Rheumatology Flashcards

1
Q

A 36-year-old woman presents to the rheumatology outpatient clinic with a
two-month history of stiff hands and wrists. She mentions that the pain is
particularly bad for the first few hours after waking up and is affecting her work as
a dentist. On examination, the wrists, metacarpophalangeal joints and proximal
interphalangeal joints are swollen and warm. What is the most likely diagnosis?
A. Rheumatoid arthritis
B. Osteoarthritis
C. Septic arthritis
D. Polymyalgia rheumatica
E. Reactive arthritis

A

A. Rheumatoid arthritis

The presentation of chronic joint pain that is characterised with morning stiffness is strongly suggestive of an rheumatiod process. The pattern of symetrical swelling and pain affecting the MCP and PIP joints is also classic.

Osteoarthritis (B) also affects the hands, however it usually affects the DIP joints first. The pain is also charecteristically worse after use or at the end of the day.

Sceptic arthritis (C) usually presents at a monoarthritis, and the time course is to long for an infective process.

Polymyalgia rheumatica (D) is rare in those under 60 years of age. It is characterised by pain and stiffness in the shoulders, neck, and lumbar spine. As expected it is worse in the morning.

Reactive arthritis (E) is usually a lower limb, asymetrical arthritis that arises following an infection, typically a STI.

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2
Q

A 45-year-old woman presents to the rheumatology outpatient clinic with a threemonth history of stiff hands and wrists. She mentions that the pain is particularly
bad first thing in the morning. On examination, the wrists, metacarpophalangeal
joints and proximal interphalyngeal joints are swollen and warm. A diagnosis of
rheumatoid arthritis is suspected. Which of the following investigations is most
specific for confirming the diagnosis?
A. X-rays
B. Rheumatoid factor levels
C. Anti-citrullinated peptide antibody (anti-CCP) levels
D. C-reactive protein
E. Erythrocyte sedimentation rate

A

C. Anti-citrullinated peptide antibody (anti-CCP) levels

anti-ccp (C) levels are teh most specific investigation for rheumatoid arthritis.

X-rays (A) will show soft tissue swelling in the early stages of the disease, and although not diagnostis is useful for establishing a baseline.

Rheumatoid factor (B) will be positive in around 70% of cases of rheumatoid arthritis but is not specific. It is however the defualt test for RA, and anti-CCP is not routinely sent for, it tends to be sent for if the rheumatoid factor is negative.

CRP (D) is very non-specific, but it it used in diagnosed rheumatoid arthritis to guidde treatment and monitor responce.

ESR (E) is equally vauge.

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3
Q

A 40-year-old woman presents to the rheumatology outpatient clinic with a three month history of stiff hands and wrists. She mentions that the pain is particularly
bad first thing in the morning. On examination, the wrists, metacarpophalangeal
joints and proximal interphalangeal joints are swollen and warm. A diagnosis of
rheumatoid arthritis is suspected. Blood tests for rheumatoid factor return as
positive. What is the most appropriate management?
A. Non-steroidal anti-inflammatory drugs (NSAIDs)
B. Intramuscular depot injection of methylprednisolone plus NSAIDs
C. Anti-TNF therapy
D. Short course of oral steroids plus NSAIDs and
methotrexate and sulfasalazine
E. Physiotherapy

A

D. Short course of oral steroids plus NSAIDs and
methotrexate and sulfasalazine

Latest NICE guidelines state:

  • Specialists will usually start a combination of disease-modifying anti-rheumatic drugs (DMARDs), plus a short-term corticosteroid.*
  • Ideally, treatment should be started within 3 months of the onset of symptoms.*
  • First-line treatment is usually methotrexate and at least one other DMARD.*
  • DMARDs require regular monitoring with blood tests. This may be done in secondary care but can carried out in primary care under a shared care agreement. For more information, see the CKS topic on DMARDs.*

Taking that into account, (D) would be the most appropriate course of action for this patient.

There has been a paradigm shift in the management of rheumatoid arthritis in recent years. Previously patients would be offered NSAIDs as a first line (A) and then progress on a stepwise ladder towards DMARDs and then biologics. The recognition now is that RA is a progressive disease and early disease modifying drug use alters the natural history and debilitation of the disease.

Option (B) here would be a useful treatment for managing an acute flare in a patient already under tretment.

Biological therapy (C) is the second line therapy for patients who continue to have symptoms despite the use of two DMARDs.

Physiotherapy (E) is a useful measure within a holistic treatment plan but is not going to change the course of the disease.

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4
Q

A 50-year-old woman, who has received a recent diagnosis of rheumatoid arthritis,
presents to her GP with ongoing pain and stiffness in her hands and feet. Which
joints are usually spared at onset of rheumatoid arthritis?
A. Proximal interphalangeal joints
B. Distal interphalangeal joints
C. Metacarpophalangeal joints
D. Wrists
E. Metatarsophalangeal joints

A

B. Distal interphalangeal joints

The classic first presentaton of rheumatoid arthritis will be seen in the MCP (C) and PIP (A) joints, often with involvement of the wrist (D). The MTP joints (E) can also be affected.

The DIP joints (B) are usually spared in the early disease process, so this is the answer to this question.

It is important to note, however, that this is a variable disease and some
patients may present with other joint involvement including elbows,
shoulders, knees or ankles. As the disease progresses and joint damage
occurs in the hands, a variety of deformities may be seen. These include
ulnar deviation and palmar subluxation of the metacarpophalangeal
joints, Boutonniere deformity (flexion of PIPs, hyperextension of DIPs),
swan-neck deformity (hyperextension of PIP, flexion of DIPs) or dorsal
subluxation of the ulnar styloid. Inflammation of the flexor tendon sheath
may result in carpal tunnel syndrome and inflammation of the extensor
tendon sheath can cause tendon rupture.

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5
Q

A 55-year-old man presents to his GP with a 2-week history of pain in his hands.
The pain is particularly bad in his right hand. On examination, brown discoloration
of the nails with onycholysis is noted and the distal interphalangeal joints are
tender on palpation. What is the most likely diagnosis?
A. Rheumatoid arthritis
B. Dermatomyositis
C. Reactive arthritis
D. Osteoarthritis
E. Psoriatic arthritis

A

E. Psoriatic arthritis

approximately 5% of patients with psoriasis (E) develop arthritis, it is a variable pattern of arthritis but commonly affects the DIP joints and is asymetrical. Nail and skin changes are usually present but may develop after the arthritis. The arthritis can also present symetrically, and therefore resemble rheumatoid arthritis. A small number of psoriatic patients can develop an extremely deforming arthritis, arthritis mutilans, this results in periarticular ostelysis and bone shortening.

The pattern of asymmetrical arthritis affecting the distal interphalangeal joints
with nail changes should indicate that psoriatic arthritis (E) is the correct answer.

An asymetrical condition with nail changes that affects the DIP joints doesn’t fit with a rheumatoid arthritis (A)

Dermatomyositis (B) presents with symmetrical proximal muscle weakness with characteristic skin changes. It does not cause a polyarthritis of the hands, thus making
this answer incorrect.

Reactive arthritis (C) is an asymmetrical lower limb
arthritis, making this answer wrong.

Osteoarthritis (D) may affect the distal interphalangeal joints, but does not cause nail changes, making this answer incorrect as well.

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6
Q

A 75-year-old woman presents to accident and emergency complaining of pain in
her knees. She mentions that this has been troubling her for several months. Pain
is generally worse in the evenings and after walking. On examination, there are
palpable bony swellings on the distal interphalangeal joints of the fingers on both
hands. In addition, there is reduced range of movement and crepitus in the knees.
What is the most likely diagnosis?
A. Rheumatoid arthritis
B. Osteoarthritis
C. Reactive arthritis
D. Polymyalgia rheumatica
E. Gout

A

B. Osteoarthritis

Osteoarthritis is the most common form of arthritis, most peple over 60 will have evidence of osteoarthritis on radiographs. Pain is characteristically worse after exercise or at the end of the day.

Over time the the hands become stiff and painful and painless bony swellings develop. Heberden’s nodes on the DIPs and Bouchard’s nodes on the PIPs

Bony swelling of the first
carpometacarpal joint may result in a squared hand appearance in nodal
osteoarthritis. Localized osteoarthritis may also affect the weight-bearing
joints of the hips and knees. Generalized osteoarthritis may include
features of nodal disease plus widespread joint involvement including
DIPs, first metatarsophalangeal joints, knees and hips. Increased pain in
the evenings, nodal disease and knee involvement point to osteoarthritis (B)
being the correct answer here.

Rheumatoid arthritis (A) would be worse in the morning and th DIPs are usually spared at the onset, making this not the case here.

Reactive arthritis (C) tends to occur after an infection such as an STI or enterobacteria. It does not affect the hands or cause nodal disease.

Polymyalgia rheumatica (D)
is wrong as this does not usually involve the hands or knees and is worse
in the mornings. Gout (E) normally presents as an acute monoarthritis,
making this answer incorrect.

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7
Q

A 79-year-old woman presents to her GP with pain in the left knee. This is
particularly bad in the evenings and is stopping her from sleeping. The GP explains
that her discomfort is most likely due to osteoarthritis and arranges for her to have
an x-ray of the knee. Which of the following descriptions are most likely to describe
the x-ray?
A. Reduced joint space, subchondral sclerosis, bone cysts and osteophytes
B. Increased joint space, subchondral sclerosis, bone cysts and osteophytes
C. Reduced joint space, soft tissue swelling and peri-articular osteopenia
D. Increased joint space, soft tissue swelling and peri-articular osteopenia
E. Normal x-ray

A

A. Reduced joint space, subchondral sclerosis, bone cysts and osteophytes

The Radiological changes seen in osteoarthritis can be remembered by the acronym ‘LOSS’

Loss of joint space

Osteophytes

Subchondral Cysts

Subchondral sclerosis.

In rheumatoid Arthritis we see ‘LESS’:

Loss of joint space

Erosions of the bone

Soft tissue swelling

Subluxation of the joint

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8
Q

A 76-year-old man presents to accident and emergency with pain in his knees.
It is worse in the right knee. He describes the pain as being worse in the evening
and after exertion. On examination, bony nodules are palpable on the distal
interphalangeal joints of both his hands. The right knee is swollen and there is a
reduced range of active movement. X-rays show reduction in the joint space,
subchondral sclerosis and osteophyte formation. What is the most appropriate
treatment?
A. Anti-TNF therapy
B. NSAIDs and urgent orthopaedic follow up
C. NSAIDs and GP follow up
D. NSAID and intramuscular depot injection of methylprednisolone with GP
follow up
E. Admit the patient for orthopaedic assessment

A

C. NSAIDs and GP follow up

This question is looking at the most appropriate management for a presentation of osteoarthritis.

You should follow a step wise approach to the management of osteoarthritis with conservative management such as weight loss, resting the joint, and custom orthotics being the first step. Following on from that the next stage in the management would be to offer simple analgesia and arange a follow up.

Anti-TNF therapy (A) is not used in the treatment of
osteoarthritis. Steroid therapy (D) is used as an joint injection in later treatment, as is intraarticular injections of hyluronic acid.

Orthopaedic follow up (B) to consider surgical options would
be appropriate if the pain is uncontrollable and particularly if the patient is
getting pain at rest or during the night. Admission (E) should be considered
in some patients where the pain is too severe for discharge or social
circumstances or co-morbidities mean that management of symptoms at
home will not be possible

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9
Q

A 32-year-old man presents to accident and emergency with a 1-day history of
pain in the right knee. He also mentions that he has had a fever and is feeling
generally unwell. On examination, the right knee is swollen, warm and extremely
painful to move. What is the most appropriate next step?
A. Empirical intravenous antibiotic treatment
B. X-rays of the right knee
C. Aspiration of the joint and blood cultures
D. Referral for physiotherapy
E. Immobilize the joint

A

C. Aspiration of the joint and blood cultures

This is a medical emergency, this is likely to be a septic arthritis. If this is not managed promptly it can lead to destruction of the joint. S.aureus is the most common causative organism, although gonococcus is also common in young people.

The systemic features and fever in this case combined with the appearance of a red, hot and swollen joint should make the diagnosis of a septic joint obvious.

The management here is to aspirate the joint and take cultures (C), following this the patient should be started on empirical antibiotic therapy as soon as possible. One possible treatment would be flucloxacillin, benzylpenicillin, and gentamicin.

Xray (B) should be performed, but is unlikely to change the management. Other blood tests including full blood count and CRP should also be sent. The joint should be initially immobilized (E), but this is not the most appropriate next step.
Physiotherapy (D) should be started early, but after an initial period of
immobilization, making this the wrong answer.

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10
Q

A 30-year-old man presents to his GP with a 1-week history of painful, swollen
knees and a painful right heel. Further history reveals that he has been experiencing
burning pains while urinating for the past 2 weeks and that his eyes have become
red and itchy. What is the most likely diagnosis?
A. Septic arthritis
B. Gout
C. Ankylosing spondylitis
D. Enteropathic arthritis
E. Reactive arthritis

A

E. Reactive arthritis

reactive arthritis (E) is a sterile arthritis that follows an infective process, such as dysentery (campylobacter, salmonella, shigella, yersinia) or urethritis (chlamydia or ureaplasma)

Clinically they will have an acute, asymetrical lower limb arthritis than comes 1-4 weeks after the infection.

Other features might be seen; conjunctivitis, enthesitis, circulate balinitis, keratoderma blenorrhagica (painless, red plaques on the soles and palmes), nail dystrophy, mouth ulcers and, rarely, aortic incompetence. The triad of urethritis, arthritis and conjunctivitis is known as Reiter’s disease.

Treatment of reactive arthritis is with NSAIDs and local steroid injection for symptomatic control. Any underlying infection should be treated but is unlikely to influence the course of the arthritis. Individuals who develop recurrent attacks of
arthritis can be considered for therapy with sulfasalazine or methotrexate.

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11
Q

A 70-year-old woman presents to accident and emergency with sudden onset pain
and swelling in the right knee. Her past medical history includes hypertension and
hypercholesterolaemia. She is currently taking aspirin, ramipril and simvastatin.
On examination, she is apyrexial and the right knee is swollen. There is reduced
range of movement in the knee due to swelling and pain. X-ray of the right knee
shows chondrocalcinosis. What is the most likely diagnosis?
A. Gout
B. Pseudo-gout
C. Septic arthritis
D. Reactive arthritis
E. Osteoarthritis

A

B. Pseudo-gout

Pseudo-gout (B) is caused by the presence of calcium pyrophosphate
crystals in the joint, causing an acute synovitis. Pseudo-gout most
commonly affects elderly women and usually involves the knee or wrists.
It may also be seen in younger patients with underlying conditions causing
the deposition of calcium pyrophosphate crystals such as hypothyroidism,
hyperparathyroidism, acromegaly, Wilson’s disease or haemochromatosis.
X-ray of the affected joint may show chondrocalcinosis (calcification of
the hyaline cartilage). Treatment of pseudo-gout is with aspiration of the
joint and NSAIDs. Intra-articular steroid injection can be used if pain is
not controlled. The acute synovitis of pseudogout resembles gout (A).
While acute gout most commonly affects the first metatarsophalangeal
joint, other joints may be affected. However, the finding of chondrocalcinosis
makes pseudo-gout more likely than gout. Septic arthritis is a differential
diagnosis of pseudo-gout and should be considered, despite the apyrexia.
Therefore, the joint aspirate should be sent for culture. The chondrocalcinosis
on x-ray makes the diagnosis of pseudo-gout more likely than septic
arthritis (C) in this question. Reactive arthritis (D) presents as an asymmetrical
polyarthritis of the lower limbs, making this answer incorrect. Osteoarthritis
(E) may affect the knee. However, the chondrocalcinosis again makes
pseudo-gout the more likely answer. In addition, a history of pain in the
knee would be expected.

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12
Q

A 74-year-old woman presents to accident and emergency with sudden onset pain
and swelling in the left knee. On examination, she is apyrexial and the left knee is
swollen. There is reduced range of movement in the knee due to swelling and pain.
X-ray of the right knee shows chondrocalcinosis. Microscopy of the fluid aspirated
from the joint is most likely to show:
A. Rhomboidal, weakly positively birefringent crystals under polarized light
microscopy
B. Needle-shaped negatively birefringent crystals under polarized light
microscopy
C. Atypical mononuclear cells
D. Reed Sternberg cells
E. Tophi

A

A. Rhomboidal, weakly positively birefringent crystals under polarized light
microscopy

The presence of rhomboidal, weakly positively birefringent crystals (A)
under polarized light microscopy in joint fluid is diagnostic of pseudogout.
Needle-shaped negatively birefringent crystals (B) are seen in gout.
Atypical mononuclear cells (C) are found on microscopy of blood samples
in patients with infectious mononucleosis.

Hodgkin’s lymphoma may show
Reed–Sternberg cells (D). Tophi (E) are the white deposits seen in skin and
soft tissue in some patients with gout. They are composed of sodium urate
and the presence of tophi in a patient with long-standing gout is called
‘chronic tophaceous gout’

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13
Q

A 23-year-old man presents to the rheumatology clinic with lower back and hip
pain. These have been occurring every day for the past two months. Pain and
stiffness are worse in the mornings. He also mentions that his right heel has been
hurting. He is previously fit and well, but had occasions of lower back pain when
he was a teenager. His symptoms have stopped him from playing tennis. Recent
blood tests organized by his GP have shown a raised C-reactive protein (CRP) and
erythrocyte sedimentation rate (ESR). What is the most appropriate treatment?
A. NSAID and spinal exercises
B. NSAID and bed rest
C. Oral prednisolone
D. Methotrexate plus sulfasalazine
E. Bed res

A

A. NSAID and spinal exercises

This is a difficult question as it requires knowledge of the diagnosis and
then knowledge of the treatment. The case presented is of a patient with
ankylosing spondylosis. The diagnosis is clinical, with involvement of
the sacroiliac joint as the earliest manifestation. The disease course is
variable and may progress to a marked kyphosis of the spine. Other
features include enthesitis (such as the Archilles tendon enthesitis in this
case), costochondritis, peripheral joint involvement (usually asymmetrical
and involving the large joints), aortic regurgitation, apical pulmonary
fibrosis and amyloidosis. The ESR and CRP are usually raised. Initial
x-rays may be unremarkable. However, later in the disease, syndesmophytes
(bony spurs due to inflammatory enthesitis) may be seen between vertebrae
resulting in the characteristic bamboo spine appearance. Ankylosing
spondylosis is managed with exercises, not bed rest. NSAIDs are given,
unless there are contraindications, for the management of pain. Therefore,
NSAID and spinal exercises (A) is the correct answer and NSAID and bed
rest (B) and bed rest (E) are incorrect. Local steroid injections may be used
for pain relief, particularly for peripheral arthritis and enthesitis. However,
oral prednisolone (C) is not normally used. Methotrexate and sulfasalazine
(D) may be given to patients with peripheral arthritis, but do not help the
back pain, making this answer incorrect

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14
Q

A 32-year-old man presents to the minor injuries walk-in clinic, complaining of
back pain. This had started suddenly that morning after he had lifted a heavy box
at home. He mentions that the pain has been shooting down his left leg and he
cannot walk without the support of his friend. He has not passed urine since the
onset of pain. On neurological examination of the lower limbs, tone and power
cannot be assessed due to pain but there are decreased ankle reflexes and a sacral
anaesthesia. What is the most appropriate next step?
A. Give NSAID analgesia and complete neurological examination
B. Send the patient home with NSAID analgesia and bed rest advice
C. Arrange urgent MRI of spine
D. Give NSAID analgesia and catheterize the patient
E. Send the patient home with NSAID analgesia and advice to avoid heavy
lifting

A

C. Arrange urgent MRI of spine

Saddle anaesthesia, back pain and urinary retention is an extremely alarming presentation and should make the clinician think of cauda equina syndrome. This patient needs an urgent MRI (C) and prompt neurosurgical management to avoid permanent neurological compromise.

A full neuro exam (A) and a catheter (D) is desirable but should not delay the MRI.

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15
Q

A 70-year-old woman with a history of vertebral crush fractures presents to the
osteoporosis outpatient clinic. Which of the following investigations is most useful
to assess the extent of her osteoporosis?
A. Spinal x-rays
B. MRI scan
C. Full blood count, bone and liver biochemistry blood tests
D. Vitamin D levels
E. DEXA scan

A

E. DEXA scan

Osteoporosis is a loss of the bone mass. It is important to note that the
mineralization of the bone is normal. This loss of bone mass means there
is an increasing likelihood of fracture with increasing age. Due to the
accelerated loss of bone mass following the menopause, elderly women
are at a higher risk of osteoporosis than men. There are numerous risk
factors for the development of osteoporosis. Among them are Caucasian
and Asian ethnic groups, female sex, increasing age, early menopause,
smoking, excess alcohol, corticosteroid use, hypogonadism and rheumatoid
arthritis. The reduced bone mass of osteoporosis may result in vertebral
crush fractures, the majority of which are asymptomatic. The extent of
osteoporosis is best investigated with a dual energy x-ray absorptiometry
(DEXA) scan (E). This gives a T score, which is the number of standard
deviations the patient’s bone mineral density differs from the population
average for a young healthy adult. The World Health Organization defines
osteoporosis as a T score of −2.5 or greater (i.e. the bone mineral density
of more than 2.5 standard deviations below that of the average for a
young healthy adult). A T score of between −1.5 and −2.5 is defined as
osteopenia.

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16
Q

A 20-year-old man presents to accident and emergency with sudden onset pain in
the right eye, with associated blurred vision and discomfort when gazing at the
lights. He has a history of back pain and has recently been diagnosed with
ankylosing spondylosis. What is the most likely cause of his eye pain?
A. Conjunctivitis
B. Retinal detachment
C. Anterior uveitis
D. Corneal ulceration
E. Acute glaucoma

A

C. Anterior uveitis

Anterior uveitis (C) is associated with ankylosing spondylosis and may
occur in up to one-third of patients. The symptoms described in this
patient with ankylosing spondylosis means anterior uveitis is the correct
answer. Conjunctivitis (A) is associated with ankylosing spondylosis but
would present with red, itchy eyes without blurred vision or photophobia,
making this answer incorrect. Retinal detachment (B) is not associated
with ankylosing spndylosis and usually presents with flashes of light in
the affected eye and increase in the number of floaters followed by visual
loss. Therefore, this answer is incorrect. Corneal ulceration (D) is not
associated with ankylosing spondylosis and presents with redness and eye
pain without visual loss, making it the wrong answer. Acute glaucoma (E)
may present with eye pain but is not associated with ankylosing
spondylosis, making this answer incorrect. Patients would present with a
red eye, sudden onset eye pain, decreased visual acuity and nausea or
vomiting.

17
Q

A 70-year-old woman presents to her GP complaining of severe unilateral headache
over the left side of her head. On further questioning, she mentions that she has
been having bilateral shoulder and neck pains over the past few weeks. She has also
been feeling lethargic. On examination, the left side of her scalp is painful to touch.
What is the most likely diagnosis?
A. Polyarteritis nodosa
B. Polymyositis
C. Hypothyroidism
D. Migraine
E. Giant cell arteritis

A

E. Giant cell arteritis

Giant cell arteritis (E) is a
large vessel vasulitis, which occurs in association with polymyalgia
rheumatica. Features of giant cell arteritis include severe headaches, which
are usually unilateral and involve the temporal region with associated
scalp tenderness. There may also be jaw claudication on eating. Involvement
of the ophthalmic arteries may result in visual loss, which may be
permanent if the condition is untreated. Polyarteritis nodosa (A) is a
medium-sized vessel vasculitis, which may affect a number of organs.
However, headache is not a feature, making this the incorrect answer.
Polymyositis (B) presents with proximal muscle weakness, resembling
polymyalgia rheumatica. Again, headache is not a feature making this
answer wrong. Hypothyroidism (C) can result in proximal muscle weakness
but does not cause unilateral headache, making this answer incorrect. The
presentation of migraine (D) is variable and includes unilateral headache,
with or without aura. However, the presence of scalp tenderness and
features of polymyalgia rheumatica make giant cell arteritis a more likely
option.

18
Q

A 77-year-old woman presents to accident and emergency complaining of severe
unilateral headache over the left side of her head. On examination, the left side of
her scalp is painful to touch. Blood tests reveal a raised ESR and CRP. What is the
most appropriate management?
A. Steroid therapy and arrange urgent temporal artery
biopsy
B. NSAID analgesia and arrange urgent temporal artery biopsy
C. Paracetamol analgesia and discharge with advice to bed rest
D. Arrange urgent MRI head
E. NSAID analgesia and arrange urgent electromyography

A

A. Steroid therapy and arrange urgent temporal artery
biopsy

19
Q

A 60-year-old woman presents to her GP with a two-month history of lethargy and
weakness. She mentions that she is finding it increasingly difficult to climb the
stairs and do the housework. On examination, there is wasting and weakness of the
proximal muscles in the upper and lower limbs. What is the most likely diagnosis?
A. Dermatomyositis
B. Polymyositis
C. Polymyalgia rheumatica
D. Kawasaki’s disease
E. Polyarteritis nodosa

A

B. Polymyositis

Polymyositis (B) occurs due to inflammation of striated muscle, resulting
in proximal muscle weakness. It affects women more than men. The onset
usually occurs over a period of months and may include systemic features
such as lethargy and weight loss. The proximal muscles of the shoulder
and pelvis girdle become weak and wasted. The disease may then progress to involve the pharyngeal, laryngeal or respiratory muscles. When features
of polymyositis are accompanied with overlying skin changes, it is termed
‘dermatomyositis’ (A). These include a heliotrope (lilac) rash over the
eyelids, scaly red papules over the knuckles, elbows or knees (Gottron’s
papules), a macular rash over the back and shoulders (shawl sign) or
painful cracking over the tips of the fingers (mechanic’s hands). The
absence of these features makes dermatomyositis the incorrect answer.

Polymyalgia rheumatica (C) may affect the proximal muscles. However, it
is associated with pain, which is an uncommon feature of polymyositis.
Although investigations have not been mentioned in this question, it is
worth noting that polymyalgia rheumatica is associated with a raised ESR
while in polymyositis, the ESR is usually not raised.

Kawasaki’s disease (D)
is a medium-vessel vasculitis that affects mainly children and does
not cause proximal muscle weakness, making this answer incorrect.

Polyarteritis nodosa (E) is also a medium vessel vasculitis, which affects
multiple organs. Proximal muscle weakness is not a feature, making this
answer incorrec

20
Q

A 30-year-old Afro-Carribean woman presents to accident and emergency with a
1-week history of progressive shortness of breath and fever. On further questioning,
she mentions that her hands have been painful and stiff over the past few months
and she has been having recurrent mouth ulcers. Chest x-ray confirms bilateral
pleural effusions and blood tests reveal a raised ESR and a normal CRP. What is the
most likely diagnosis?
A. Systemic lupus erythematosus
B. Systemic sclerosis
C. Sjögren’s syndrome
D. Discoid lupus
E. Beçhet’s disease

A

A. Systemic lupus erythematosus

SLE is a multisystem, inflammatory disorder which is nine times more
common in women than men. It is also more common in people of Afro-
Carribean origin and peak age of onset is usually 20–40 years of age.
Multiple genetic and aetiological factors have been associated with the
development of this disease. SLE has an extremely variable presentation,
with clinical features usually caused by underlying vasculitis. These
include polarthritis (the most common clinical feature, often a symmetrical
small joint polyarthritis similar to that seen in early RA), photosensitive
rashes (characteristic erythematous rash over malar eminences, sparing
the nasolabial folds), mouth ulcers, serositis (affecting pleura or
pericardium) or renal disease (usually nephrotic syndrome of renal failure
due to underlying glomerulonephritis). The features presented in this
case are, therefore, most suggestive of SLE (A) as the diagnosis. The raised
ESR and CRP should also identify SLE as the correct answer. Systemic
sclerosis (B) can be localized or diffuse and does not characteristically
cause mouth ulcers, pleural effusions or a raised ESR. Sjögren’s (C) is a
syndrome of dry eyes and dry mouth. It can be a primary syndrome or
occur secondary to other autoimmune diseases. While it is associated
with athralgia, the pleural effusion, mouth ulcers and blood results make
this diagnosis unikely. Discoid lupus (D) is a variant of SLE where skin
involvement is the only feature. Therefore, this is the incorrect answer.
Beçhet’s disease (E) usually manifests with oral ulceration. However, the
peripheral polyarthritis, pleural effusuions and blood results (in Beçhet’s
both ESR and CRP may be elevated) make this the incorrect answer in
this case.

21
Q

A 34-year-old Afro-Carribean woman has been admitted for management and
investigation of increasing shortness of breath. On further questioning, she
mentions that her hands have been painful and stiff over the past few months and
she has been having recurrent mouth ulcers. Chest x-ray confirms bilateral pleural
effusions and blood tests reveal a raised ESR and a normal CRP. A diagnosis of
systemic lupus erythematosus (SLE) is suspected and a full autoantibody screen is
sent to the laboratory. Which of the following auto-antibodies is most specific to
the suspected diagnosis?
A. Anti-nuclear antibody
B. Rheumatoid factor
C. Anti-double stranded DNA antibody
D. Anti-centromere antibody
E. Anti-mitochondrial antibody

A

C. Anti-double stranded DNA antibody

Anti-double stranded DNA antibody (C) is the most specific antibody for
SLE and is raised in 60 per cent of cases. Levels of other autoantibodies
may also be raised in SLE but these are less specific. They include antinuclear
antibody (A) and rheumatoid factor (B). Anti-centromere antibody
(D) levels may be raised in patients with limited systemic sclerosis but not
in patients with SLE. Raised anti-mitochondrial antibody (E) levels are
associated with primary biliary cirrhosis.

22
Q

A 55-year-old woman presents to her GP with shortness of breath and dry cough. The
symptoms began a few months ago and have progressed. She has a past medical
history of rheumatoid arthritis, diagnosed ten years earlier. On respiratory examination,there are bibasal fine inspiratory crackles on auscultation. What is the most likely cause of her symptoms?
A. Pulmonary oedema
B. Consolidation
C. Pleural effusions
D. Pulmonary fibrosis
E. Intrapulmonary nodules

A

D. Pulmonary fibrosis

The fine inspiratory crackles point towards pulmonary fibrosis, this makes sense given teh background of rheumatoid arthritis. Pulmonary fibrosis can be as a result of RA directly or as a result of treatment with drugs such as methotrexate.

23
Q

A 27-year-old woman presents to accident and emergency complaining of sudden
onset shortness of breath and right-sided pleuritic chest pain. She has a past medical
history of three miscarriages and a deep venous thrombosis in the right leg. On
examination, pulse is 110 bpm, respiratory rate is 24 bpm, oxygen saturation is 88
per cent on room air. An arterial blood gas shows pH 7.40, PO2 8.0, PCO2 3.1. What
is the diagnostic investigation of choice?
A. Full blood count
B. Chest x-ray
C. D-dimer
D. CT pulmonary angiogram (CTPA)
E. ECG

A

D. CT pulmonary angiogram (CTPA)

The clinical features outlined in this question of sudden onset shortness of
breath and pleuritic chest pain with tachycardia and tachypnoea with a
low oxygen saturation on room air, should raise the suspicion of pulmonary
embolism.

The diagnostic
investigation of choice in patients with pulmonary embolism is CTPA (D).
Full blood count (A) is not useful in the diagnosis of pulmonary embolism.
Chest x-ray (B) is often normal in patients with pulmonary embolism but
may show a wedge-shaped infarct. D-dimer (C) is a fibrin degradation
product that is commonly used in the diagnosis of deep venous thrombosis or pulmonary oedema. When negative, it indicates that venous thrombosis
is unlikely. D-dimer may be positive for a number of reasons. Therefore, it
is often used in patients where the clinical picture is unclear. In the case
outlined in the question, there should be a high degree of suspicion for
pulmonary embolism and a CTPA should be arranged without delay,
making D-dimer the incorrect answer. ECG (E) may be useful and may
commonly demonstrate sinus tachycardia. In addition, ECG might show
signs of right heart strain, such as right bundle branch block or right axis
deviation. Rarely, the S1Q3T3 pattern is seen (large S wave in lead I,
Q wave in lead III and inverted T wave in lead III). While ECG is useful, it
is not the diagnostic investigation of choice, making this the incorrect
answer.

24
Q

A 27-year-old woman presents to accident and emergency complaining of sudden
onset shortness of breath, right-sided pleuritic chest pain and haemoptysis. She has
a past medical history of three miscarriages and a deep venous thrombosis in the
right leg. CTPA confirms a large pulmonary embolism. What is the most likely
underlying diagnosis?
A. SLE
B. Primary anti-phospholipid syndrome
C. Raynaud’s disease
D. Systemic sclerosis
E. Beçhet’s disease

A

B. Primary anti-phospholipid syndrome

Recurrent venous or arterial thromosis with a history of miscarriages
should point to primary anti-phospholipid syndrome (B) as the correct
answer in this question. Anti-phospholipid syndrome is associated with
SLE (A) and a proportion of patients with SLE may develop secondary
anti-phospholipid syndrome. The absence of any clinical features of SLE
in this case means that this answer is the incorrect option.

25
Q

A 27-year-old woman presents to accident and emergency complaining of sudden
onset shortness of breath, right-sided pleuritic chest pain and haemoptysis. She has a
past medical history of three miscarriages and a deep venous thrombosis in the right
leg. CTPA confirms a large pulmonary embolism. A diagnosis of anti-phospholipid
syndrome is suspected and a full autoantibody screen is sent. Which of the following
auto-antibodies would confirm the diagnosis if detected?
A. Anti-cardiolipin antibody
B. Anti-centromere antibody
C. Anti-nuclear antibody
D. Anti-mitochondrial antibody
E. Anti-histone antibody

A

A. Anti-cardiolipin antibody

The presence of anti-cardiolipin antibodies (A) would confirm the diagnosis.
Both anti-cardiolipin antibody and lupus anticoagulant antibodies should
be sent. Raised levels of anti-centromere (B) antibodies are associated with
limited systemic sclerosis, not anti-phospholipid syndrome, making this
answer incorrect. Anti-nuclear antibody (C) levels may be raised in a
number of conditions including SLE, systemic sclerosis and rheumatoid
arthritis, but are usually negative in anti-phospholipid syndrome. Raised
anti-mitochondrial antibody (D) levels are associated with primary biliary
cirrhosis, but not anti-phospholipid syndrome, making this answer
incorrect. Similarly, raised anti-histone (E) antibody levels are associated
with drug-induced SLE, but not anti-phospholipid syndrome.

26
Q

A 45-year-old woman presents to the rheumatology clinic with a three-month
history of itchy, dry eyes and a persistently dry mouth. She also mentions that her
fingers have been extremely cold, occasionally turning blue after going outside in
the morning. Shirmer’s test is positive. What is the most likely diagnosis?
A. Systemic sclerosis
B. Raynaud’s disease
C. SLE
D. Primary Sjögren’s syndrome
E. Secondary Sjögren’s syndrome

A

D. Primary Sjögren’s syndrome

Primary Sjögren’s syndrome (D) occurs due to underlying fibrosis of exocrine
glands. Clinical features include decreased tear production, decreased
salivation and parotid gland swelling. There may be systemic disease, including arthritis, vasulitis or organ involvement (including pulmonary
disease, renal tubular involvement, thyroid disease, myaesthenia gravis,
primary biliary cirrhosis). Schirmer’s test uses filter paper under the lower
eyelid to measure tear production. Reduced tear production and salivation
are not features of systemic sclerosis (A), making this answer incorrect.
While Raynaud’s phenomenon is a systemic manifestation of Sjögren’s
syndrome, primary Raynaud’s disease (B) just affects the hands and is thus
the incorrect answer. Secondary Sjögren’s syndrome may occur in patients
with other connective tissue disorders including SLE or systemic sclerosis.
The absence of features suggestive of these other connective tissue disorders
means SLE (C) and secondary Sjögren’s syndrome (E) are the incorrect
answers.

27
Q

A 24-year-old woman presents to her GP complaining of cold hands and feet. This
has been ongoing for the past three months and is especially bad when she goes out
in the mornings and may last for hours. On further questioning, she mentions that
her hands sometimes turn blue or red and that gloves are unhelpful. She has
otherwise been feeling well and has no past medical history. What is the most
appropriate treatment?
A. Propanolol
B. Aspirin
C. Nifedipine
D. Subcutaneous injection of low molecular weight heparin
E. Prednisolone

A

C. Nifedipine

The case in this question is describing Raynaud’s syndrome. Vasospasm
results in peripheral digital ischaemia. Precipitating factors include
exposure to cold. Patients may describe their hands becoming pale, then
blue and then red. Raynaud’s disease is when Raynaud’s syndrome occurs
without an underlying cause, as in this case. However, Raynaud’s syndrome
is associated with a wide range of underlying connective tissue diseases. In
these cases, it is termed Raynaud’s phenomenon. Treatment of Raynaud’s
disease is with conservative measures such as warm gloves. In addition,
nifedipine (C) can be used, making this the correct answer. Propanolol (A)
is contraindicated in patients with Raynaud’s syndrome as it may worsen
the digital ischaemia. Aspirin (B), subcutaneous injection (D) and
prednisolone (E) are not treatments for Raynaud’s syndrome.

28
Q

A 42-year-old woman presents to accident and emergency with retrosternal
discomfort. She was diagnosed with systemic sclerosis a year ago. Which of the
following statements is true about systemic sclerosis?

A. Microstomia is only seen in diffuse cutaneous systemic sclerosis
B. Skin involvement is limited to face, hands and feet in limited cutaneous systemic sclerosis
C. Oesophageal dysmotility is only seen in limited cutaneous systemic sclerosis
D. Anti-double stranded DNA antibodies are normally detected in patients
with systemic sclerosis
E. Raynaud’s phenomenon occurs as a result of skin fibrosis (scleroderma)

A

B. Skin involvement is limited to face, hands and feet in limited cutaneous systemic sclerosis

Systemic sclerosis is a multisystem disease of unknown cause. It may be
limited to skin and soft tissue in limited cutaneous systemic sclerosis or
also involve the organs in diffuse cutaneous systemic sclerosis. Skin
involvement of the face may be seen in both forms and may produce the
characteristic beak-like nose and small mouth, termed microstomia (A).
In limited cutaneous systemic sclerosis, skin involvement is limited to the
hands, face and feet, making this the correct answer (B). In diffuse
cutaneous systemic sclerosis, skin across most of the body can be affected
in the worst cases. Oesophageal dysmotility (C) may occur in both forms
of the disease, making this option incorrect. Raised levels of anti-nuclear
antibody, anti-centromere antibody (limited cutaneous systemic sclerosis),
anti-Ro and anti-topoisomerase antibodies are associated with systemic
sclerosis. Anti-double stranded DNA antibodies (D) are normally
associated with SLE not systemic sclerosis, making this answer wrong.
Raynaud’s phenomenon (E) results from digital ischaemia, not skin
fibrosis as mentioned here.

29
Q

A 30-year-old woman presents to accident and emergency with worsening stiffness
in the hands, wrists and feet. She mentions that the pain has been particularly bad
in the mornings. On examination, there is a palpable spleen. Initial blood tests reveal
a low neutrophil count and a raised C-reactive protein. The most likely diagnosis is:
A. Felty’s syndrome
B. Reactive arthritis
C. Still’s disease
D. Infectious mononucleosis
E. Serum sickness

A

A. Felty’s syndrome

Felty’s syndrome (A) is splenomegaly and neutropenia in a patient with
rheumatoid arthritis. Reactive arthritis (B) is an asymmetrical lower limb
arthritis occurring 1–4 weeks following an infection. A range of other
clinical features may also be present (see question 8). Still’s disease (C) is
systemic juvenile idiopathic arthritis and is characterized by swinging
pyrexia, rash and arthritis. Juvenile idiopathic arthritis is the most common
form of persistent arthritis in those under 16 years of age. There is a
variable pattern of arthritis including oligoarthritis, polyarthritis and
systemic arthritis. Infection mononucleosis (D) may cause splenomegaly.
However, arthritis and neutropenia are not features of infectious
mononucleosis, making this answer incorrect. Serum sickness (E) is caused
by a hypersensitivity reaction to antibodies derived from animals. The
clinical features include fever, rashes, arthralgia, malaise, splenomegaly
and lymphadenopathy. Arthritis affecting and hands and feet and
neutropenia are not common features, making this option incorrect

30
Q

A 53-year-old man, who works as a chef, presents to accident and emergency with
sudden onset severe pain, tenderness and swelling of the first metatarsophalangeal
joint. The pain is making it difficult for him to mobilize. He has had two previous
similar episodes. Blood tests reveal a raised serum urate level. The most likely
diagnosis is:
A. Gout
B. Pseudo-gout
C. Septic arthritis
D. Reactive arthritis
E. Osteoarthritis

A

A. Gout

This case describes a typical presentation of acute gout (A). It is caused
by a raised plasma urate level and most commonly affects the first
metatarsophalangeal joint. Occassionally it is polyarticular. Acute
manifestations may be precipitated by a range of causes such as trauma,
surgery or the use of diuretics. Pseudo-gout (B) may present as a monoarthritis
but usually affects other joints such as the knee, wrist or hip. In
addition, the raised urate levels are not seen

31
Q

A 59-year-old man presents to his GP with sudden onset severe pain, tenderness
and swelling of the first metatarsophalangeal joint. He is known to suffer from
acute gout and has had several previous similar episodes. What is the most
appropriate treatment?
A. Allopurinol
B. NSAIDs
C. Conservative measures including reduced alcohol intake and weight loss
D. Intra-articular steroid injection
E. Methotrexate

A

B. NSAIDs

The most appropriate treatment of acute episodes is with a strong NSAID
(B) such as indomethacin. If NSAIDs are contraindicated, for example in
peptic ulcer disease, colchicine can be used. For patients with recurrent
attacks of gout, such as this case, serum urate should be reduced with
conservative measures and long-term allopurinol (A). Conservative
measures (C) include weight loss and avoiding excess alcohol, purine-rich
food and low-dose aspirin. However, the treatment of the acute episode is
the immediate priority here. Allopurinol may exacerbate an acute episode
and must only be started after the attack has resolved. Steroids (D) by oral,
intramuscular, intra-articular routes can be given in an acute episode when NSAIDs and colchicine are contraindicated, for example in renal
failure. Methotrexate (E) is a DMARD that is not used in the management
of gout.

32
Q

A 30-year-old Turkish man presents to accident and emergency with oral ulcers,
genital ulcers and painful legs. On examination, there are apthous ulcers in the
mouth, genital ulceration, erythema nodosum over the shins. He is admitted under
the medical team on call and a skin pathergy test is positive. What is the most likely
diagnosis?
A. Henoch–Schönlein purpura
B. Lyme disease
C. Berger’s disease
D. Caplan’s syndrome
E. Behçet’s disease

A

E. Behçet’s disease

Behçet’s disease (E) is a systemic vasculitis of unknown cause. Clinical
features of the disease include oral ulceration, genital ulceration, ocular
involvement (including anterior uveitis, posterior uveitis or retinal vascular
lesions), cutaneous lesions (including erythema nodosum or papulopustular
lesions), arthritis (mono- or oligo-arthritis), gastrointestinal features
(including diarrhoea and anorexia) and neurological features (including
encephalitis, confusion or cranial nerve palsies). A skin pathergy test is
when a needle prick leads to papule formation within 48 hours and is
specific to Behçet’s disease.

33
Q

A 23-year-old woman presents to accident and emergency with a purpuric rash
over the buttocks and lower limbs and haematuria. She is finding it difficult to
mobilize due to pain in her ankles and knees. What is the most likely diagnosis?
A. Henoch–Schönlein purpura
B. Perthes’ disease
C. Behçet’s disease
D. Still’s disease
E. Ehlers–Danlos syndrome

A

A. Henoch–Schönlein purpura

Henoch–Schönlein purpura (A) is small vessel vasculitis, which usually
occurs in children after an upper respiratory tract infection. Clinical
manifestations include a lower limb purpuric rash, arthritis (most commonly
involving the ankles, knees or elbows) and abdominal pain. Haematuria
may occur due to underlying glomerulonephritis. Perthes disease (B) is
characterized by avascular necrosis of the proximal femoral epiphysis. A
purpuric rash and haematuria are not features, making this answer incorrect.
Similarly, while oral and genital ulceration are features in Beçhet’s disease,
(C) purpuric rash and haematuria are not seen. Similarly, purpuric rash and
haematuria are not features of Still’s disease (D), making this answer
incorrect. Ehlers–Danlos syndrome (E) is a hypermobility syndrome caused
by a defect in collagen. The features outlined in this case are not seen in
Ehlers–Danlos, making this the incorrect answer

34
Q

A 67-year-old man presents to his GP with pain in his pelvis. During the consultation,
he mentions that his friends have been commenting that his head appears larger
than before. In addition, he has noticed deterioration in hearing in his left ear. On
neurological examination, a left-sided sensorineural deafness in detected. Closer
inspection of the legs reveals bowing of the tibia. What is the most likely diagnosis?
A. Osteomalacia
B. Osteoporosis
C. Acromegaly
D. Ricketts
E. Paget’s disease

A

E. Paget’s disease

Paget’s disease (E) is a disorder of bone remodelling, in which the constant
resorption and formation of bone can lead to deformity. Sites that are
typically affected include the skull, spine, pelvis, femur and tibia. Typical
deformities include skull changes and bowed tibia.

The remodelling of bone can lead to nerve compression. Nerves affected may include cranial nerve VIII, resulting in sensorineural deafness. This is a frequently
examined point! It is important to note that other cranial nerves may be
affected. In addition, Paget’s disease may often be asymptomatic or present
with bone pain (typically of the spine or pelvis) or joint pain. X-rays of the
bones involved in Paget’s disease may show lytic lesions.

35
Q

In a patient with Paget’s disease of the bone, which of the following blood test
results are most likely to be seen?
A. Normal serum calcium, normal serum phosphate, raised serum alkaline
phosphatase
B. Normal serum calcium, normal serum phosphate, normal serum alkaline
phosphatase
C. Raised serum calcium, low serum phosphate, normal serum alkaline
phosphatase
D. Normal serum calcium, low serum phosphate, raised serum alkaline
phosphatase
E. Low serum calcium, low serum phosphate, low serum alkaline
phosphatase

A

A. Normal serum calcium, normal serum phosphate, raised serum alkaline
phosphatase

The increased bone turnover in Paget’s disease is reflected by a normal
serum calcium and phosphate and a raised alkaline phosphatase (A). The
alkaline phosphatase in Paget’s may be markedly raised at over 1000 U/L.
Normal serum calcium, phosphate and alkaline phosphatase (B) may be
seen in the absence of disease. It is important to note that normal blood
tests are also seen in osteoporosis. Raised serum calcium, low serum
phosphate and normal serum alkaline phosphatase (C) are seen in patients
with primary hyperparathyroidism. Normal serum calcium, low serum
phosphate and raised serum alkaline phosphatase (D) may be seen in
hyperparathyroidism secondary to osteomalacia. The raised alkaline
phosphatase is indicative of osteoblast activity causing bone resorption.
Meanwhile, the raised levels of parathyroid hormone may increase serum
calcium levels to normal and leave a low serum phosphate level. A low
serum calcium, low serum phosphate and low serum alkaline phosphatase
(E) is not characteristic of Paget’s.