcal pneumonia.
Two months prior to that he had sinus surgery.
However, this did not improve his recurrent
sinusitis. Which of the following is not in the
differential diagnosis?
A Antibody deficiency
.
B HIV infection
.
C Bronchiectasis secondary to recurrent
. infection
D Complement C6 deficiency
.
E. Smoking 5 cigarettes per day.
Correct Answer: D
Comment
Antibody deficiency is typically associated with
respiratory tract infections. Ask about diarrhoea and
bacterial skin infections which are also common. Take
a careful drug history and bear in mind the possibility
of lymphoproliferative disease. HIV infection,
although primarily associated with CD4 loss, also
results in antibody dysfunction, resulting in recurrent
respiratory tract infections in some patients. Ask
about features of cellular immune deficiency (oral
candida, herpes simplex and zoster, warts). Ask
about risk factors.
Recurrent bacterial chest infections, whatever their
cause, will eventually result in bronchiectasis,
hence the importance of early diagnosis and
treatment. Terminal complement deficiencies (C59) are extremely rare. Patients are well but have
increased susceptibility to neisserial infection.
Smoking causes ciliary paralysis. The resultant
mucociliary dysfunction is a common (and reversible)
cause of recurrent respiratory tract infection.
arthropathy
D tophaceous gout
.
E. Charcot joints.
Correct Answer: C
Comment
This is the classic appearance of arthritis mutilans
in psoriatic arthropathy, with a deforming destructive
arthritis of the small joints. Osteolysis results in
shortening of some of the digits. The pattern is not
that of rheumatoid arthritis and the wrists appear to
be spared. There is too much destruction for
osteoarthritis. The appearance of gout may be
similar, though the tophi would be easier to see in
life (as opposed to a photo). Charcot joints
(neuropathic arthropathy) usually affect the foot and
ankle.
57. A 56-year-old man presents with recurrent
attacks of polyarticular gout despite treatment
with allopurinol 300 mg daily for the last year.
He is adamant that his compliance with his
drug treatment regimen is good.
Which of the following factors is most likely to
explain his poor response to allopurinol?
A
. Concurrent treatment with
colchicine
D Lung cancer
.
E. Osteomalacia
Correct Answer: B
Comment
Cancer of the prostate is typically associated
with sclerotic bone lesions in contrast to the lytic
lesions seen in multiple myeloma. Osteomalacia is
associated with Looser's zones.
60. A 33-year-old woman presents with a 6month history of Raynaud's phenomenon
affecting her hands and feet. She is previously
well and takes no medication.
Which two of the following clinical features or
investigation results are the strongest
predictors that she will develop a connective
tissue disease in the future?
A Age >25 years
.
B Abnormal nail-fold capillary
. microscopy
C Elevated erythrocyte sedimentation
. rate
D History of recurrent miscarriage
.
E. Family history of Raynaud's
F. Anaemia
G Strongly positive antinuclear
. antibody
H History of chilblains
.
I. Raised platelet count
J. Dry eyes and dry mouth
Correct Answer: BG
Comment
These factors are strongly predictive of a future
connective tissue disease (CTD), particularly
abnormal nail-fold capillaries. The likelihood of
developing a CTD also increases with age of onset of
Raynaud's, with a particularly high risk in those aged
over 35 years. All the other features apart from a
family history (which suggests primary Raynaud's)
are associated with CTD, but have not been shown to
have the same predictive value as B and G.
61. A 22-year-old man has chronic diarrhoea
and has had several episodes of sinusitis and
pneumonia. A diagnosis of common variable
immunodeficiency (CVID) is made. Which two
of the following are recognized long-term
complications of this disorder?
A Type II diabetes
.
B Bronchiectasis
.
C Hyperparathyroidi
. sm
D Atheroma
.
E. Epilepsy
F. Enteropathy
G Cardiomyopathy
.
H Chronic renal
. failure
I. Optic atrophy
J. Sacroileitis.
Correct Answer: BF
Comment
CVID patients suffer from recurrent pulmonary
infections, and resultant repeated structural lung
damage can progress to bronchiectasis.
Gastrointestinal symptoms are a common problem in
CVID with a significant proportion being attributed to
a celiac like enteropathy.
62. A 40-year-old woman presents with a 6month history of a purpuric rash on her legs,
non-specific joint pains and vague ill health.
Initial investigations reveal a positive
rheumatoid factor. The GP makes a tentative
diagnosis of rheumatoid arthritis and refers
her to hospital. Results of further
investigations are as follows: Urinalysis: red
.
E. serum IgE
Correct Answer: B
Comment
A history of penicillin allergy is relatively common in
hospital patients, and is in most not due to type I
hypersensitivity reactions. A diagnosis of
penicillin allergy crucially requires a detailed history
of the drug reaction, and can be confirmed by a
positive skin prick test to the major and minor
determinants of penicillin. Skin prick testing is carried
out if there is a clinical need for penicillin treatment
e.g. treatment of infective endocarditis. A patient is
unlikely to develop anaphylaxis with a negative
penicillin skin prick test. The detection of penicillin
specific IgE in the serum is unreliable.
68. A 36-year-old man presents with a 2-day
history of severe pain in the left knee. He has
recently returned from a holiday in Spain.
Examination shows low grade fever (37.5C)
and marked synovitis of the left knee with a
tense effusion. Which are the two most likely
diagnoses?
A Lyme disease
.
B Rheumatoid arthritis
.
C Pseudogout
.
D Gout
.
E. Septic arthritis
F. Osteosarcoma
G Osteoarthritis
.
H Reactive arthritis
.
I. Torn medial meniscus
J. Spontaneous
haemarthrosis.
Correct Answer: DH
Comment
Reactive arthritis, consequent upon either genital
chlamydia trachomatis or enteric infection, is the
most likely diagnosis. He is a little younger than
many patients presenting with gout, but this is not a
rare diagnosis in this age group, particularly if
alcohol intake is high. The important diagnosis to
exclude is septic arthritis, but this is a rare cause of
monoarthritis in this age group. Aspiration of synovial
fluid is the key diagnostic tool to differentiate
between these diagnoses.
69. A 65-year-old woman with primary
Sjgren's syndrome, which was diagnosed 1
year previously, is noted to have a persistently
elevated erythrocyte sedimentation rate (ESR)
Comment
Almost all patients with SLE have a positive
ANA test result. The ANA test is sensitive but not
specific for SLE. A negative result argues strongly
against a diagnosis of active SLE, but does not
exclude the possibility of other autoimmune
diseases.
Antibodies to Sm antigen are highly specific for
a diagnosis of SLE (>99%). However, only about
25% of patients with SLE have anti-Sm antibodies.
Anti-DNA antibodies are diagnostic of SLE
(specificity >99%). However, only 60% of patients
with SLE will have these antibodies. Therefore,
absence of anti-DNA or anti-Sm antibodies should not
exclude SLE as a diagnosis. Anti-Ro/SS-A antibodies
are found in 30% of patients with SLE. Anti-histone
antibodies are identified in small proportion of SLE
patients. They are more often seen with druginduced lupus.
71. A 60-year-old-man complained of pain in
both wrist evolving over 8 weeks. He noted
swelling around that area but denied stiffness.
On examination there was swelling and
tenderness just proximal to the wrist joints
without limitation of movement. There was
also prominent finger clubbing. Radiographs
revealed periosteal reaction over the lower end
of the radius and ulnar. Each of the following
disorders could be the cause behind this
patient complain EXCEPT?
A Mesothelioma
.
B Bronchiectesis
.
C Diabetes
. mellitus
D Crohn's disease
.
E. Whipple's
disease.
Correct Answer: C
Comment
Hypertrophic osteoarthropathy (HOA) is a
syndrome characterized by proliferative changes in
the skin and skeleton. Proliferative periostitis of the
radius and fibula and digital clubbing are commonly
seen. Two forms of the syndrome are seen, a rare
idiopathic from called pachydermoperiostitis (35%) (familial autosomal dominant), and more
common secondary form (95-97%).
Secondary HOA was initially described in
association with chronic suppurative infection and
malignancy of the lung and pleura. Therefore it used
to be called hypertrophic pulmonary
osteoarthropathy (HPOA).
Pleural causes include pleural fibroma and
mesothelioma.
Pulmonary causes include bronchogenic
72. A 56-year-old patient presented with 6month history of excessive tiredness, stiffness
and pain across the upper and lower limbs. On
examination he has proximal muscle weakness.
The creatinine kinase was 10 times the upper
limits of normal. The skin lesions shown in the
image are most likely to be:
A garrods pads
.
B tendon xanthoma
.
C Gottron's papules
.
D rheumatoid
. nodules
E. gouty tophi.
Correct Answer: C
Comment
Gottron's papules, violaceous papules overlying
the dorsal interphalangeal or metacarpophalangeal
areas, elbow or knee joints, occur in approximately
70% of patients with dermatomyositis.
Garrods fatty pads (knuckle pad syndrome) is a
relatively common and harmless condition
characterized by discrete fibromatous skin pads over
the dorsum of the interphalangeal joints.
73. A 36-year-old woman presents with
deteriorating nocturnal paraesthesia affecting
both hands. It improves during the morning.
Which of the following tests is least likely to be
helpful in establishing the cause?
A Thyroid function
. test
B Rheumatoid factor
.
C Tinels test
.
D MRI scan hands
.
E. Urinary hCG.
Correct Answer: D
Comment
Carpal tunnel syndrome classically presents with
nocturnal paraesthesia in the palms of the hands
which improves by shaking / using the hands. It is
caused by compression of the median nerve within
the carpal tunnel in the wrist. MRI hands would not
be indicated.
Causes of Carpal tunnel syndrome include
hypothyroidism, synovitis including rheumatoid
arthritis and pregnancy. Tinels test is a provocation
test to reproduce symptoms by percussion over the
median nerve.
74. A 47-year-old woman had a splenectomy 10
years previously. She is at increased risk of
severe infection with which of the following
organisms:
A Aspergillus nigrans
.
B Epstein-Barr virus
.
C Falciparum malaria
.
D Mycobacterium avium
. intracellular
E. Papillomavirus
F. Chlamydia trachomatis
G Mycobacterium tuberculosis
.
H Mycoplasma hominis
.
I. Pneumococcus
J. Candida albicans
Correct Answer: CI
75. An 8-year-old has been admitted for a third
time with haemolytic-uraemic syndrome and is
found to have a low C3. Which of the following
is the most likely diagnosis?
A Lupus
.
B Gram-negative
. septicaemia
C Factor H deficiency
.
D Factor I deficiency
.
E. C3 deficiency.
Correct Answer: C
Comment
Factor H deficiency accounts for 5-10% of all cases
of haemolytic-uraemic syndrome. Typically it has
a higher mortality and over half of patients will suffer
relapses. Over-activation of the alternative
complement pathway results in a low serum C3.
Although able to cause a low C3, lupus, factor I and
C3 deficiency are not associated with recurrent
haemolytic-uraemic syndrome.
76. A 50-year-old man gives an 8-month history
of episodic, painful soft tissue swellings
involving his hands, eyes and lips. There is no
temporal relationship to food. Which of the
following tests is the most useful?
A Skin prick tests to various food
. allergens
B Complement
.
C Full blood count (FBC)
.
D Glucose tolerance test
.
E. Urinary 5-hydroxyindoleacetic acid.
Correct Answer: B
Comment
Correct Answer: E
Comment
Poor adherence is a common cause of treatment
failure or early relapse; directly observed therapy
(DOT) may improve adherence if this is the case. TB
usually responds well to conventional treatment even
if the patient is co-infected with HIV. In this situation,
recurrent TB is most often caused by poor
adherence (early) or reinfection (late).
Underlying IFNg receptor/ IL12 deficiency is
extremely rare and is associated with disseminated
disease, usually with poorly pathogenic
environmental mycobacteria.. Environmental
mycobacteria may occasionally grow as
contaminants in culture, but it is most unusual for
MTB to do so.
78. The A&E SHO rings for your advice
concerning a 57-year-old man who attended
following a dog bite which required suturing.
He has had a tetanus booster and is taking
penicillin 250mg bd. Twenty four years
previously he had a splenectomy following a
road traffic accident. He has remained well
since. Which of the following statements are
true?
A Large granular lymphocytes may be visible
. on blood film.
B He should avoid killed vaccines.
.
swelling
C
. Fatigue
D Arthralgia
.
E.
Pleural
effusion
Correct Answer: E
Comment
Sjrgrens syndrome results from an inflammatory
lymphocyte infiltrate of salivary glands. The most
common symptoms are dry eyes and mouth, with
fatigue. Many people do not volunteer these
symptoms unless asked, for example by the dentist,
aware that lack of saliva predisposes to caries.
Arthralgia is common in primary Sjrgrens.
Secondary Sjrgrens is associated with a variety of
connective tissue diseases, which may themselves
be a cause of arthralgia.
Tricyclic antidepressants are an unrelated cause of
dry mouth.
81. A 79-year-old man presents feeling very
unwell with recent and sudden onset of severe
pain and stiffness around his shoulders and
hips. He was previously well and admits to no
other symptoms, in particular no headache or
visual disturbance. Examination shows painful,
restricted shoulders and hips, but no
sacroiliac joints
symphysis pubis
the elbow
the ankle
acromioclavicular joint.
CPPD is associated with hemochromatosis,
primary hyperparathyroidism, hypothyroidism,
and hypomagnesemia. Other findings include
joint space narrowing, large subchondral cysts and
many intra-articular bodies from subchondral bone
fragmentation.
Comment
The constellation of acute symptoms in this case is
highly suggestive of a systemic allergic reaction,
either anaphylaxis (if IgE mediated) or an
anaphylactoid reaction (if non-IgE mediated). Both of
these reactions are due to extensive mast cell
degranulation leading to release of large amounts of
tryptase in to the circulation. Elevated tryptase
levels in the context of this lady's reaction are
highly suggestive of an
anaphylactic/anaphylactoid reaction.
This lady will need to be investigated during
convalescence in an allergy clinic to determine the
cause of her reaction.
89. A 68-year-old woman with longstanding
congestive cardiac failure (ejection fraction
20%) presents with a hot, swollen right knee.
The following results are obtained: FBC
normal, Urea 11 mmol/l, Creatinine 196 umol/l.
Synovial fluid: many monosodium urate
crystals seen on microscopy, culture sterile.
What is the best treatment for her acute
arthritis?
A Allopurinol
.
B Colchicine 0.5mg every 2-4
. hours
C Indomethacin 50mg tds
.
A Anticentromere antibody
.
B Anti-double-stranded DNA
. antibody
C Anti-Ro antibody
.
D
. Anti-Scl-70 antibody
E. Anti-Jo-1 antibody
Correct Answer: D
Comment
This patient has clinical symptoms suggestive of
diffuse cutaneous systemic sclerosis. Pulmonary
fibrosis and anti-Scl-70 are more common in
patients with diffuse disease. Anticentromere
antibody is associated with limited cutaneous
systemic sclerosis. Anti-double-stranded DNA
antibody is associated with systemic lupus
erythematosus. Anti-Ro antibody is associated with
lupus and primary Sjgren's syndrome. Anti-Jo-1 is
associated with polymyositis, particularly in patients
with inflammatory lung disease.
93. A 30-year-old teacher presents with a 6month history of swelling and pain involving
the distal interphalangeal joints of her hands.
The ESR is 65 mm in the first hour. What is the
most likely diagnosis?
A Generalised osteoarthritis
.
B Rheumatoid arthritis
.
C Psoriatic arthritis
.
D Systemic lupus erythematosus
. (SLE)
E. Gout.
Correct Answer: C
Comment
Arthritis with predominant involvement of the distal
interphalangeal joint occurs most often in
generalised osteoarthritis and psoriatic arthritis. The
fact that this patient is relatively young and has a
raised ESR indicates an underlying inflammatory
disease is the most likely cause of her symptoms.
Therefore psoriatic arthritis is the most likely
diagnosis in this case.
Rheumatoid arthritis and SLE are known to affect
the proximal interphalangeal (PIPs) and the
metacarpophalangeal (MCPs) joints.
Chronic gouty arthritis might involve the DIPs, but
more often it involves the MCPs and PIPs in
asymmetrical fashion with or without tophus
formation.
Examination of the skin and nail for psoriasis is very
important in confirming the diagnosis.
.
C Rheumatoid
. arthritis
D Driving a car
.
E. Frusemide
F. Oral contraceptive
pill
G Hydrocortisone
.
H Typing
.
I. Wrist held in
flexion
J. Osteoarthritis.
Correct Answer: EG
Comment
This patient has developed carpal tunnel
syndrome due to pressure on the median nerve.
Carpal tunnel syndrome (CTS) is more common in
patients with hypothyroidism, diabetes mellitus (DM),
rheumatoid arthritis (RA), osteoarthritis and those
taking the oral contraceptive pill. Mechanical factors
can also exacerbate CTS, particularly those that
increase flexion of the wrist e.g. driving and typing.
98. A 20-year-old woman is rejected as a blood
donor on account of Fe deficiency anaemia.
antibodies
E. Anti-PM-Scl
Correct Answer: A
Comment
The picture shows puffy hands, and fingers with swan
neck and boutonniere deformities. The fact that she
had gross deformities of the hands without joint
damage or erosions, might suggest that she has
Jaccoud's arthropathy, which is more often seen in
patients with connective tissue disease such as
systemic lupus erythematosus (SLE). This patients
also exhibits features probably suggestive of an
overlap between SLE, myositis and scleroderma
which constitute the diagnosis of mixed connective
tissue disease (MCTD).
MCTD is an overlap syndrome characterised by
combinations of clinical features of SLE, systemic
scleroderma and polymyositis. The presenting
symptoms of MCTD are most often:
Raynaud's phenomenon
puffy hands
arthralgias
myalgias
fatigue.
The various features of the connective tissue
disorders making up MCTD develop over months and
years. A defining feature of MCTD is the presence
of antibodies against the U1 ribonucleoprotein
(U1 RNP) complex, and hence the presence of high
titre anti-U1 RNP will confirm the clinical diagnosis of
Correct Answer: AF
Comment
The C-reactive protein is a more sensitive marker
of inflammation and infection than the ESR. The
normal CRP in this lady fits with the clinical picture of
disease inactivity. Therefore, the raised ESR must
be secondary to a cause other than inflammation or
infection. Such causes include hyperlipidaemia,
anaemia and hypergammaglobulinaemia, all of
which frequently occur in SLE.
104. A 32-year-old man presents with a 4month history of back pain. The pain is worse
in the morning and after sitting watching TV.
Plain radiograph of the spine/pelvis shows
evidence of sacroiliitis. Each of the following
clinical features might be identified on clinical
examination of this patient EXCEPT?
A Keratoderma
. blenorhegica
B Rheumatoid nodule
.
C Onycholysis
.
D Uveitis
.
E. Urethritis.
Correct Answer: B
Comment
The long (> 1 hr) early morning and rest stiffness are
highly suggestive of an underlying inflammatory
condition. The pelvic radiograph confirms the
presence of sacroiliitis. Bilateral sacroiliitis are typical
for ankylosing spondylitis. The frequency of
asymmetric sacroiliitis may be higher in other
spondyloarthropathies, e.g., reactive arthritis,
Reiters syndrome, spondylitis associated with
psoriasis, or inflammatory bowel disease.
Reiter's syndrome is characterised by a triad of
arthritis, urethritis, and conjunctivitis. Reiter's
syndrome develops in the setting of postdysenteric
or postvenereal illness. The characteristic rashes of
keratoderma blennorrhagicum and circinate
balanitis may be present.
Psoriatic spondyloarthropathy is characterised by
psoriatic plaques. The skin involvement may be
subtle and should be searched for carefully. The cleft
of the buttock, scalp hairline, and penis are site often
involved but may be missed easily if thorough
examination of these areas was not actively
conducted. Psoriatic nail changes include
onycholysis, yellow nails and nail pitting.
Inflammatory bowel disease consists of ulcerative
colitis and Crohn's disease.
A diagnosis of ankylosing spondylitis may be made
when specific features of Reiter's syndrome,
psoriasis, or inflammatory bowel disease are absent.
D Systemic sclerosis
.
E. Psoriatic arthritis
F. Systemic lupus
erythematosus
G Ankylosing spondylitis
.
H Rheumatoid arthritis
.
I. Reactive arthritis
J. SAPHO syndrome.
Correct Answer: BE
Comment
Swelling of the distal interphalangeal joints really
only occurs in nodal osteoarthritis and one of the
forms of psoriatic arthritis, which is usually easily
distinguished by nail involvement (not present in this
case).
Involvement of the base of the thumb is also
pathognomonic of nodal osteoarthritis, giving the
thumb base a characteristically square appearance.