Clinical SBAs
Clinical Rheumatology: (20 questions)

Questions

• 1
  A 25-year old man with ankylosing spondylitis has been treated with NSAIDs and physio for 2 years but still has significant symptoms. He has no peripheral arthritis, but severe sacroiliitis. What is the most appropriate next treatment?
  Difficulty: Hard     Topic: Ankylosing spondylitis treatment
  a

  Leflunomide

  b

  Methotrexate

  c

  Infliximab

  d

  Indomethacin

  e

  Sulfasalazine
  Explanation: Ankylosing spondylitis is initially treated with physiotherapy and NSAIDs. Active movement helps to relieve stiffness and NSAIDs are effective in most patients at reducing inflammation. If severe symptoms are still present then anti-TNF-alpha therapy can be instigated with any of: infliximab, etanercept or adalimumab. These medications are very effective for patients with resistant disease. Methotrexate and sulfasalazine are 1st line for rheumatoid arthritis, with leflunomide being a 2nd line agent. Indomethacin is a strong NSAID and given failure of NSAID treatment, it is unlikely to have significant further impact.
• 2
  A 81-year old man presents with an enlarged skull, deafness and curved, thickened, sclerotic tibia. What is the most likely diagnosis?
  Difficulty: Easy     Topic: Enlarged skull
  a

  Osteopetrosis

  b

  Osteogenesis imperfecta

  c

  Paget's disease

  d

  Osteomalacia

  e

  Osteoporosis
  Explanation: This man has Paget's disease, a condition of increased bone turnover that results in bone enlargement, deformity and weakness. The skull, clavicle, femur and tibia (causing 'bowing') are the most commonly affected sites. Involvement of the skull can cause neural deafness, there is risk of secondary osteosarcoma and rarely it may result in high-output cardiac failure. It is treated with the bisphosphonate alendronic acid. Osteopetrosis is where there is loss of differentiation between medulla and cortex, which results in thick bones that are brittle. Osteogenesis imperfecta is range of a conditions with skeletal dysplasia that results in fragile bones that sustain multiple fractures. Osteomalacia is vitamin D deficiency causing inadequate mineralisation of bones. Osteoporosis is reduced bone density.
• 3
  A 56-year old woman presents with itchy, dry eyes and trouble chewing due a dry mouth. She also complains of multiple joint pains but no other symptoms. Which of the following are not associated with this condition?
  Difficulty: Medium     Topic: Dry eyes
  a

  Dyspareunia

  b

  Primary biliary cirrhosis and Hashimoto's thyroiditis

  c

  Parotiditis

  d

  Increased risk of non-Hodgkin's lymphoma

  e

  Positive Simmond's' test
  Explanation: This woman has primary Sjogren's syndrome (SS). It is characterised by lymphocytic infiltration of the exocrine glands, which usually presents as keratoconjunctivitis sicca and xerostomia. It can also cause: arthritis or arthralgia, dysphagia, Raynaud's, myositis, lymphadenopathy and rarely organs may be involved. SS is common in association with other connective tissue diseases (especially RA) but in the absence of these it is primary SS. Dyspareunia is pain during sex and occurs from vaginal dryness. SS is associated with PBC, autoimmune thyroid disease and autoimmune hepatitis. As it affects the salivary glands it causes inflammation, resulting in parotiditis. There is an increased risk of NHL. There is a positive Schirmer's test, which demonstrates reduced tear production. Simmond's' test is used to examine for Achilles tendon rupture by squeezing the calf.
• 4
  A 53-year old woman presents with difficulty swallowing. She has tight skin over her hands with hard, white subcutaneous deposits on her fingers. Her back and chest are unaffected. Nail capillaroscopy demonstrates dilated nail fold loops without drop-out. What is the most likely diagnosis?
  Difficulty: Medium     Topic: Dysphagia
  a

  Behcet's syndrome

  b

  Diffuse systemic sclerosis

  c

  Mixed connective tissue disease

  d

  Limited systemic sclerosis

  e

  Sjogren's syndrome
  Explanation: This woman has features from 'CREST' syndrome: calcinosis, Raynaud's syndrome, oEsophageal dysmotility, sclerodactyly and telangiectasia. CREST is part of limited systemic sclerosis, a small vessel vasculitis that causes fibrosis of blood vessels and organs. Scleroderma (fibrosis of the skin) is characteristic of systemic sclerosis; it the hands, feet and face in the limited form whereas it affects the whole body in the diffuse form. Limited sclerosis shows dilated nail folds without drop-out, whereas diffuse disease does show drop-out. Organ involvement is treated with cyclophosphamide. Behcet's causes orogenital ulceration, arthritis, thrombophlebitis and uveitis. Mixed connective tissue disease shows features of SLE, systemic sclerosis and polymyositis. Sjogren's syndrome causes dry eyes and mouth with or without systemic involvement.
• 5
  A 41-year old woman presents with swollen, painful and stiff joints in her hands and feet. She has had lost some hair recently and her skin is sensitive to the sun. Which of the following is not a diagnostic criteria for this condition?
  Difficulty: Medium     Topic: Stiff hands and feet
  a

  Psychosis

  b

  Dysphagia

  c

  Recurrent apthous ulcers

  d

  Pericardial effusions

  e

  Proteinuria
  Explanation: This woman has systemic lupus erythematosus (SLE), which is the most common systemic autoimmune connective tissue disease. There are 11 features that form part of the ACR diagnostic criteria, of which 4 are required. They are: malar rash, ANA positive, renal disorder (causing proteinuria) discoid rash, haematological abnormalities, photosensitivity, serositis (causing pericardial effusions), oral ulcers, CNS effects (causing psychosis), non-erosive arthritis and other auto-antibodies (anti-dsDNA-Ab, anti-SMA, anti-phospholipid-Ab). The 3 best markers of activity are: ESR, C3 and C4 levels (low with increased activity) and anti-dsDNA-Ab titre. Acute flares are treated with cyclophosphamide and prednisolone. Chronically, patients are maintained on a combination of NSAIDs, hydroxychloroquine and low-dose prednisolone. Dysphagia is a feature of systemic sclerosis, which causes oesophageal dysmotility.
• 6
  A 71-year old woman with a long history of back pain presents with a new, severe pain in her lumbar spine. Blood tests show: Hb 9.1g/dl, urea 10.7mmol/L. What is the most appropriate management?
  Difficulty: Easy     Topic: Acute on chronic back pain
  a

  Serum electrophoresis

  b

  Reassure and encourage movement

  c

  Isotope bone scan

  d

  Refer for physiotherapy

  e

  Lymph node biopsy
  Explanation: Despite the long history of mechanical back pain, this elderly woman's combination of pain, anaemia and renal failure must investigated to exclude myeloma. Serum electrophoresis is the best investigation as it will demonstrate a paraprotein; if >30g/L then myeloma is much more likely. Urine testing for Bence-Jones may demonstrate free light-chain in the urine. Isotope bone scans show areas of increased osteoblast activity, therefore are good for infection and metastases. However myeloma causes inhibition of osteoblast activity, which is negative on isotope bone scans. A skeletal survey is the investigation of choice to demonstrate the lytic lesions. Simple mechanical back strain is treated with reassurance and movement initially. Lymph node biopsy would be used if a lymphoma were suspected but myeloma rarely causes lymphadenopathy.
• 7
  Which of the following disease – autoantibody associations is correct?
  Difficulty: Medium     Topic: Autoantibodies
  a

  Limited systemic sclerosis - anti-Scl70 antibody

  b

  Diffuse systemic sclerosis - anti-smooth muscle antibody

  c

  Psoriatic arthritis - Anti-Fc IgG antibody

  d

  Drug-induced SLE - Anti-histone antibody

  e

  SLE - anti-mitochondrial antibody
  Explanation: There are many autoantibodies associated with rheumatological conditions. Drug-induced SLE is most commonly caused by: hydralazine, procainamide, phenytoin, isoniazid and quinidine. It is associated with anti-histone Ab in 100% of cases. Drug-induced SLE tends to remit on withdrawal of the drug. Limited systemic sclerosis is specifically associated with anti-centromere Ab and also anti-nuclear antibody (ANA). Anti-Scl70-Ab is specific for diffuse systemic, and it may also be positive for ANA and anti-Ro Ab. Psoriatic arthritis is a seronegative arthritis therefore does not have any antibody associations. An autoantibody directed against the Fc-region of IgG is known as rheumatoid factor. SLE is most specifically as, associated with anti-dsDNA-Ab but ANA is more sensitive. It can also be positive for anti-phospholipid Ab, anti-Ro, anti-La, anti-smooth muscle and anti-RNP (in mixed connective tissue disease).
• 8
  A 45-year old man with rheumatoid arthritis presents with purpuric bleeding over his arms and leg ulcers. There is splenomegaly on examination with anaemia, low WCC and low platelets. What is the most likely diagnosis?
  Difficulty: Hard     Topic: RA and purpura
  a

  Chronic lymphocytic leukaemia

  b

  Chronic myeloid leukaemia

  c

  Caplan's syndrome

  d

  Felty's syndrome

  e

  Autoimmune thrombocytopenia (ITP)
  Explanation: Felty's syndrome describes splenomegaly with leukopenia without or without thrombocytopenia occurring in rheumatoid arthritis. It complicated 1% of patient's disease whereas isolated splenomegaly is more common. It is also associated with leg ulcers, lymphadenopthy, infection and hyperpigmentation. Chronic myeloid and lymphocytic leukaemia cause raised WCC therefore are unlikely. They may also present with systemic features or symptoms of leukostasis (especially CML). Caplan's syndrome is the rare association of: rheumatoid arthritis, pulmonary nodules, coal worker's pneumoconiosis. It would present with breathlessness and peripheral blood counts are unaffected. ITP is a possible diagnosis however it rarely causes low WCC and it is not associated with splenomegaly.
• 9
  A 22-year old man presents with a swollen right knee with a small effusion. He has been well apart from a recent episode of gastroenteritis and there is no history of trauma. What is the most appropriate first-line management?
  Difficulty: Medium     Topic: Swollen knee in 22-year old
  a

  Metronidazole

  b

  Methotrexate

  c

  Ibuprofen

  d

  X-ray

  e

  Physiotherapy
  Explanation: This man has reactive arthritis as evidenced by the history of recent infection followed by a large joint lower limb monoarthritis. The best 1st line treatment is ibuprofen, which gives analgesia and helps to reduce inflammation. Treating the original infection (e.g. with metronidazole) has little impact on the arthritis. Methotrexate is a DMARD which is used 1st line in RA and it may only be considered in very severe, recurrent cases of reactive arthritis. There are no X-ray changes in reactive arthritis. This is a transient arthritis and physiotherapy will not be of any benefit.
• 10
  A 32-year old woman presents with a painful, swollen, non-erythematous left knee. She also has iritis and raised, brown plaques on her palms and soles. What is the most likely diagnosis?
  Difficulty: Medium     Topic: Swollen knee and palmar rash
  a

  Reactive arthritis

  b

  Systemic lupus erythematosus

  c

  Septic arthritis

  d

  Pseudogout

  e

  Sjogren's syndrome
  Explanation: Reactive arthritis is a non-infective spondyloarthropathy that occurs following infection. The most common precipitants are: streptococcus, Chlamydia urethritis and campylobacter dysentery. It usually presents with a lower limb mono- or oligo-arthritis. Other features include iritis, conjunctivitis, inflammatory spinal pain, circinate balanitis and mouth ulcers. The rash described is keratoderma blenorrhagica, which is also associated with reactive arthritis, but not with any of the other diagnoses listed. Pseudogout & septic arthritis cause erythema overlying the affected joint. SLE most affects small joints and Sjogren's most often presents with keratoconjunctivitis sicca or xerostomia.
• 11
  A 68-year old woman presents with fatigue, proximal muscle weakness, an oedematous purple rash on her eyelids and red papules over her knuckles. ESR and CK are markedly raised. Which is the most appropriate series of investigations?
  Difficulty: Medium     Topic: Fatigue and proximal weakness
  a

  Blood cultures

  b

  24-hour electrocardiogram

  c

  Anti-neutrophil cytoplasmic antigen (ANCA)

  d

  Mammogram, CXR, CEA and faecal occult blood

  e

  HIV, hepatitis B and C virus screen
  Explanation: This lady has presented with features of dermatomyositis: periorbital heliotrope rash, Gottron's papules on her knuckles and proximal weakness. Dermatomyositis is associated with a high risk of underlying malignancy, especially in those >65years and should be screened appropriately - history and examination will guide further investigation. There is no association with infectious causes and ANCA is negative. There is no indication for a 24hr ECG at this stage.
• 12
  A 40-year old presents with recurrent enthesitis, anterior uveitis and sacroiliitis. Which of the following is the least likely underlying diagnosis?
  Difficulty: Medium     Topic: Recurrent enthesitis
  a

  Systemic sclerosis

  b

  Ulcerative colitis

  c

  Psoriasis

  d

  Reactive arthritis

  e

  Ankylosing spondylitis
  Explanation: This man has one of the seronegative arthritidies, which main conditions are: AS, enteropathic-associated spondyloarthropathy, reactive arthritis and psoriatic arthritis. They all share certain features, including severe resistant enthesitidies, anterior uveitis, inflammatory spinal pain, asymmetrical large joint oligoarthritis and aortic regurgitation. 95% of patients are HLA-B27 positive. Systemic sclerosis is a rheumatoid factor positive small vessel vasculitis that results in organ fibrosis. The features above do not occur in systemic sclerosis.
• 13
  A 21-year old man presents with a painful, red right eye. He also describes having a stiff back, especially during the mornings. Schober’s test is positive. In the past he has suffered from severe Achilles tendonitis. Which of the following is not a complication of the most likely diagnosis?
  Difficulty: Hard     Topic: Red eye and stiff back
  a

  Cardiac conduction defects

  b

  Aortic stenosis

  c

  Spinal fracture

  d

  Amyloidosis

  e

  Apical pulmonary fibrosis
  Explanation: This man has ankylosing spondylitis (AS), which is one of the seronegative arthitidies that are characterised by HLA-B27 association and absence of rheumatoid factor. AS causes sacroiliitis, which results in back stiffness especially in the morning, typically presenting in young men. Patients develop vertebral syndesmophytes, which fuse vertebrae. Along with calcification of the spinous ligament, this results with the appearance of 'bamboo spine' on X-ray. Schober's test measurer a 15cm line, 10cm above the posterior superior iliac spine and 5cm below. Full lumbar flexion should cause over 5cm increase in length, which is absent in AS. The complications of AS are as listed above, plus iritis and aortic regurgitation, not stenosis.
• 14
  A 64-year old man presents with an acutely painful, swollen and red right knee. He is unwell, pyrexial and blood cultures are positive. He had his right knee replaced 3 years ago due to osteoarthritis. Diagnostic aspiration is performed. What is the most appropriate management?
  Difficulty: Medium     Topic: Swollen knee and pyrexia
  a

  Oral flucloxacillin

  b

  IV flucloxacillin

  c

  IV flucloxacillin and therapeutic aspiration

  d

  IV flucloxacillin, ciprofloxacin and therapeutic aspiration

  e

  Arthrocentesis, lavage and debridement plus IV flucloxacillin
  Explanation: This man has septic arthritis of a prosthetic joint. He has presented systemically unwell and blood cultures are positive. Diagnostic aspiration of a previously replaced joint should occur in theatre under full aseptic conditions. The joint needs wash-out and any necrotic areas debrided. If it is the joint space but not prosthesis that is infected then wash-out may be sufficient. If the prostheses itself is infected then it will need to be removed with primary or delayed replacement. Flucloxacillin is the 1st treatment because most septic arthritis is caused by Staph. aureus. Ciprofloxacin would be added if a Gram negative cause were found. Haemophilus influenzae is increased in children however the most common organism in children is still S. aureus.
• 15
  Which one of the following drug – side effect pairs is incorrect?
  Difficulty: Medium     Topic: DMARD side-effects 2
  a

  Methotrexate - oral ulcers

  b

  Methotrexate - thyroid dysfunction

  c

  Sulfasalazine - reduced sperm count

  d

  Leflunomide - hepatotoxicity

  e

  Etanercept - injection site reaction
  Explanation: DMARDs act to improve the outcome of rheumatoid arthritis however the drugs used often have significant side-effects. Methotrexate causes: oral ulcers, pneumonitis leading to pulmonary fibrosis, hepatitis causing cirrhosis and less commonly myelosuppression (FBC monitoring is needed). Thyroid dysfunction is not a feature. Sulfasalazine causes: low sperm count, rash and oral ulcers. Leflunomide is highly teratogenic (including for men), causes oral ulcers, diarrhoea, HTN and hepatitis. Etanercept is an antibody directed against the TNF-alpha receptor that is given by injection, it also causes TB reactivation and deterioration in heart failure.
• 16
  A 51-year old man with rheumatoid arthritis attends a regular vision acuity check. His vision has deteriorated due development of a drug-induced retinopathy, which medication is he most likely to be taking?
  Difficulty: Medium     Topic: DMARD side-effects 1
  a

  Infliximab

  b

  Gold

  c

  Ciclosporin

  d

  Azathioprine

  e

  Hydroxychloroquine
  Explanation: The main side effect of hydroxychloroquine is irreversible retinopathy therefore patients should have annual checks of visual acuity. Hydroxychloroquine, along with methotrexate and sulfasalazine, is one of the drugs most commonly used to treat rheumatoid arthritis. Infliximab is a monoclonal antibody directed against TNF-alpha, it causes reactivation of TB and worsening of heart failure. Gold is given by IM injection and can cause skin rashes and membranous nephropathy. Ciclosporin and azathioprine are immunosuppressants rarely used for RA. Their main side-effect is myelosuppression and FBC monitoring is required.
• 17
  A 74-year old man presents with 1 week of a painful swollen right knee without effusion or overlying erythema. What is the most likely diagnosis?
  Difficulty: Medium     Topic: Swollen knee without erythema
  a

  Rheumatoid arthritis

  b

  Gout

  c

  Pseudogout

  d

  Septic arthritis

  e

  Osteoarthritis
  Explanation: This elderly man has a relatively acute presentation of osteoarthritis. It normally presents as a chronic history of pain associated with movement that is worst at the end of the day and gelling after not using the joint. However it may cause mild synovitis and present with a swollen joint. Rheumatoid arthritis is very unlikely to present at this age and with a monoarthritis. All the other conditions cause acute monoarthritis however all result in effusion and erythema overlying the joint.
• 18
  A 52-year old man presents with stiff, painful wrists especially in the mornings. He is anti-cyclic citrullinated peptide positive. What is the most appropriate first treatment?
  Difficulty: Easy     Topic: Stiff wrists in morning
  a

  Paracetamol and encourage movement

  b

  Ibuprofen and encourage movement

  c

  Methotrexate

  d

  Leflunomide

  e

  Infliximab
  Explanation: This man has presented with rheumatoid arthritis. Anti-CCP is an autoantibody specifically associated with RA. As early as possible disease-modifying anti-rheumatic drugs (DMARDs) need to be started of which, methotrexate or sulfasalazine are the 1st line treatments. These 2 can be combined and hydroxychloroquine can be added. 2nd line agents include leflunomide, D-penicillamine and gold. If 2 DMARDs (including methotrexate) have failed and disease activity score is >5.1 then anti-TNF-alpha treatment can be started, which would include infliximab.
• 19
  A 48-year old woman presents with fatigue and painful, stiff hands. She has experienced 1 previous episode of scleritis. Which of the following would be least consistent with the most likely diagnosis?
  Difficulty: Easy     Topic: Fatigue and stiff hands
  a

  Pleural effusion

  b

  Pulmonary nodules

  c

  Palmar erythema

  d

  Jaccord's arthropathy

  e

  PIP swelling
  Explanation: This woman has rheumatoid arthritis; it most commonly presents in 40-50yr old females with systemic upset and small joint arthritis. Scleritis can be caused by many connective tissue diseases but RA is the most common cause. RA has many extra-articular features, including: serositis (causing pleural effusions), subcutaneous and pulmonary nodules (caused by fibrinoid necrosis), pneumonitis, neuropathy, peripheral cytopenias, Raynaud's and increased coronary artery disease. It often affects the PIP joints, but very rarely the DIP joints. Palmar erythema is associated with RA. Jaccord's arthropathy is a deforming arthritis (often causing ulnar deviation) that is reducible and occurs in SLE.
• 20
  A 70-year old man has long-standing knee osteoarthritis, which causes him such severe pain he can no longer do the shopping. He has had treatment with topical and oral NSAIDs and has had multiple intra-articular steroid injections, which give 3-4 month relief. What is the most appropriate management?
  Difficulty: Medium     Topic: OA treatment
  a

  Intra-articular triamcinolone injection

  b

  Knee replacement

  c

  Oral glucosamine

  d

  Oral slow-release morphine

  e

  Vastus medialis strengthening exercises
  Explanation: In the management of osteoarthritis, joint replacement is the definitive treatment, however conservative management must be trialled first. This man has tried all other treatment modalities, his pain is severely restricting his life and there are X-ray changes, which fulfils the criteria for joint replacement. Triamcinolone is a steroid injection, which cannot be given too often because ultimately it causes osteoporosis of the underlying bone that makes later joint replacement more difficult. Oral glucosamine has not been shown to have a significant impact on severe OA. Opiates are not appropriate analgesia for long-term conditions such as this due to the risks of dependency. Vastus medialis strengthening can be used for conditions with lateral displacement of the patella but is unlikely to help this man.