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					                   MCQs in Rheumatology:

                 Connective tissue diseases




Contributors: These MCQs were written by Dr Pravin Patil, Dr Kar-Ping Kuet, Dr

Frances Rees, Dr Michelle Hui, and Dr Dipti Patel; and were reviewed by Dr Neal

Hopkinson, Dr Marian Regan, Dr Adrian Pendleton, and Prof. Nigel Arden. The

MCQs were edited by Dr A Abhishek who also facilitated the review process.




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Question 1

You have been asked to see a 65 year old lady who presented with a 1 year history
of diarrhoea. She reports abdominal pain and bloating. Her appetite is normal. She
denies any rectal bleeding, dysphagia, cough, fever or foreign travel. Her past
medical history includes diffuse systemic sclerosis, Raynaud’s phenomenon and
gastroeosphagial reflux disease. She is on Ranitidine 150mg BD and Nifedipine
10mg TDS. Examination of chest, heart and abdomen is unremarkable. Abdominal
X-ray, stool MC&S, routine blood tests including full blood count, serum amylase and
inflammatory markers came were normal. CT abdomen and pelvis, and flexible
sigmoidoscopy were normal.

What should be the next step in the management of her diarrhoea?

        1.   Cyclical antibiotics
        2.   Mebeverine
        3.   Metoclopramide
        4.   Regular anti-diarrhoeal agents
        5.   Stop ranitidine

Question 2

A 35 year old man is referred to you by the on-call medical registrar. This previously
well gentleman presented to accident and emergency department with intermittent
fevers, rash and arthralgia. The medical team suspects a diagnosis of adult-onset
Still's disease.

Which one of the following does not form part of the Yamaguchi classification criteria
for adult-onset Still's disease?

   1.   Arthralgias >2 week
   2.   High ferritin level
   3.   Neutrophil leukocytosis
   4.   Temperature of >39°C for >1 week
   5.   Salmon-pink, maculopapular, evanescent, pruritic rash




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Question 3

You see a 53 year old man in a follow up rheumatology clinic. He has limited
cutaneous systemic sclerosis diagnosed 12 years ago. You notice elevated blood
pressure, and are concerned regarding the possibility of scleroderma renal crisis.

Which one of the following is a risk factor for scleroderma renal crisis?

       1.   Early limited cutaneous systemic sclerosis
       2.   Female gender
       3.   Corticosteroid therapy
       4.   Presence of anti-GBM antibodies
       5.   Slowly progressive skin involvement

Question 4

A 25-year old female with Behcet's disease, presents with erythema nodosum, and
oral ulcers that are unresponsive to treatment with corticosteroid mouth washes, and
corticosteroid paste.

What would be the next most appropriate drug treatment?

            1.   Azathioprine
            2.   Colchicine
            3.   Hydroxychloroquine
            4.   Interferon –α
            5.   Thalidomide

Question 5

A previously fit and healthy 37 year old man presents to the medical assessment unit
with haemoptysis. His only other past medical history is a DVT diagnosed 1 month
ago, and he is on warfarin. He has a history of oral ulcers, and had one episode of
genital ulcers. INR is 2.9, all investigations are still pending.

What is the most likely diagnosis?

       1.   Behcet’s disease
       2.   Goodpastures’ syndrome
       3.   Massive pulmonary embolism
       4.   Over anticoagulation
       5.   SLE with pulmonary haemorrhage




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Question 6

A 59 year old lady attends her routine follow up appointment in the rheumatology
clinic. She was diagnosed with diffuse systemic sclerosis 5 years ago. She also has
hypertension. Her medications include omeprazole 20mg od, nifedipine 10mg bd,
and ramipril 5mg od. She has severe Raynaud’s and on questioning has developed
digital ulcers on several occasions. On examination, her BP is 110/70 mm/Hg, there
is sclerodactyly and nail fold vasculopathy. You notice several healing digital ulcers
which do not look infected and atrophy of the finger pulp.

Which of the following options would be the best management of her digital ulcers?

   1.   Digital sympathectomy
   2.   Fluoxetine
   3.   I.V iloprost
   4.   Sildenafil
   5.   Thermal gloves

Question 7

A 28 year old previously healthy lady presents to the rheumatology clinic with a 6
month history of Raynaud’s phenomenon and progressive shortness of breath. She
has developed a few digital ulcers. Her hands are puffy and the skin feels tight. She
denies sicca symptoms.

On examination she has puffy hands with nail fold vasculopathy, fine bibasal
crackles, loud second heart sound, and a pansystolic murmur. She had 4/5 proximal
weakness of her upper and lower limbs. She is ANA, RNP positive. FBC, UE and
LFTs are normal. Her CRP is elevated at 17 mg/l. Her CK is 600 iu/l. Chest X-ray
reveals interstitial changes.

Which of the following is the most likely diagnosis?

           1.   Dermatomyositis
           2.   Diffuse cutaneous systemic sclerosis.
           3.   MCTD
           4.   Sjogren’s syndrome
           5.   SLE




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Question 8

A 45 year old female with recent onset Raynaud’s phenomenon, is referred to the
gastroenterologists with difficultly swallowing and anaemia. On endoscopy she is
found to have gastric antral vascular ectasia (watermelon stomach).

Which of the following antibodies is most likely to be present?

   1.   Anti-centromere antibody
   2.   Anti- dsDNA antibody
   3.   Anti-histone antibody
   4.   Anti-Ro antibody
   5.   Anti-Scl70 antibody

Question 9

A 30 year old woman presents with a 2 year history of recurrent oral ulcers and
arthralgia. Over the past 2 weeks she has developed genital ulcers. She was seen in
the GUM clinic, and sexually transmitted disease was excluded. She has a past
history of irritable bowel syndrome. On examination, there are two large aphthous
ulcers in her mouth, several areas of scarring, and a nodular rash on the shins.

What is the most likely diagnosis?

   1.   Behcet’s disease
   2.   Crohn’s disease
   3.   Reactive arthritis
   4.   Sarcoidosis
   5.   Ulcerative colitis

Question 10

A 46 year old female attends the rheumatology clinic with dry gritty eyes, dry mouth
and occasional oral ulcers. She is lethargic and her legs feel numb. Occasionally
her parotids have been swollen. She is also a smoker of 30 pack years.
Examination reveals dry mucous membranes and a peripheral sensory loss.
Investigations show normal FBC and renal function, ESR 77 mg/l, CRP 6mg/l, and a
polyclonal increase in immunoglobulins. Urine dipstick is normal and chest X-ray is
unremarkable. ANA, ENA and RF are negative.

Which of the following would be the most definitive investigation to establish the
diagnosis?

   1.   Labial gland biopsy
   2.   Nerve conduction studies
   3.   Parotid gland biopsy
   4.   Schirmer’s test
   5.   Ultrasound of parotid gland




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Question 11

A 31 year old female with primary Sjogren’s syndrome on hydroxychloroquine
attends rheumatology clinic for a follow-up appointment. She wants to start a family,
and has a few questions regarding this. In particular she wishes to know whether
there is any way in which her condition can affect the pregnancy adversely. She is
anti-Ro, and anti-La positive, and her disease is well controlled.

Which of the following would be the most appropriate advice for her?

1.   Pregnancy is contraindicated
2.   The fetus is not at risk of congenial heart block.
3.   The fetus has a 0.5-1% risk of congenital heart block
4.   The fetus has a 5-15% risk of congenital heart block
5.   The fetus has a 50% risk of congenital heart block

Question 12

A 53 year old lady presented to the medical assessment unit. She has been unwell
for 6 months with arthralgia, fevers, weight loss and has more recently developed
pleuritic chest pain. On examination, she was febrile at 38.1 oC. The vital signs were
as follows: BP 110/70 mmHg, RR 18/minute, SaO2 97% on air. She had synovitis of
small joints of hands. There were no rashes or nail fold infarcts. Heart sounds were
normal. A CTPA done to rule out pulmonary embolism showed a small left pleural
effusion. There was no consolidation, pulmonary embolus and no lymphadenopathy.
ECG showed sinus tachycardia.
A summary of the investigations (with normal values in brackets) is as follows
Hb 8.9 g/dl(11-14.7)
WCC 3.2 x10/l(3.5- 9.5)
Plts 99 x10/l(140-370)
Neut 2.0 x10/l(1.7-6.5)
Lymph 0.9 x10/l(1.0-3.0)
MCV 78.9 fl(80-98.1)
CRP 99 mg/l (<6)
ESR 56 mm/hr (<10)
Urine dipstick showed a trace of protein.
U&E and LFTs normal
Clotting normal
Ferritin 600 microgm/l (35-140)
Serial blood cultures (x 3) are negative at 48 hours.
ANA positive (1:1600), ANCA screen, ENA antibodies, and anti-CCP antibodies
were negative.

Which of the following is the most likely diagnosis?

        1.   Adult-onset Still’s disease
        2.   Infective endocarditis
        3.   Lymphoma
        4.   Rheumatoid arthritis with vasculitis
        5.   Systemic lupus erythematosus


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Question 13

A 63 year old lady presents to the medical assessment unit with a flu-like illness and
myalgia. In the last few days she has noticed a rash across her back. She has no
other symptoms.
On examination she looks unwell. There is an erythematous rash across her back.
She has grade 4/5 weakness proximally. You check her CK which is elevated at
1052 IU/l. Her renal function is normal. You suspect she has dermatomyositis.

Which of the following investigations is most likely to help establish the diagnosis?

           1.   EMG
           2.   ENA screen
           3.   MRI quadriceps
           4.   Muscle biopsy
           5.   Skin biopsy

Question 14

A 35 year old lady with limited cutaneous systemic sclerosis attends the
rheumatology clinic and complains of shortness of breath on exertion, for last 2-3
months. On examination, the chest is clear with normal heart sounds. Pulmonary
function tests done 2 weeks before clinic revealed FVC 80% predicted, FEV1 81%
predicted, TLCO 40% predicted.

Which one of the following is the most definitive investigation for reaching a
diagnosis in this patient?

        1. HRCT chest
        2. Right heart catheter study
        3. Six minute walk test
        4. Trans oesophageal echocardiogram
        5. Trans thoracic echocardiogram

Question 15

A 60 year old gentleman is referred by his general practitioner with bilateral external
ear pain, inability to sleep on either side and arthralgia. You suspect a diagnosis of
relapsing polychondritis.

Which one of the following type of joint involvement is typically seen in relapsing
polychondritis?

   1.   Erosive symmetric polyarthritis
   2.   Non-erosive symmetric polyarthritis
   3.   Erosive asymmetric polyarthritis
   4.   Non-erosive asymmetric polyarthritis
   5.   Proliferative monoarthritis




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Question 16

A 26 yr old Afro Caribbean married teacher with SLE presents with one week history
of worsening joint symptoms, mouth ulcers, fatigue, and pleurisy. Laboratory
investigations indicate active SLE, with worsening renal function, anemia,
leucopenia, raised anti double stranded DNA, and low complement levels. A decision
is made to treat with rituximab.

Which of the following vaccination should be prioritised prior to treatment with
rituximab?

           1.   Tetanus vaccine
           2.   Pneumococcal vaccine
           3.   Diphtheria vaccine
           4.   BCG vaccine
           5.   Hepatitis B vaccine

Question 17

A 70-year-old patient presented with a one-year history of progressive skin
tightening. He denies symptoms of Raynauds, shortness of breath, dysphagia, and
any gastro-intestinal reflux symptoms. On examination he has widespread
erythematous, indurated skin with diffuse tightness involving face, fingers,
extremities and trunk.
Hb- 14 g/dl, WBC 8 x 10^9/l, Neutrophils- 6 x 10^9/l, Lymphocytes 1.0 x 10^9/l,
eosinophils 0.3 x 10^9/l, platelet 350 x 10^9/l.
ANA, Anti- centromere, Scl-70 negative. Thyroid function test- Normal.
Serum electrophoresis- monoclonal gammopathy of the IgG type, with lambda light
chains. Skin biopsy shows mucin deposition.

What is the diagnosis?

      1.   Eosinophilic fasciitis
      2.   Lichen sclerosus
      3.   Nephrogenic systemic fibrosis
      4.   POEMS syndrome
      5.   Scleromyxoedema




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Question 18

A 23 year old girl with SLE (previous lupus nephritis, currently in remission), and
APAS requests an urgent appointment as she is pregnant. Her past medical history
includes epilepsy, stroke, and TIA. On examination, she has a photosensitive malar
rash, patchy alopecia, and puffy MCPJs, and wrists. Current medications include
Mycophenolate 2gm od, Aspirin 75mg od, Prednisolone 2.5mg/day, Dalteparin
18,000IU (treatment dose calculated for body weight 84kg) per day, and
Hydroxychloroquine 400 mg od.

What will you advice regarding her medications in view of the pregnancy?

       1.   Stop mycophenolate
       2.   Stop aspirin
       3.   Switch mycophenolate to azathioprine
       4.   Switch mycophenolate to ciclosporin
       5.   Switch mycophenolate to rituximab

Question 19

A 20 year old woman is referred from the medical admissions unit. She has a 4 week
history of spiking fevers and has now developed a symmetrical polyarthritis. Her
mother tells you she was treated for arthritis of her knees when she was a child. On
examination you notice she has a pink macular rash.

What is the most likely diagnosis?

1. Adult-onset Still’s disease
2. Familial Mediterranean Fever (FMF)
3. Muckle-Wells syndrome
4. Systemic onset Juvenile Idiopathic arthritis
5. TNF receptor-associated periodic syndrome (TRAPS)




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Answers:

Q1.                  1. Cyclical antibiotics

Gastro-intestinal involvement is common in systemic sclerosis. This lady has
symptoms of bacterial overgrowth secondary to small bowel dysmotility. Diagnosis
can be established by hydrogen breath test. Treatment involves cyclical use broad-
spectrum antibiotics. Futher investigations like CT or colonoscopy are not indicated.
There no indicators for irritable bowel syndrome.

Q2.                  2. High ferritin levels

Serum ferritin does not form part of the Yamaguchi criteria for classification of adult-
onset Still's disease. Patients can be regarded as having AOSD by this classification
criteria if there is presence of 5 or more criteria, of which at least 2 are major (96%
sensitivity; 92% specificity)
Major Criteria -
     Temperature of >39°C for >1 wk
     Leukocytosis >10,000/mm3 with >80% PMNs
     Typical rash
     Arthralgias >2 wk
Minor Criteria
     Sore throat
     Lymph node enlargement
     Splenomegaly
     Liver dysfunction (high AST/ALT)
     Negative ANA, RF

Q3.                  3. Corticosteroid therapy

An estimated 65-86% of scleroderma renal crisis (SRC) occurs in patients with
diffuse SSc, and patients with early dcSSc are at greatest risk. Rapidly progressive
skin disease (found in the early phase of dcSSc) represents another risk factor; the
estimated median duration of SSc at SRC diagnosis is 8 months. An estimated 66%
of patients with SSc develop SRC within a year of diagnosis, rising to 86% at 4
years. Patients with limited SSc typically develop SRC later in SSc disease course,
and sex appears not to influence the risk. Corticosteroid therapy increases the risk of
SRC.
Penn H, Howie AJ, Kingdon EJ, et al. Scleroderma renal crisis patient characteristics
and long-term outcomes. QJM 2007; 100485-494. A large retrospective analysis of
110 SRC cases treated in the era of ACEI taken from a cohort of SSc, this study
shows risk factors, outcomes, and clinicopathological correlates.

Q4.                  2. Colchicine

Colchicine is the most appropriate next choice, particularly in the presence of
erythema nodosum. Thalidomide has also been recommended for severe mouth
ulcers. However, it needs appropriate counselling in someone of child-bearing age
due to a high risk of teratogenicity. Azathioprine, ciclosporin (except where there is
neurological involvement), Interferon –α and TNF- α antagonists can also be


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considered in resistant cases (Hatemi G et al. EULAR recommendations for the
management of Behçet disease, Ann Rheum Dis 2008;671656–1662).

Q5.                  1. Behcet’s disease

This gentleman has Behcet’s disease. He has a history of oral genital ulcers and
DVT and has now presented with haemoptysis probably as a result of vasculitis, and
arterio-venous malformations.

Q6.                  3. I.V iloprost

Initial management includes keeping core body temperature warm and wearing
glove warmers. Pharmacological agents include calcium channel blockers including
nifedipine and amlodipine. Fluoxetine has some vasodilator effects. For patients
with severe Raynaud’s developing digital ulceration not responding to conventional
treatment, i.v iloprost is an effective treatment option. Oral Sildenafil is also an
alternative effective treatment option in those who do not respond to i.v iloprost.

Q7.                  3. MCTD

This lady has scleroderma, myositis and features of SLE. This would suggest MCTD
since she is also RNP positive.


Q8.                  5. Anti-Scl70

Gastric antral vascular ectasia (watermelon stomach), although not specific to
systemic sclerosis, is a characteristic finding in diffuse cutaneous systemic sclerosis.
As anti-Scl70 is the most common autoantibody found in diffuse cutaneous systemic
sclerosis, it is most likely to be present. However, GAVE can also be present in
Sjogren’s syndrome.

Q9.                  1. Behcet’s disease

This patient has Behcet’s disease. The ISSG diagnostic criteria for Behcet’s disease
is as follows

Recurrent aphthous mouth ulcers plus any 2 of the following
    Genital ulcers
    Skin involvement (acne or erythema nodosa)
    Pathergy (skin reaction to pin prick)
    Eye involvement (conjunctivitis or uveitis)

Q10.                 1. Labial gland biopsy

This lady’s clinical presentation is consistent with Sjogren’s syndrome. Some
patients with Sjogren’s syndrome are ENA negative. Schirmer’s test would be
helpful in demonstrating reduced tear production but the most definitive investigation
to establish the diagnosis would be a labial gland biopsy.



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Q11.                 4. The fetus has a 5-15% risk of congenital heart block

If the mother is anti-Ro/La positive, there is a five percent risk of the fetus developing
congenital heart block. The fetus must be monitored carefully for this complication
throughout pregnancy. If the fetus develops congenital heart block this will require a
pace maker. Hydroxychloroquine is safe during pregnancy.

Q12.                 5. SLE

This lady has SLE with pleurisy. Pleurisy is present in up to 60% of patients with
SLE. Pericardial disease is the most common cardiac involvement in SLE. A
pericardial effusion which evolves quickly can be life threatening. She has arthritis,
serositis, positive ANA, and lymphopaenia, and therefore meets the classification
criteria for SLE. She is unlikely to have infective endocarditis in the presence of
normal echocardiogram, and three sets of negative blood cultures. Serum ferritin is
an acute phase reactant, and in AOSD it is extremely elevated (in range of several
thousands). Similarly, rheumatoid vasculitis is unlikely in someone who is anti-CCP
antibody and rheumatoid factor negative

Q13.                 4. Muscle biopsy

This lady has dermatomyositis. The most helpful investigation here would be EMG
and muscle biopsy to confirm the diagnosis. Whilst immunology is part of the
investigations if negative this does not exclude the diagnosis. MRI of the quadriceps
can identify muscle oedema and aid site for muscle biopsy. In some units, a muscle
biopsy may be performed without an EMG.

Q14.                 2. Right heart catheter study

Isolated drop in TLCO with preserved lung volumes suggests diagnosis of pulmonary
hypertension. Gold standard investigation for diagnosis of pulmonary hypertension is
right heart catheter study. Echocardiograms are only screening tests and may yield
false positives or negatives. Six minute walk test is used in monitoring progress but
does not have diagnostic value.

Q15.                 4. Non-erosive asymmetric polyarthritis

Arthritis is the second most common presenting symptom of relapsing polychondritis
and it eventually affects more than 70% of patients. The arthritis of pure relapsing
polychondritis is intermittent, migratory, asymmetric, seronegative, and usually non-
erosive.

Q16.                 2. Pneumococcal vaccine


B cells play an important role in host defense in pneumococcal infections. Moreover,
in context of active SLE with low complements, leucopenia, pneumococcal
vaccination should be considered. Influenza vaccine is recommended in for all
patients prescribed immunosuppressive agents. Prospective studies have shown
that immunisation with inactivated/killed vaccines is probably safe in patients with



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SLE. Individuals on immunosuppressive agents and/or high dose steroid (more than
20 mg/d) should not receive live vaccines, as stated in guidelines proposed by the
British Society of Rheumatology. The safety of hepatitis B vaccination in patients
with SLE is as yet undetermined and a prospective study is required. However, until
such evidence becomes available the advice of the BSR should be followed and
patients with SLE who are at risk of exposure to hepatitis B should be vaccinated.
Q17.                 5.Scleromyxoedema
Scleromyxedema is characterized by the presence of infiltrative and confluent skin
induration due to an increased deposition of glycosaminoglycans within the dermis.
When the lesions are diffuse, scleromyxedema can clinically mimic SSc. Skin biopsy
shows a diffuse mucin depositions. Nephrogenic systemic fibrosis is a progressive
fibrotic disorder occurring in patients with end-stage renal disease on exposure to
gadolinium-containing contrast agents. Eosinophilic fasciitis is characterized by the
inflammation and sclerosis of the fascia and the subcutaneous tissue of the limbs
with sparing of the hands, feet, and the face. POEMS syndrome is a rare
multisystemic disease occurring in the setting of a plasma cell dyscrasia which is
characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal
gammopathy, and skin changes. Lichen sclerosus is a chronic lymphocyte mediated
inflammation of the skin which most commonly affects the ano-genital epithelium in
women. Scleroderma-like disorders Autoimmunity Reviews Volume 7, Issue 4,
February 2008, Pages 331-339.

Q18.                 3.Switch mycophenolate to azathioprine
Mycophelolate is not safe during pregnancy, and should be discontinued. Similarly,
ciclosporin, and rituximab are contraindicated during pregnancy. As she has ongoing
active SLE she requires immunosuppression. Azathioprine is the safest choice
among the options. Azathioprine is contra-indicated in pregnancy – except when
clinically indicated. This scenario would seem to permit the use of Azathioprine after
careful discussion of risks and benefits. Guidelines from the American College of
Obstetricians and Gynecologists regarding prenatal and postpartum care for women
with APS recommend prophylaxis for those with no history of thrombosis and full
anticoagulation for those with a history of thrombosis. Patients with pregnancy loss
receive prophylactic low–molecular-weight heparin and low-dose aspirin. Therapy is
withheld at the time of delivery and is restarted after delivery, continuing for 6-12
weeks postpartum. Patients with a history of thrombosis receive therapeutic doses of
heparin during pregnancy; long-term anticoagulation is then continued postpartum.

Q19.                 1.Adult-onset Still’s disease
Adult-onset Still’s disease is a multi-organ inflammatory condition. It usually presents
with a spiking quotidian fever, symmetrical polyarthritis and a transient salmon pink
macular rash which occurs with the fever. FMF is one of the periodic fever
syndromes. It more commonly presents in young males of Mediterranean origin.
Muckle-Wells is a rare autosomal dominant disease which causes periodic fevers,
arthritis, recurrent urticaria and ultimately sensorineural deafness. It usually first
presents in childhood. Deafness becomes apparent in the teenage years. Systemic
onset Juvenile Idiopathic arthritis presents similarly to this case, but in children.
TRAPS is another rare inherited periodic fever syndrome characterised by most


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commonly by recurrent fever, abdominal pain, myalgia, and arthralgia, followed by
tender erythematous skin lesion, chest pain, conjunctivitis, periorbital oedema,
testicular pain, headaches, lymphadenopathy and amyloidosis.




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