Passmedicine Flashcards
Which of the following features are not typically seen in a patient with adult onset Still’s disease?
A. Maculopapular rash B. Rheumatoid factor C. Pyrexia D. High ferritin level E. Lymphadenopathy
B RF
Adult-onset Still’s disease is a diagnosis of exclusion and can only be diagnosed if rheumatoid factor and anti-nuclear antibody are negative
Features arthralgia elevated serum ferritin rash: salmon-pink, maculopapular pyrexia typically rises in the late afternoon/early evening in a daily pattern and accompanies a worsening of joint symptoms and rash lymphadenopathy rheumatoid factor (RF) and anti-nuclear antibody (ANA) negative
The diagnosis of Still’s disease in adults can be challenging. The Yamaguchi criteria is the most widely used criteria and has a sensitivity of 93.5%.
Management
NSAIDs
should be used first-line to manage fever, joint pain and serositis
they should be trialled for at least a week before steroids are added.
steroids
may control symptoms but won’t improve prognosis
if symptoms persist, the use of methotrexate, IL-1 or anti-TNF therapy can be considered
Which one of the following is least associated with the development of gout?
A. Psoriasis B. Lesch-Nyhan syndrome C. Lymphoma D. Lithium toxicity E. Renal failure
A psoriasis
An 28-year-old man is investigated for recurrent lower back pain. A diagnosis of ankylosing spondylitis is suspected. Which one of the following investigations is most useful?
A. ESR B. X-ray of the sacro-iliac joints C. HLA-B27 testing D. X-ray of the thoracic spine E. CT of the lumbar spine
B. X-ray of the sacro-iliac joints
Inflammatory markers (ESR, CRP) are typically raised although normal levels do not exclude ankylosing spondylitis.
HLA-B27 is of little use in making the diagnosis as it is positive in:
90% of patients with ankylosing spondylitis
10% of normal patients
Plain x-ray of the sacroiliac joints is the most useful investigation in establishing the diagnosis. Radiographs may be normal early in disease, later changes include:
sacroiliitis: subchondral erosions, sclerosis
squaring of lumbar vertebrae
‘bamboo spine’ (late & uncommon)
syndesmophytes: due to ossification of outer fibers of annulus fibrosus
chest x-ray: apical fibrosis
If the x-ray is negative for sacroiliac joint involvement in ankylosing spondylitis but suspicion for AS remains high, the next step in the evaluation should be obtaining an MRI. Signs of early inflammation involving sacroiliac joints (bone marrow oedema) confirm the diagnosis of AS and prompt further treatment.
Spirometry may show a restrictive defect due to a combination of pulmonary fibrosis, kyphosis and ankylosis of the costovertebral joints.
You see a 70-year-old male patient with back pain. He says he has had lower back pain for about 12 months which is slowly getting worse. It now radiates to his buttocks, thighs and legs bilaterally (but his left leg is worse than the right). He describes the pain as ‘cramping’ and ‘burning’. He says that when he walks for more than a few minutes his legs become weak and numb. If he sits down and leans forward the symptoms go. He finds standing exacerbates the symptoms. He is worried as he is losing his independence as he does not feel stable on his feet, he now has a walking aid. His wife thinks he has a more stooped posture than 12 months ago.
His past medical history includes tablet controlled hypertension. He has never smoked and has a normal BMI.
On examination, he has a wide-based gate. Neurological examination of his lower limbs is normal. His peripheral pulses feel normal.
What diagnosis is most likely given the presentation and examination findings?
A. Ankylosing spondylitis B. Spinal stenosis C. Sciatica D. Vascular claudication E. Non-specific lower back pain
B. Spinal stenosis
Spinal stenosis is the most likely diagnosis in a patient with gradual onset leg and back pain, weakness and numbness which is brought on by walking (with a normal clinical examination)
This man’s presentation is most consistent with a diagnosis of spinal stenosis. Spinal stenosis typically presents with gradual onset leg and back pain, weakness and numbness which is brought on by walking. Patients often say that the pain is relieved by sitting and leaning forward and is better if walking up a hill. Physical examination findings are frequently normal in patients with lumbar spinal stenosis. Therefore, the correct answer is 2.
The main differential diagnosis is vascular claudication but he has normal pulses peripherally. Therefore, option 4 is wrong.
Sciatica is more likely to present with unilateral leg pain. Therefore, option 3 is incorrect.
Non-specific lower back pain is common but does not present with lower limb symptoms. Therefore, option 5 is wrong.
Which one of the following statements regarding raloxifene in the management of osteoporosis is incorrect?
A. Has been shown to prevent bone loss and to reduce the risk of vertebral fractures
B. Is a selective oestrogen receptor modulator
C. May worsen menopausal symptoms
D. Increases risk of thromboembolic events
E. Increases the risk of breast cancer
E - increase the risk of breast cancer
Raloxifene may actually decrease the risk of breast cancer
A 57-year-old woman has presented to the hospital with a six-hour history of intermittent retrosternal chest pain radiating into the shoulder and jaw with associated diaphoresis, dyspnoea, and dizziness.
She has a past medical history of Crohn’s disease and takes azathioprine 150mg daily. She was previously started on sulfasalazine but developed facial swelling.
ECG shows normal sinus rhythm at 78 bpm but demonstrates 3mm of ST depression in leads II, III, and aVF.
A diagnosis of an inferior non-ST elevation myocardial infarction is made.
Which medication should be avoided? A. Aspirin B. Bisoprolol C. Clopidogrel D. Ramipril E. Ticagrelor
A - aspirin
Patients who are allergic to aspirin may also react to sulfasalazine
A 55-year-old right-handed male cleaner presents with elbow pain. He describes a gradual onset of pain in his right elbow over a period of six weeks with only mild relief from paracetamol. The patient plays golf and exercises at the gym weekly. He does remember a fall at work six weeks ago where he fell onto his left elbow. The patient feels systemically well and denies any pyrexia. On examination, the patient describes tenderness over the right elbow with resisted wrist extension/ supination and the elbow held in extension. There is no significant swelling over the olecranon.
What is the most likely cause of this man’s elbow pain?
A. Lateral epicondylitis B. Medial epicondylitis C. Olecranon bursitis D. Radial head fracture E. Septic arthritis
A - lateral epicondylitis
Lateral epicondylitis: worse on resisted wrist extension/suppination whilst elbow extended
The most likely answer here is lateral epicondylitis. Lateral epicondylitis or ‘tennis elbow’ presents with gradual onset pain around the lateral epicondyle of the humerus radiating to the forearm. This patient’s job is a cleaner, therefore undertaking repetitive movements with his dominant hand increases the chance of lateral epicondylitis.
Medial epicondylitis or ‘golfer’s elbow’ presents with pain around the medial epicondyle. Pain is elicited during wrist flexion and pronation and does not match the examination findings in the scenario.
Olecranon bursitis presents with swelling and erythema over the olecranon, this patient does not have this as outlined in the question.
Radial head fractures present with immediate pain and swelling, the patient in this case presented with gradual onset pain; making a fracture a less likely differential in this patient.
As this patient is systemically well, septic arthritis is unlikely. Also, the affected joint will appear red, painful and swollen.
A 38-year-old woman is reviewed. She has a diagnosis of rheumatoid arthritis. She has recently been switched from methotrexate to leflunomide. Monitoring of the full blood count and liver function tests has been arranged. What else should be monitored during treatment?
A. Peak expiratory flow rate B. Blood pressure C. QT interval on ECG D. Blood glucose E. Urine for microscopic haematuria
Leflunomide may cause hypertension
B
A 60-year-old woman who has recently started treatment for polymyalgia rheumatica presents with a five day history of headaches and reduced vision on the right side since this morning There is no eye pain but the there is a ‘large, dark shadow’ covering the superior visual field on the right side. On examination she has a tender, palpable right temporal artery. What is the most likely explanation for the reduced vision?
Anterior ischemic optic neuropathy Central retinal vein occlusion Optic neuritis Ophthalmic arteritis Central retinal artery occlusion
Anterior ischemic optic neuropathy
Which one of the following best describes rheumatoid factor?
IgG against the Fc portion of IgM IgM against the Fc portion of IgA IgM against the Fc portion of IgM IgM against the Fc portion of IgG IgG against the Fc portion of IgA
IgM against the Fc portion of IgG
Which one of the following cytokines is the most important in the pathophysiology of rheumatoid arthritis?
IFN-beta IFN-alpha IL-4 Tumour necrosis factor IL-2
Rheumatoid arthritis - TNF is key in pathophysiology
This young man presented with the sign and symptoms of an aortic dissection. Aortic dissection occurs when the there is a tear in the inner wall of the aorta, the intima, leading to an accumulation of blood within the wall of the blood vessel (false lumen as opposed to the true lumen of the aorta). This can occur due to high stress on the aortic wall (chronic uncontrolled hypertension) or a weakening of the aortic wall. This man does not have a history of hypertension but he has signs of a genetically inherited condition strongly associated with aortic dissection. He also has an uncle who died young of a heart complication which could be an aortic dissection due to Marfan syndrome. This condition is known as Marfan syndrome and is inherited in an autosomal dominant pattern.
1: This occurs due to normal aging and is responsible for the appearance of wrinkles and the decreased elasticity of the skin in the elderly population.
2: This is the mechanism via which vitamin C deficiency leads to scurvy.
3: In Marfan syndrome, there is a defection of the glycoprotein fibrillin which normally envelopes elastin and as a result individuals with the condition has a range of signs symptoms such as joint hypermobility, chest deformities and long toes and finger amongst others.
4: This is usually seen in a genetically inherited condition called Menkes disease. The condition is inherited in an X-linked recessive pattern and involves an accumulation of copper in some body tissues.
5: This is characteristic of alpha-1-antitrypsin deficiency whereby there is a deficiency of the enzyme alpha-1-antitrypsin which normally functions to inhibit elastase. The excessive degradation of collagen leads to pan-acinar emphysema and liver impairment.
Decrease in the production of collagen
Impaired hydroxylation of lysine and proline amino acids on collagen
A defect of the glycoprotein structure which usually wraps around elastin
Copper deficiency affecting the normal function of lysyl oxidase enzyme
Overactive elastase enzyme leading to an excessive breakdown of collagen
A defect of the glycoprotein structure which usually wraps around elastin
Decrease in the production of collagen
Impaired hydroxylation of lysine and proline amino acids on collagen
A defect of the glycoprotein structure which usually wraps around elastin
Copper deficiency affecting the normal function of lysyl oxidase enzyme
Overactive elastase enzyme leading to an excessive breakdown of collagen
A defect of the glycoprotein structure which usually wraps around elastin
A 45-year-old is referred to the clinic due to recurrent, progressive episodes of illness, affecting different sites of her body. She reports that the episodes can result in pain in several areas including the nose, eyes, ears and joints. No clear diagnosis has been established despite several specialists’ input.
She currently feels her symptoms are ‘flaring’ and describes pain around the ears, nose, large joint with associated breathlessness and a mild cough. On exam, there is redness and swelling of the ears and a collapse of the nasal bridge. A mild wheeze is heard on the chest.
On review of her notes, she has had previous episodes of scleritis/iritis and is under investigation for hearing loss.
What is the most likely diagnosis?
Anti-glomerular basement membrane disease (Goodpasture syndrome)
Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)
Granulomatosis with polyangiitis (Wegener’s granulomatosis)
Reactive arthritis
Relapsing polychondritis
Relapsing polychondritis
This patient has presented with features most in keeping with the autoimmune condition of relapsing polychondritis. This is a multi-system condition associated with repeat episodes of cartilage inflammation and deterioration. Common examples of areas affected, and therefore symptoms patients present with, include the ears (auricular chondritis, vertigo, hearing loss), the nose (saddle nose deformity), the respiratory tract (wheezing, inspiratory stridor, voice changes), the eyes (episcleritis, scleritis, iritis,) and the joints (arthralgia). Although there are several conditions that present with similar features, the involvement of the ears and nose in a multi-system disease is characteristic of relapsing polychondritis. Given its multi-organ involvement, various scoring systems have been created, based on clinical, pathological, and radiological criteria, to assist make the diagnosis.
A 45-year-old man who is known to have Marfan’s syndrome presents with lower back pain. This has been present for a few months now and is associated with headaches, leg pain and intermittent episodes of urinary incontinence. What is the most likely diagnosis?
Depression Spinal stenosis Leaking aortic abdominal aneurysm Multiple sclerosis Dural ectasia
Marfan’s syndrome - dural ectasia
Dural ectasia affects around 60% of patients with Marfan’s syndrome. It may cause lower back pain associated with neurological problems such as bladder and bowel dysfunction.
A 24-year-old man reports a 1-year history of increasing low back pain. He reports that the symptoms are usually worse after being sedentary. There is also stiffness in this area that can last up to 30 minutes in the morning. He is normally a keen footballer and gym-goer and finds that both of these actually improve his pain levels. However, he has been increasingly troubled by pain in his right Achilles tendon which has limited his activity levels.
His GP has referred him to a rheumatologist and has arranged lumbar spine and sacroiliac joint x-rays in the meantime.
Which of these collection of findings are most likely to be seen?
A. Block vertebra and excessive lumbar lordosis
B. Joint space narrowing, osteophytes and subchondral cysts
C. Marginal erosions, soft tissue swelling and periarticular osteoporosis
D. Soft tissue swelling, punched-out bone lesions and overhanging sclerotic margins
E. Subchondral erosions, sclerosis and squaring of vertebrae
E. Subchondral erosions, sclerosis and squaring of vertebrae
This man has typical features of ankylosing spondylitis: low back pain which is worse at rest and improves on activity, early morning stiffness exceeding 15 minutes in duration, and associated Achilles tendinopathy (enthesitis). Onset is most common in males between 20-30 years of age. Plain radiography commonly shows subchondral erosions and sclerosis affecting the sacroiliac joints (sacroiliitis). In the lumbar spine, abnormalities such as vertebral body squaring, ligament calcification and syndesmophytes may be seen, eventually leading to the formation of a ‘bamboo spine’.
A 50-year-old man with no past medical history is investigated for ongoing back pain. He is found to have a vertebral collapse secondary to osteoporosis. What is the most appropriate test to determine the cause of his osteoporosis?
Thyroid function tests Prostate specific antigen Oestrogen level Prolactin level Testosterone level
Osteoporosis in a man - check testosterone
Low levels of which one of the following types of complement are associated with the development of systemic lupus erythematous?
C4 C5 C6 C7 C8
SLE: complement levels are usually low during active disease - may be used to monitor flares
Low levels of C4a and C4b have been shown to be associated with an increased risk of developing systemic lupus erythematous
A 43-year-old Chinese man is reviewed after describing painful paraesthesia affecting the palmar aspect of his right hand. He also reports occasional blood when passing urine. His past medical history is significant for chronic hepatitis B infection, for which he takes lamivudine.
On examination, he reports numbness and tenderness in the distribution of the ulnar nerve on the right hand side. He also has a patch of anaesthesia over the dorsum of his left foot.
His blood test results are shown below:
Hb 132 g/L Male: (135-180) Female: (115 - 160) Platelets 398 * 109/L (150 - 400) WBC 9.6 * 109/L (4.0 - 11.0) Na+ 136 mmol/L (135 - 145) K+ 4.8 mmol/L (3.5 - 5.0) Urea 13.5 mmol/L (2.0 - 7.0) Creatinine 168 µmol/L (55 - 120) CRP 35 mg/L (< 5)
What is the most likely diagnosis?
Polyarteritis nodosa Takayasu's arteritis Microscopic polyangiitis Granulomatosis with polyangiitis Eosinophilic granulomatosis with polyangiitis
Polyarteritis nodosa can cause a mononeuritis multiplex syndrome
This patient has a number of features of vasculitis, including haematuria with renal impairment, and a mildly raised CRP. The specific diagnosis of polyarteritis nodosa (PAN), a medium vessel vasculitis, is clinched by the presence of mononeuritis multiplex i.e. peripheral neuropathy affecting two or more non-contiguous nerve trunks, as well as hepatitis B infection, which is significantly associated with PAN.
A 40-year-old man presents with pain in his lower back and ‘sciatica’ for the past three days. He describes bending down to pick up a washing machine when he felt ‘something go’. He now has severe pain radiating from his back down the right leg. On examination he describes paraesthesia over the anterior aspect of the right knee and the medial aspect of his calf. Power is intact and the right knee reflex is diminished. The femoral stretch test is positive on the right side. Which nerve root is most likely to be affected?
Common peroneal nerve Lateral cutaneous nerve of the thigh L5 L3 L4
L4
A 50-year-old man presented with gradual onset low backache for 2-years. There was no history of any chronic illness or alcoholism. His clinical examination revealed lumbar spine tenderness with a soft and non-tender abdomen.
A bone scan revealed the presence of intense abnormal activity within the skull, lower thoracic and lumbar vertebrae as well as his pelvic bones. Biopsy performed of the L3 vertebra demonstrated trabeculae of cortical and cancellous bone with fibrotic marrow.
Given the likely diagnosis, which of the following would be elevated?
Urinary coproporphyrin Urinary delta-aminolevulinic acid Urinary hydroxyproline Urinary porphobilinogen Urinary uroporphyrin
Paget’s disease - increased serum and urine levels of hydroxyproline
Which one of the following drugs has been associated with an increased risk of atypical stress fractures of the proximal femoral shaft?
Spironolactone Alendronate Quetiapine Venlafaxine Clopidogrel
Alendronate
Bisphosphonates are associated with an increased risk of atypical stress fractures
A 27-year-old woman is referred to orthopaedics. Three years ago she had chemotherapy for non-Hodgkin’s lymphoma. Follow up scans to date have shown no evidence of any disease recurrence. For the past two months, she has been experiencing gradually increasing pain in her right hip which is worse on exercising. On examination, passive movement of the hip is painful in all directions, especially internal rotation. An x-ray ordered by her GP has been reported as normal.
What is the most likely diagnosis?
Trochanteric bursitis Avascular necrosis of the femoral head Primary hyperparathyroidism Metastatic deposits Hypoparathyroidism
Previous chemotherapy is a significant risk factor for avascular necrosis
Initial x-rays are often normal in patients with avascular necrosis, but it would be unlikely
A 43-year-old who is noted to have a high-arched palate, arachnodactyly and a late-systolic murmur presents with visual problems. Which one of the following eye disorders is most associated with his underlying condition?
Superotemporal ectopia lentis Inferonasal ectopia lentis Retinitis pigmentosa Acute glaucoma Retinal detachment
Superotemporal ectopia lentis
Marfan’s syndrome - upwards lens dislocation
54-year-old female is reviewed in the rheumatology clinic due to dry eyes and arthralgia. A diagnosis of primary Sjogren’s syndrome is suspected. Which one of the following features is least associated with this condition?
Renal tubular acidosis Xerostomia Sensory polyneuropathy Dilated cardiomyopathy Raynaud's phenomenon
Dilated cardiomyopathy
A 55-year-old woman presents with a four week history of shoulder pain. There has been no obvious precipitating injury and no previous experience. The pain is worse on movement and there is a grating sensation if she moves the arm too quickly. She also gets pain at night, particularly when she lies on the affected shoulder. On examination there is no obvious erythema or swelling. Passive abduction is painful between between 60 and 120 degrees. She is unable to abduct the arm herself past 70-80 degrees. Flexion and extension are preserved. What is the most likely diagnosis?
Adhesive capsulitis (frozen shoulder) Supraspinatus tendonitis Acromioclavicular joint injury Glenohumeral arthritis Superior labral lesion
Supraspinatus tendonitis
A 26-year-old renal transplant recipient secondary to polycystic kidney disease enters the follow-up clinic. She has been started on multiple immunosuppressant agents including mycophenolate mofetil.
What is the mechanism of action of this drug?
HMG-CoA reductase inhibitor Calcineurin inhibitor Protease inhibitor Inosine-5'-monophosphate dehydrogenase inhibitor Ribonucleotide reductase inhibitor
Mycophenolate mofetil inhibits of inosine-5’-monophosphate dehydrogenase which is needed for purine synthesis
Mycophenolate mofetil (MMF) reduces lymphocyte production through inhibition of iosine-5’-monophosphate dehydrogenase. It is typically used in organ transplantation and autoimmune conditions. Both MMF and azathioprine inhibit purine synthesis but through different mechanisms.
Calcineurin inhibitors (tacrolimus and ciclosporin) are immunosuppressants which work through reducing t-cell differentiation.
Protease inhibitors such as ritonavir and darunavir are antivirals used against HIV and hepatitis.
Statins are examples of HMG-CoA reductase inhibitors. They work by reducing LDL cholesterol.
Hydroxycarbamide is an example of a ribonucleotide reductase inhibitor which decreases the production of deoxyribonucleotides reducing DNA synthesis. It is used to treat cancers.
Looser’s zones x-ray are most characteristically associated with which one of the following conditions?
Primary hyperparathyroidism Hypoparathyroidism Osteomalacia Paget's disease Osteoporosis
Osteomalacia
Which of the following is associated with a good prognosis in rheumatoid arthritis?
Rheumatoid factor negative HLA DR4 Anti-CCP antibodies Rheumatoid nodules Insidious onset
Rheumatoid factor negative
A 45-year-old woman is seen in the rheumatology clinic with a 2-month history of joint pain. She has pain in her wrists and fingers bilaterally, which is worse in the morning and improves throughout the day. She is an artist by trade and finds it increasingly difficult to hold a paintbrush and have the same dexterity due to her fingers’ pain and swelling. She has no past medical history.
On examination, she has tender wrist joints. Her range of motion is limited due to pain and stiffness. Her entire fingers are swollen and painful, particularly over the distal interphalangeal joints with a reduced range of movement. There are no visible nodules, skin or nail changes.
What is the most likely diagnosis?
CREST syndrome Osteoarthritis Psoriatic arthritis Raynauds phenomenon Rheumatoid arthritis
Dactylitis + DIP involvement = psoriatic arthritis
A 41-year-old woman is reviewed in the oncology clinic, following a recent diagnosis of ER-positive, HER2-negative breast cancer, for which she is receiving adjuvant treatment with tamoxifen. She reports that she has been feeling increasingly tired and weak recently.
On examination, there is a new, reddish-purple rash surrounding her eyelids. She also reveals symmetrical, violaceous papules over the dorsal aspect of her proximal and distal interphalangeal joints.
Which of the following auto-antibodies is most likely to be positive in this patient?
Anti-Mi-2 antibodies c-ANCA (c-antineutrophil cytoplasmic antibodies Anti-Yo antibodies Anti-nuclear antibodies (ANA) Rheumatoid factor
ANA
Dermatomyositis antibodies: ANA most common, anti-Mi-2 most specific
This patient has a diagnosis of dermatomyositis - a paraneoplastic condition seen in the context of a range of solid and haematological malignancies, including breast cancer. This diagnosis is supported by the classic description of the ‘heliotrope’ rash around her eyelids, as well as Gottron’s papules. Although the presence of fatigue and weakness is non-specific in patients with cancer, in this instance, it points towards the myopathic element of her condition. The most common antibodies found in patients with dermatomyositis are anti-nuclear antibodies, which are seen in 60% of patients.
A 17-year-old male presents to General Practice with recurrent cramping in his arms during a rowing training session. On closer questioning, he has noticed a reduction in his exercise tolerance recently. He reports getting a ‘second wind’ phenomenon after minutes of rest.
On examination, he is well with no apparent deficit.
Bloods reveal:
Hb 142 g/l Platelets 204 * 109/l WBC 6.7 * 109/l Na+ 139 mmol/l K+ 4.2 mmol/l Urea 8.9 mmol/l Creatinine 148 µmol/l Adjusted calcium 2.23 mmol/L Creatine kinase 1037 IU/L Autoimmune profile pending
What is the most likely underlying diagnosis? Polymyositis Dermatomyositis Von Gierke's disease McArdle's disease Pompe's disease
McArdle’s disease is characteristically associated with a ‘second-wind’ phenomenon and present with distal muscle cramping in this demographic following exercise.
Polymyositis and dermatomyositis would, typically but not always, cause a more marked increase in creatine kinase which can be up to 100 times the upper limit of normal.
Von Gierke patients present with manifestations related to hypoglycemia around three to four months of age. Pompe’s disease tends to also present in infants but with cardiomyopathy and severe, generalised muscular hypotonia.
A 65-year-old man with a history of type 2 diabetes mellitus and peripheral arterial disease is investigated for fatigue and pyrexia of unknown origin. He recently had an amputation of a toe on his left foot. A diagnosis of osteomyelitis is suspected in the left foot. What is the most appropriate investigation?
MRI Plain x-ray Positron emission tomographic (PET) scan Bone biopsy CT scan
Osteomyelitis: MRI is the imaging modality of choice
A 2-year-old girl is brought to her paediatrician. The mother complains that she noticed her daughter has been having repeated ear infections despite repetitive oral antibiotic treatment courses. The mother is very concerned and also thinks that her daughter has not been putting much weight recently. The mother is also worried that the condition might affect her child’s hearing and subsequent development. Upon examination, the paediatrician finds a scaly and erythematous rash on the girl’s scalp.
The paediatrician decides to get an X-ray of the skull which shows lytic bone lesions. The girl was born via spontaneous vaginal delivery and has been well previously. The doctor later explains to her junior colleague that electron microscopy of the skin lesions in this condition typically shows tennis racket-shaped granules.
What is the most likely diagnosis?
Recurrent otitis media Osteopetrosis Paget disease of the bone Langerhans cell histiocytosis Sarcoidosis
Langerhans cell histiocytosis is characterized by Birbeck granules on electron microscopy
A 57-year-old woman with a history of polymyalgia rheumatica has been taking prednisolone 10 mg for the past 5 months. A DEXA scan is reported as follows:
L2 T-score -1.6 SD
Femoral neck T-score -1.7 SD
What is the most suitable management?
A. No treatment
B. Vitamin D + calcium supplementation + repeat DEXA scan in 6 months
C. Vitamin D + calcium supplementation
D. Vitamin D + calcium supplementation + hormone replacement therapy
E. Vitamin D + calcium supplementation + oral bisphosphonate
\
E. Vitamin D + calcium supplementation + oral bisphosphonate
This patient has been taking 10mg of prednisolone for the past 5 months and hence should be assessed for bone protection. The T score of less than -1.5 SD is an indication for a bisphosphonate. This should be co-prescribed with calcium +
vitamin D.
Which one of the following is most consistently associated with a poor prognosis in rheumatoid arthritis?
Anti-CCP antibodies HLA DR2 allele Rapid onset Being a smoker Female sex
Anti-CCP antibodies
Which one of the following cells secretes the majority of tumour necrosis factor in humans?
Neutrophils Macrophages Natural killer cells Killer-T cells Helper-T cells
Macrophages
A 43-year-old woman who has rheumatoid arthritis is reviewed in clinic. She has responded poorly to methotrexate and consideration is being given to starting sulfasalazine. An existing allergy to which one of the following drugs may be a contradiction to the prescription?
Penicillin Trimethoprim Aspirin Sulpiride Leflunomide
Aspirin
Patients who are allergic to aspirin may also react to sulfasalazine
You are working in the emergency department. A 30-year-old lady with rheumatoid arthritis has been referred by her GP with a two day history of worsening pain and swelling in the left wrist. It has now become so bad in the last few hours that she can barely move it. She returned from Thailand two weeks ago where she was travelling with some friends. She had no problems with her wrist when travelling, but does remember getting a rash on her chest and arms one day when it was particularly hot. This only lasted a day. Her rheumatoid arthritis is usually well controlled on methotrexate. She has no drug allergies. You aspirate the wrist which returns a turbid fluid and send it for gram stain. Given the likely diagnosis which intravenous antibiotic do you start her on?
Vancomycin Flucloxacillin Ceftriaxone Ciprofloxacin Clarithromycin
Flucox
The diagnosis is septic arthritis. The most common bacteria being Staph.aureus. Patients with rheumatoid arthritis are particularly prone to septic arthritis. The rash was a heat rash and a red-herring. Flucloxacillin is the first line treatment for non-penicillin allergic septic arthritis.
Vancomycin is reserved for MRSA positive septic arthritis.
Ceftriaxone is used in gonococcal arthritis.
Ciprofloxacin and clarithromycin are not used in septic arthritis (unless microbiology
Osteopetrosis is due to a defect in:
Osteoclast function PTH receptorS Osteoblast function Calcium resorption in proximal tubule Calcium absorption
Osteoclast function
A 28-year-old male with pseudoxanthoma elasticum attends the outpatient clinic for follow up. He has a family history of coronary artery disease and is concerned about his risk. A routine echo is done as part of his cardiovascular evaluation.
What is the most common finding on echocardiography in these patients?
Aortic regurgitation Mitral valve prolapse Thoracic aortic aneurysm Dilated cardiomyopathy Pulmonary hypertension
Pseudoxanthoma elasticum is associated with mitral valve prolapse and increased risk of ischaemic heart disease
A 36-year-old woman presents with progressive tingling and numbness of the 4th and 5th fingers of her right hand. Initially, this was intermittent but recently has become constant. She notices that the symptoms are worse when leaning on her right elbow. She recalls hitting her elbow against a door some time ago.
What is the most likely diagnosis?
Carpal tunnel syndrome Cubital tunnel syndrome De Quervain's tenosynovitis Medial epicondylitis Radial tunnel syndrome
Cubital tunnel syndrome is caused by compression of the ulnar nerve and can present with tingling/numbness of the 4th and 5th finger
Which one of the following is not a risk factor for developing osteoporosis?
Smoking Obesity Sedentary lifestyle Premature menopause Female sex
Low body mass, rather than obesity is associated with an increased risk of developing osteoporosis
A 58-year-old woman complains of aches and pains in her bones. Her family have noticed she is generally weak and lethargic. A series of blood tests are requested:
Calcium 2.04 mmol/L (2.2 - 2.6) Albumin 39 g/L (35 - 50) Phosphate 0.63 mmol/L (0.8 - 1.4) Alkaline phosphatase 271 U/L (30 - 100) Vitamin D3 15 nmol/L (75 - 200) Parathyroid hormone 10.8 pmol/L (0.8 - 8.5)
What is the most appropriate management?
Arrange a liver ultrasound
Refer for a technetium-MIBI subtraction scan
Arrange a DEXA scan
Arrange serum electrophoresis and a skeletal survery
Start vitamin D3 supplementation
Start vitamin D3 supplementation
A 34-year-old kitchen worker presents with a two week history of pain in her right wrist. She has recently emigrated from Ghana and has no past medical history of note. On examination she is tender over the base of her right thumb and also over the radial styloid process. Ulnar deviation of the wrist recreates the pain. What is the most likely diagnosis?
Rheumatoid arthritis Osteoarthritis of the carpometacarpal joint De Quervain's tenosynovitis Carpal tunnel syndrome Systemic lupus erythematosus
Pain on the radial side of the wrist/tenderness over the radial styloid process ? De Quervain’s tenosynovitis
Cristiano, 36-year-old gentleman presents to the emergency department complaining of a painful, swollen knee. On examination, he is limping and unable to fully extend or flex his knee. He has a tense effusion in his knee, which is warm to touch and swollen. His eyes also appear red and irritated. He reports dysuria and tells you that he was diagnosed and treated for an STI three weeks ago. The patient records show that Cristiano has previously tested HLA-B27 positive and has presented several times in the past with similar symptoms. You perform arthrocentesis. Which one of the following sets of characteristics would you expect the synovial fluid to show?
A. Cloudy yellow colour, Culture negative, Calcium pyrophosphate crystals, White cell count: 15,000/mm
B. Turbid grey colour, Weakly gram-negative ovoid shaped bacteria, No crystals, White cell count: 90,000/mm
C. Turbid grey colour, Gram-negative diplococci, No crystals, White cell count: 90,000/mm
D. Cloudy yellow colour, Culture negative, No crystals, White cell count: 20,000/mm
E. Cloudy yellow colour, Culture negative, Urate crystals, White cell count: 20,000/mm
D. Cloudy yellow colour, Culture negative, No crystals, White cell count: 20,000/mm
Reactive arthritis: develops after an infection where the organism cannot be recovered from the joint
A 50-year-old diabetic right-handed lady presents with left shoulder pain. She describes a stiff shoulder often more painful at night and has difficulty dressing or doing up her bra. On examination, there is no point tenderness and you notice weakness in external rotation.
What is the most likely cause of her shoulder pain?
Acromioclavicular degeneration Subacromial impingement Rotator cuff tear Calcific tendinopathy Adhesive capsulitis
External rotation is classically impaired in adhesive capsulitis
Which one of the following statements concerning discoid lupus is correct?
Commonly progresses to SLE Causes non-scarring alopecia Characterised by follicular keratin plugs Is rarely photosensitive Typically presents in older males
Discoid lupus erythematosus is characterised by follicular keratin plugs
Which one of the following is least recognised as a risk factor for developing osteoporosis?
Cushing's syndrome Turner's syndrome Hyperparathyroidism Hypothyroidism Diabetes mellitus
Hypothyroidism
Hyperthyroidism is associated with an increased risk of osteoporosis. Patients with hypothyroidism who take excessive amounts of levothyroxine may also be at risk of osteoporosis
A 37-year-old female presents with pain and stiffness in her hands. Her symptoms are worse in the morning and seem to improve throughout the day. She has recently returned from a holiday in Ibiza and is taking the oral contraceptive pill. On examination, she has bilateral swelling and tenderness in the distal and proximal interphalangeal joints.
What is the most likely diagnosis?
Gonococcal arthritis Psoriatic arthritis Reactive arthritis Rheumatoid arthritis Systemic lupus erythematosus
Inflammatory arthritis involving DIP swelling and dactylitis points to a diagnosis of psoriatic arthritis
A 33-year-old female is admitted to the Emergency Department due to right-sided weakness. She has a past history of deep vein thrombosis following the birth of her daughter. The only other past history of note is two miscarriages. A CT head confirms an ischaemic stroke in the left middle cerebral artery territory. What is the likely finding on echocardiography?
Normal Dilated cardiomyopathy Bicuspid aortic valve Atrial septal defect Ventricular septal defect
Normal
A patient attends a rheumatology clinic for follow-up. They have been brought back to discuss starting an alternative medication for their rheumatoid arthritis after experiencing side-effects from their previous treatment. A decision is made to commence methotrexate.
Two weeks later, they are referred to the medical admissions unit with rigours, right flank pain, dysuria and pyrexia. You review them and a decision is made to start antibiotic treatment.
Which antibiotic is contraindicated?
Cefalexin Gentamicin Vancomycin Co-trimoxazole Nitrofurantoin
Methotrexate works by inhibiting dihydrofolate reductase
Methotrexate acts by inhibiting dihydrofolate reductase which thereby inhibits the synthesis of purines which, in turn, arrests cell division. Anti-inflammatory effects are mediated by an increase in extracellular ATP which leads to an anti-inflammatory response. Co-trimoxazole contains sulfamethoxazole and trimethoprim. Both of these antibiotics exert their therapeutic effect through inhibition of folate metabolism. Therefore there is a greater risk of pancytopenia.
A 54-year-old farm worker presents for review. She has recently been diagnosed with osteoarthritis of the hand but has no other past medical history of note. Despite regular paracetamol she is still experiencing considerable pain, especially around the base of both thumbs. What is the most suitable next management step?
Add oral diclofenac + lansoprazole Switch paracetamol for co-codamol 8/500 Add topical ibuprofen Add oral ibuprofen Add oral glucosamine
Add topical ibuprofen
Osteoarthritis - paracetamol + topical NSAIDs (if knee/hand) first-line
A 44-year-old female with a history of Raynaud’s phenomenon is reviewed in the rheumatology clinic. She is currently being investigated for dysphagia. On examination she is noted to have tight, shiny skin over her fingers. Which one of the following complications is she most likely to develop?
Early onset dementia Erythema nodosum Malabsorption Constrictive pericarditis Erosive joint disease
Malabsorption
This patient is likely to have CREST syndrome, a subtype of limited cutaneous systemic sclerosis. Malabsorption can develop in these patients secondary to bacterial overgrowth of the sclerosed small intestine
Whilst diffuse systemic sclerosis is associated with more severe and rapid internal organ involvement it is also seen in the limited form.
A 44-year-old female with a history of Raynaud’s phenomenon is reviewed in the rheumatology clinic. She is currently being investigated for dysphagia. On examination she is noted to have tight, shiny skin over her fingers. Which one of the following complications is she most likely to develop?
Early onset dementia Erythema nodosum Malabsorption Constrictive pericarditis Erosive joint disease
Malabsorption
This patient is likely to have CREST syndrome, a subtype of limited cutaneous systemic sclerosis. Malabsorption can develop in these patients secondary to bacterial overgrowth of the sclerosed small intestine
Whilst diffuse systemic sclerosis is associated with more severe and rapid internal organ involvement it is also seen in the limited form.
A 63-year-old man presents to the Emergency Department with a 2 day history of a painful and swollen left knee joint. Aspiration reveals positively birefringent crystals and no organisms are seen. Which of the following conditions are not recognised causes of the underlying condition?
Haemochromatosis Low magnesium High phosphate Acromegaly Hyperparathyroidism
High Phosphate
A low phosphate predisposes to pseudogout
A low phosphate predisposes to pseudogout
Each one of the following is seen in reactive arthritis, except:
Urethritis Keratoderma blenorrhagica Conjunctivitis Aseptic meningoencephalitis Circinate balanitis
Aseptic meningoencephalitis
A 57-year-old woman presents with a three month history of right-sided hip pain. This seems to have come on spontaneously without any obvious precipitating event. The pain is described as being worse on the ‘outside’ of the hip and is particularly bad at night when she lies on the right hand side.
On examination there is a full range of movement in the hip including internal and external rotation. Deep palpation of the lateral aspect of the right hip joint recreates the pain.
An x-ray of the right hip is reported as follows:
Right hip: Minor narrowing of the joint space otherwise normal appearance
What is the most likely diagnosis?
Fibromyalgia Lumbar nerve root compression Osteoarthritis Greater trochanteric pain syndrome Meralgia paraesthetica
Greater trochanteric pain syndrome
A 48-year-old man presents to the emergency department with reports of abdominal pain, fever, joint swelling and sharp chest pain. This constellation of symptoms has affected him on three previous occasions, resolving after 1-3 days. He is originally from Turkey.
Observations:
Heart rate 82 beats per minute
Blood pressure 120/77 mmHg
Temperature 38.4ºC
On examination, there the abdomen is mildly tender throughout. There is synovitis affecting the left ankle and right knee. Leaning forward eases his chest pain.
The patient is treated with colchicine and rapidly improves.
What is the most likely diagnosis?
Behcet's syndrome Dressler's syndrome Familial Mediterranean fever Gout Reactive arthritis
Familial Mediterranean fever (FMF) is an autosomal recessive, hereditary inflammatory disorder characterised by reoccurring episodes of abdominal pain, fever, arthralgia, and chest pain
What is the most common cardiac defect seen in Marfan’s syndrome
Mitral valve prolapse Coarctation of the aorta Bicuspid aortic valve Dilation of the aortic sinuses Ventricular septal defect
Marfan’s syndrome is associated with dilation of the aortic sinuses which may predispose to aortic dissection
A 76-year-old gentleman is seen in Rheumatology clinic for a painful left first metatarsophalangeal joint. He has a past medical history of chronic kidney disease stage 4, heart failure and poorly controlled type 2 diabetes.
His GP suspects an acute flare-up of gout and would like to commence treatment. What is the most appropriate medication to initiate?
Naproxen Prednisolone Colchicine Allopurinol Ibuprofen
Colchicine
The best medication for this patient would be colchicine titrated to his renal function. BNF recommends reducing the dose or increasing the dosage interval if eGFR 10-50ml/minute/1.73m²; avoid if eGFR less than 10mL/minute/1.73m².
NSAIDs would be contraindicated due to his chronic kidney disease, prednisolone would worsen his already poorly controlled diabetes, and allopurinol would not be indicated for an acute flare.
A 50-year-old woman presents to the rheumatology clinic. Her underlying condition initially presented with signs and symptoms consistent with Raynaud’s syndrome. She then had painful, swollen joints and felt increasingly fatigued. Later her skin began to become increasingly tight, leathery, and shiny.
What is the most common cause of death in patients with this underlying diagnosis?
Acute kidney injury Myocardial infarction Respiratory failure Seizure Stroke
Respiratory failure
The most common cause of death in systemic sclerosis is respiratory involvement: interstitial lung disease and pulmonary arterial hypertension
A 45-year-old woman with a history of primary Sjogren’s syndrome is reviewed in clinic. Her main problem is a dry mouth, which unfortunately has not responded to artificial saliva. Which one of the following medications is most likely to be beneficial?
Rivastigmine Neostigmine Clonidine Atropine Pilocarpine
Pilocarpine
A 31-year-old female intolerant of methotrexate is started on azathioprine for rheumatoid arthritis. Routine blood monitoring shows:
Hb 7.9 g/dl
Plt 97 * 109/l
WBC 2.7 * 109/l
Which of the following factors will predispose her to azathioprine toxicity?
Cimetidine Rifampicin Fast acetylator status Thiopurine methyltransferase deficiency Alcohol excess
Azathioprine - check thiopurine methyltransferase deficiency (TPMT) before treatment
Thiopurine methyltransferase (TPMT) deficiency is present in about 1 in 200 people and predisposes to azathioprine related pancytopaenia
hich one of the following is least associated with Behcet’s syndrome?
Mouth ulcers Genital ulcers Conjunctivitis Deep vein thrombosis Aseptic meningitis
Conjunctivitis
Oral ulcers + genital ulcers + anterior uveitis = Behcet’s
A 48- year-old patient presented to her family physician complaining of pain all over her body for the past 4 months. She initially thought it was because she started taking aerobic exercise classes 6 months ago. However, the pain has been persistently bothering her and taking over-the-counter pain relievers did not help. The pain is more pronounced around her shoulders and lower back. She also complains feeling tired and not being able to sleep because of the pain. She has a body mass index of 28 kg per m2 and has never smoked in her life. She denies any weight loss or episodes of fever. She currently lives alone and is usually able to do her household chores. Her mother who passed away last year suffered from rheumatoid arthritis. On examination, the doctor finds that the patient has tender areas on her neck, elbow regions, and knees. Which is the most likely diagnosis in this patient?
Polymyalgia rheumatica Fibromyalgia Polymyositis Ankylosing spondylitis Rheumatoid arthritis
Fibromyalgia
This patient presented with a chronic pain described as all over her body for the past 6 months. The pain did not appear to be originating from the joints and therefore this makes a diagnosis of rheumatoid arthritis unlikely. The two most likely diagnoses are fibromyalgia and polymyalgia rheumatica. However, polymyalgia rheumatica would have also presented with weight loss and fever. This patient most likely has fibromyalgia. She also feels tired and suffers from sleep disturbances, which are both common complaints in patients suffering from fibromyalgia.
A 72-year-old woman presents with a severe right-sided headache and some blurring of vision that comes and goes. She has no significant past medical history. On examination, there is tenderness on palpation of her right temporal region. Visual acuity is 6/36 in her right eye and 6/9 in her left.
Investigations:
Erythrocyte sedimentation rate 154mm/hr (1-36)
C-reactive protein 116mg/L (<10)
What drug should be given initially?
Cyclosporine (intravenous) Leflunomide (oral) Methotrexate (oral) Prednisolone (oral) Methylprednisolone (intravenous)
Uncomplicated GCA (no visual involvement and/or jaw/tongue claudication) should be treated with oral prednisolone 40-60mg daily until symptoms and investigations normalise.
Complicated GCA (with visual involvement and/or jaw/tongue claudication), as in this scenario, should be given IV methylprednisolone 500-1000mg for 3 days before starting oral prednisolone.
A 54-year-old man is diagnosed as having gout. You are discussing ways to help prevent future attacks. Which one of the following is most likely to precipitate an attack of gout?
Chocolate Brazil nuts Eggs Sardines Smoking
Foods to avoid include those high in purines e.g. Liver, kidneys, seafood, oily fish (mackerel, sardines) and yeast products
A 70-year-old woman presents with loss of vision in her left eye. For the past two weeks she has painful frontal headaches and has been feeling generally lethargic. On examination visual acuity is 6/9 in the right eye but on the left side only hand movements can be made seen. Fundoscopy of the left side reveals a pale and oedematous optic disc. What is the most likely diagnosis?.
Acute angle closure glaucoma Central retinal artery occlusion Multiple sclerosis Methanol poisoning Temporal arteritis
Temporal arteritis
This patient has likely developed anterior ischemic optic neuropathy on the left side
You are doing the annual review of a 50-year-old woman who has rheumatoid arthritis. Which one of the following complications is most likely to occur as a result of her disease?
Chronic lymphocytic leukaemia Hypertension Colorectal cancer Type 2 diabetes mellitus Ischaemic heart disease
Rheumatoid arthritis: patients have an increased risk of IHD
Patients with Sjogren’s syndrome are at an increased risk of which one of the following malignancies?
Myeloma Oesophageal cancer Intraocular melanoma Squamous cell skin cancer Non-Hodgkin's lymphoma
Patients with Sjogren’s syndrome have an increased risk of lymphoid malignancies
A 53-year-old woman with a background of seropositive non-erosive rheumatoid arthritis presents to the rheumatology outpatient clinic with pain and swelling in her hands, feet and shoulders. She is currently taking sulfasalazine as disease-modifying therapy. She has not trialled any other disease-modifying agents for rheumatoid arthritis. She is post-menopausal.
On examination, she has 7 tender and 5 swollen joints. The CRP is 35mg/L. Her patient global health score is 70/100. She has a DAS28-CRP of 5.34, indicating high disease activity.
Her rheumatologist decides to add an additional disease-modifying drug anti-rheumatic drug (DMARD).
What is the mechanism of action of the next logical medication choice?
Anti-TNF monoclonal antibody Inhibition of dihydrofolate reductase Inhibition of janus kinase Inhibition of IL-6 Anti-CD20 monoclonal antibody
Inhibition of dihydrofolate reductase
Anti-TNF monoclonal antibody is not the right answer. A different class of medications used to treated rheumatoid arthritis known as ‘anti-TNF’ biologic medications work by this mechanism of action. This class of medication is typically reserved for those patients who have trialled at least two conventional DMARDs (i.e. sulfasalazine, methotrexate, hydroxychloroquine or leflunomide) and have persistently high disease activity (DAS28-CRP or DAS28-ESR > 5.1). They include etanercept, adalimumab, certolizumab, golimumab and infliximab. They are the first line biologic disease-modifying anti-rheumatic drug in the absence of contraindications.
Inhibition of dihydrofolate reductase is the correct answer. This is the mechanism of action of methotrexate, which is considered to be an ‘anchor’ drug in rheumatoid arthritis. Before progressing to trying some of the newer biologic medications, patients with rheumatoid arthritis must try at least two conventional disease-modifying therapies (i.e. sulfasalazine, methotrexate, hydroxychloroquine or leflunomide). Hydroxychloroquine and leflunomide are not listed as answers to this question. One of the two medications trialled should be methotrexate unless contraindicated.
You are the ST1 working in the rheumatology out-patient clinic. Your next patient is a 25-year-old man who was diagnosed with ankylosing spondylitis (AS) 12 months ago. Despite regular physiotherapy and trials of two different non-steroidal anti-inflammatory drugs (NSAIDs) he remains symptomatic and asks you about the potential benefits of TNF-inhibitor therapy. You should tell him that TNF-inhibitors will improve all of the following except:
Quality of life Radiological progression Spinal mobility Extra-articular features Early morning stiffness
Radiological Progression
Radiographic damage in AS is quantified by the number of syndesmophytes, squaring, erosions and sclerosis developing at vertebral corners. Quantified radiographic damage has been shown to correlate well with spinal mobility and overall physical function. However, unlike rheumatoid arthritis and psoriatic arthritis, where TNF-inhibitors have demonstrated significant effect on progression of structural damage, the evidence to date is that the radiographic progression of AS is unaltered with the use of these agents. The only therapy showing promise for a disease modifying effect in this regard so far has been sustained use of NSAIDs.
30-year-old man with a history of mitral valve prolapse, recurrent pneumothorax, lower back pain secondary to scoliosis and pectus excavatum is considering starting a family. Given the likely diagnosis, what is the mode of inheritance of this condition?
X-linked recessive Mitochondrial Autosomal codominant Autosomal recessive Autosomal dominant
Autosomal dominant
A 50-year-old woman complains of pain in her right elbow. This has been present for the past four weeks and is maximal around 4-5cm distal from the lateral aspect of the elbow joint. The pain is made worse by extending the elbow and pronating the forearm. What is the most likely diagnosis?
Lateral epicondylitis Radial tunnel syndrome De Quervain's tenosynovitis Cubital tunnel syndrome Medial epicondylitis
Radial tunnel syndrome
Which of the following is true regarding rheumatoid factor?
It is usually an IgM molecule reacting against patient’s own IgG
High titres are not associated with severe disease
Rose-Waaler test involves agglutination of IgG coated latex particles
80% of SLE patients are RF positive
50% of patients with Sjogren’s syndrome are RF positive
Rheumatoid factor is an IgM antibody against IgG
A 44-year-old woman presents with pain in her right hand and forearm which has been getting worse for the past few weeks. There is no history of trauma. The pain is concentrated around the thumb and index finger and is often worse at night. Shaking her hand seems to provide some relief. On examination there is weakness of the abductor pollicis brevis and reduced sensation to fine touch at the index finger. What is the most likely diagnosis?
C6 entrapment neuropathy Thoracic outlet syndrome Carpal tunnel syndrome Cervical rib Pancoast's tumour
Carpel tunnel syndrome
More proximal symptoms would be expected with a C6 entrapment neuropathy e.g. weakness of the biceps muscle or reduced biceps reflex.
Patients with carpal tunnel syndrome often get relief from shaking their hands and this may be an important clue in exam questions.
A 60-year-old man with worsening dyspnoea was seen in the emergency department. His past medical history included diabetes mellitus. Additionally, a number of years before this, he had seen a dermatologist for intermittent erythema and swelling in the right auricle and nasal deformity, but no conclusive diagnosis was made. He had intermittent breathing difficulties for the past year, which was getting worse.
What is the overarching diagnosis in this case?
Bronchiolitis obliterans Churg-Strauss syndrome Leprosy Mouth and genital ulcers with inflamed cartilage (MAGIC) syndrome Relapsing polychondritis
As well as ear problems, relapsing polychondritis may present with nasal chondritis, respiratory tract involvement and arthralgia
Relapsing polychondritis is a rare autoimmune disorder characterised by recurrent episodes of inflammation and destruction of cartilaginous tissues resulting in widespread chondritis of the auricular, nasal, and tracheal cartilages as described in this case. Clinical features can include recurrent chondritis of the laryngotracheobronchitis tree and potentially end up with life-threatening laryngotracheal stenosis.
A 65-year-old Asian female presents with generalised bone pain and muscle weakness. Investigations show:
Calcium 2.07 mmol/l
Phosphate 0.66 mmol/l
ALP 256 U/l
What is the most likely diagnosis?
Bone tuberculosis Hypoparathyroidism Myeloma Osteomalacia Paget's disease
Osteomalacia
low: calcium, phosphate
raised: alkaline phosphatase
A 46-year-old female presents with a burning sensation over the antero-lateral aspect of her right thigh. A diagnosis of meralgia paraesthetica is suspected. Which nerve is most likely to be affected?
Common peroneal nerve Anterior cutaneous nerve of thigh Posterior cutaneous nerve of thigh Lateral cutaneous nerve of thigh Sciatic nerve
Burning thigh pain - ? meralgia paraesthetica - lateral cutaneous nerve of thigh compression
Potential complications of Paget’s disease include each of the following except:
Deafness Cerebral calcification Skull thickening Bone sarcoma Fractures
Cerebral calcification
Which one of the following features is least commonly seen in drug-induced lupus?
Glomerulonephritis Arthralgia Myalgia Malar rash Pleurisy
Glomerulonephritis
A 32-year-old woman presents with a facial rash that appears to be worse in summer. She has also noticed intermittent swelling of the small joints of her hands and describes suffering from Raynaud’s syndrome for 5 years.
Given the likely diagnosis which of these auto-antibodies is most likely to be positive?
Anti-dsDNA Anti-nuclear cytoplasmic antibody Anti-nuclear antibody Anti-Sm Anti-Ro
ANA
This lady presents with a photosensitive rash, Raynaud’s syndrome and small joint arthritis - giving a likely diagnosis of systemic lupus erythematosus (SLE).
Anti-nuclear (ANA) antibody is the most likely antibody to be present. Approximately 95% of people with SLE have a positive ANA, however it is not very specific.
Anti-Sm are the most specific antibodies for SLE, but only around 35% of patients with SLE will be positive. Anti-dsDNA antibodies are positive in around 70% of SLE cases.
A 32-year-old woman presents with a facial rash that appears to be worse in summer. She has also noticed intermittent swelling of the small joints of her hands and describes suffering from Raynaud’s syndrome for 5 years.
Given the likely diagnosis which of these auto-antibodies is most likely to be positive?
Anti-dsDNA Anti-nuclear cytoplasmic antibody Anti-nuclear antibody Anti-Sm Anti-Ro
ANA
This lady presents with a photosensitive rash, Raynaud’s syndrome and small joint arthritis - giving a likely diagnosis of systemic lupus erythematosus (SLE).
Anti-nuclear (ANA) antibody is the most likely antibody to be present. Approximately 95% of people with SLE have a positive ANA, however it is not very specific.
Anti-Sm are the most specific antibodies for SLE, but only around 35% of patients with SLE will be positive. Anti-dsDNA antibodies are positive in around 70% of SLE cases.
A 24-year-old man is investigated for chronic back pain. Which one of the following would most suggest a diagnosis of ankylosing spondylitis?
Reduced lateral flexion of the lumbar spine Pain gets worse during the day Accentuated lumbar lordosis Pain on straight leg raising Loss of thoracic kyphosis
Reduced lateral flexion of the lumbar spine is one of the earliest signs of ankylosing spondylitis. There tends to be a loss of lumbar lordosis and an accentuated thoracic kyphosis in patients with ankylosing spondylitis
A 54-year-old lady presents with one-month history of worsening right groin pain. The pain is worse on walking and she has developed a limp. She has a background of systemic lupus erythematosus and is on long-term hydroxychloroquine with a recent course of prednisolone for an acute flare. On examination, the patient has pain and reduced range of movement particularly on internal rotation of the right hip.
Which of the following is the most sensitive investigation to confirm the likely diagnosis?
Pelvic x-ray MRI right hip CT hip Anti-phospholipid antibodies Right hip aspiration and microscopy
MRI Right Hip
This patient has avascular necrosis of her right femoral head due to both her history of systemic lupus erythematosus and recent steroid use. The most sensitive investigation is MRI scanning. X-rays may show increased density of the femoral head in advanced cases. In practice, x-rays would usually be done first-line.
Explanations for other options:
- In practice, pelvic x-rays would usually be done first-line but they may be normal in early cases of avascular necrosis.
- CT scan of the hip is not as sensitive as MRI scanning.
- Antiphospholipid antibodies are a possible cause of avascular necrosis but would not confirm the diagnosis.
- Hip aspiration and microscopy would not be useful in diagnosing avascular necrosis. It is helpful in cases of possible septic arthritis, gout or pseudogout, none of which are suggested by this history.
