Mock Rheumatology Module 2

A patient with scleroderma has normal lung volumes but a low DLCO of 40 percent (predicted). There is minimal fibrosis on HRCT. You suspect the patient may have pulmonary hypertension.

What is the next best investigation?

Right heart catheter

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Echocardiogram

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HRCT

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V/Q scan

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Lung function test

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Which of the following medications is helpful for scleroderma-associated interstitial lung disease, especially when the FVC is less than 70 percent predicted and the extent of fibrosis on HRCT is above 20 percent?

Nintedanib

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High dose prednisone

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Rituximab

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Etanercept

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Oral cyclophosphamide

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A 52-year-old woman with a 6-year history of limited cutaneous systemic sclerosis (scleroderma) attends her annual rheumatology review. She reports feeling generally well, with no new symptoms of breathlessness, chest pain, or reduced exercise tolerance.

Her scleroderma has been stable on her current immunosuppressive regimen.

As part of the routine annual screening protocol for pulmonary arterial hypertension in patients with connective tissue disease, she undergoes a transthoracic echocardiogram.

Echocardiogram findings:

Preserved left ventricular systolic function (LVEF 60%)
No significant valvular disease
Estimated systolic pulmonary artery pressure (sPAP): 56 mmHg
Tricuspid regurgitant jet velocity: 3.5 m/s
Mildly dilated right ventricle

Her observations are stable, and her physical examination is unremarkable.

What should be the next step in her management?

Proceed to right heart catheterisation even if asymptomatic

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Perform a 6-minute walk test only

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Proceed to right heart catheterisation once symptomatic.

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Repeat echocardiogram in 12 months

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No further action required

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The following treatments are useful in pulmonary arterial hypertension associated with systemic sclerosis, except:

Tadalafil

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Nifedipine (as long term monotherapy)

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Macitentan

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Epoprostenol

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Riociguat

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The following are associated with scleroderma renal crisis, except:

Glomerulonephritis

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Malignant hypertension

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Microangiopathic haemolytic anaemia

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Mild protenuria

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RNA polymerase III antibody positivity

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A 48-year-old woman with a 2-year history of diffuse cutaneous systemic sclerosis (scleroderma) presents to the emergency department with a severe headache, blurred vision, and general malaise over the past 48 hours.

She has recently noticed worsening of her skin tightening and has been started on corticosteroids by her rheumatologist for progressive disease.

On examination:

Blood pressure: 220/120 mmHg (previously normotensive)
Fundoscopy reveals bilateral retinal haemorrhages and papilloedema
No peripheral oedema

Investigations:

Haemoglobin: 92 g/L (low)
Platelets: 98 × 10⁹/L (low)
Blood film: fragmented red cells (schistocytes) present
Creatinine: 285 µmol/L (rising from a baseline of 78 µmol/L)
Urea: 18 mmol/L (elevated)
Urinalysis: mild proteinuria

What is the medication of choice for the treatment of this condition?

ACE-inhibitors

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Beta blockers

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Calcium channel blockers

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Diuretics

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Alpha blockers

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Which of the following medications is least useful in the treatment of Raynaud’s phenomenon?

Aspirin

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Nifedipine

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Topical nitroglycerine

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Fluoxetine

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Sildenafil

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Which of the following clinical features is NOT part of the classification criteria used to diagnose rheumatoid arthritis?

Presence of rheumatoid nodule.

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Number and size of involved joints

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ACPA or rheumatoid factor positivity

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Abnormal CRP or ESR

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Duration of symptoms

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All of the following joints may be affected by rheumatoid arthritis, except:

Distal interphalangeal (DIP) joints

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Elbow joint

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Proximal interphalangeal (PIP) joints

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Cervical spine

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Metacarpophalangeal (MCP) joints

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What is the typical white cell count (in cells/mm³) and neutrophil (PMN) proportion in synovial fluid from a patient with rheumatoid arthritis?

Reference synovial fluid categories:

Normal <200 cells/mm³ with <25 percent PMNs.
Inflammatory 2,000–50,000 cells/mm³
Septic typically >50,000 cells/mm³ with >75 percent PMNs.

WCC 2,000–10,000, PMNs >50 percent

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WCC <200, PMNs <25 percent

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WCC 200–2,000, PMNs <25 percent

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WCC >50,000, PMNs >75 percent

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WCC <100, PMNs <10 percent

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A 58-year-old woman with a 20-year history of seropositive, erosive rheumatoid arthritis presents to the clinic with recurrent bacterial chest infections and two episodes of cellulitis over the past 6 months.

She has been maintained on low-dose prednisone and hydroxychloroquine, having previously been intolerant of several other agents.

On examination she has established rheumatoid deformities, a palpable spleen 4 cm below the costal margin, and a healing lower-limb ulcer.

Investigations reveal:

Haemoglobin 108 g/L (reference 115–165 g/L)
White cell count 2.1 × 10⁹/L (reference 4.0–11.0 × 10⁹/L)
Absolute neutrophil count 0.7 × 10⁹/L (reference 2.0–7.5 × 10⁹/L)
Platelets 142 × 10⁹/L (reference 150–400 × 10⁹/L)
Rheumatoid factor and anti-CCP strongly positive
A blood film shows no large granular lymphocytes; bone marrow examination shows myeloid maturation arrest

Which of the following is the most appropriate treatment for her underlying condition?

Methotrexate

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Long-term broad-spectrum antibiotic prophylaxis alone

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Splenectomy as first-line therapy

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Lithium carbonate

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Escalating dose of prednisone as monotherapy

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A 45-year-old woman with a background of chronic hepatitis C infection presents with a 3-month history of pain in both hands, specifically involving the distal interphalangeal joints.

A rheumatoid factor test returns strongly positive at high titre.

What is the most likely explanation for the rheumatoid factor positivity?

The high titre is related to her hepatitis C infection

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The most likely diagnosis is rheumatoid arthritis.

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Rheumatoid factor positivity excludes systemic lupus erythematosus

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This is likely a laboratory error

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This is a normal finding for her age

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A 55-year-old woman is being investigated for joint pain and fatigue. As part of her workup, a rheumatoid factor test is requested.

Her physician explains that while rheumatoid factor is associated with rheumatoid arthritis, it lacks specificity and can be positive in a number of other conditions, including certain infections and connective tissue diseases.

Rheumatoid factor positivity can be seen in all of the following conditions, except:

Ankylosing spondylitis

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Chronic infection

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Cryoglobulinemia

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Sjogrens

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Rheumatoid arthritis

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Which of the following markers is most specific for rheumatoid arthritis?

Anti-cyclic citrullinated peptide (anti-CCP) antibodies

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Rheumatoid factor

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CRP

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ESR

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TNF-alpha

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The following are poor prognostic markers in rheumatoid arthritis, except:

HLA-DRB1*04 heterozygosity

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High rheumatoid factor and ACPA titres

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Smoking

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High CRP

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Radiographic erosions at baseline

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Which of the following radiographic features is NOT typical of rheumatoid arthritis?

Asymmetric joint involvement

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Periarticular soft tissue swelling

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Juxta-articular (periarticular) osteopenia

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Marginal erosions

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Symmetric joint space narrowing

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You are conducting an annual review of a 50-year-old woman with rheumatoid arthritis.

Which of the following complications is most likely to occur as a direct consequence of her disease?

Ischaemic heart disease

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Primary hypertension

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Non-Hodgkin lymphoma

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Colorectal cancer

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Type 2 diabetes mellitus

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Which of the following markers is most predictive of subsequent death from cardiovascular disease in patients with rheumatoid arthritis?

CRP

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ESR

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Rheumatoid factor

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Anti-CCP

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ANA

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A 42-year-old woman with rheumatoid arthritis has persistent moderate-to-high disease activity despite 6 months of methotrexate at maximally tolerated dose combined with sulfasalazine and hydroxychloroquine (triple therapy), plus low-dose prednisone.

What is the most appropriate next step in management?

Add a TNF inhibitor

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Increase prednisone to 25 mg daily long term

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Commence a prolonged course of IV methylprednisone

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Increase methotrexate above 25 mg weekly

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Add regular diclofenac

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A 52-year-old woman with rheumatoid arthritis, previously well controlled for years on methotrexate with low-dose prednisone, presents with a 2-day polyarticular flare affecting multiple joints without features of infection.

What is the most appropriate step in management?

Short course of glucocorticoids

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Add sulfasazine

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Add leflunomide

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Give IV morphine

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Add hydroxychloroquine

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Which of the following is least likely to slow the radiographic progression of rheumatoid arthritis?

Hydroxychloroquine

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Prednisone

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Methotrexate

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Sulfasalazine

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Abatacept

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A 30-year-old woman with rheumatoid arthritis has been on the same DMARD for 5 years with good symptom control. She now develops reduced visual acuity.

Which of the following is the most likely culprit drug?

Hydroxychloroquine

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Methotrexate

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Leflunomide

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Sulfasalazine

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Infliximab

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A 52-year-old man presents to the rheumatology clinic with a 4-month history of symmetrical polyarthritis affecting his hands, wrists, and feet.

He reports early morning stiffness lasting over an hour and significant functional impairment affecting his work as a carpenter.

On examination, he has synovitis of the metacarpophalangeal and proximal interphalangeal joints bilaterally.

Investigations confirm a diagnosis of rheumatoid arthritis, with a positive anti-CCP antibody, raised ESR and CRP, and no evidence of erosions on hand radiographs.

During your assessment, he discloses that he drinks approximately 50–60 units of alcohol per week and has done so for many years.

He has no known liver disease, and his baseline liver function tests are currently within normal limits. He declines input from alcohol services at this stage.

You wish to commence a disease-modifying antirheumatic drug (DMARD) to control his disease activity and prevent joint damage.

Given his significant and ongoing alcohol intake, which of the following DMARDs would be the safest choice for this patient?

Sulfasalazine

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Methotrexate

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Leflunomide

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Hydroxychloroquine

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Azathioprine

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Which one of the following cytokines is the most important therapeutic target and central mediator in the pathophysiology of rheumatoid arthritis?

Tumour necrosis factor (TNF)

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IFN-beta

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IFN-alpha

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IL-4

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IL-2

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A patient taking leflunomide for rheumatoid arthritis develops severe hepatotoxicity and myelosuppression.

What is the most appropriate management?

Stop leflunomide and give cholestyramine

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Stop leflunomide and simply monitor LFTs

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Reduce dose of leflunomide

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Switch leflunomide to methotrexate

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Add methotrexate.

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Which of the following drugs is not a tumour necrosis factor inhibitor?

Abatacept

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Infliximab

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Adalimumab

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Etanercept

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Certolizumab

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A 45-year-old woman with a 10-year history of seropositive rheumatoid arthritis attends the rheumatology clinic. Despite treatment with methotrexate and hydroxychloroquine, she continues to have active disease with persistent synovitis, elevated inflammatory markers, and progressive erosive changes on hand radiographs.

She originally moved to the UK from a country with a high TB prevalence and recalls no history of active TB or previous treatment.

The multidisciplinary team decides to escalate her treatment to a biologic agent.

As part of the pre-treatment work-up, she undergoes a comprehensive screening assessment, including a chest radiograph and an interferon-gamma release assay (IGRA).

Which of the following drugs is most strongly associated with reactivation of latent tuberculosis?

Infliximab

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Abatacept

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Methotrexate

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Leflunomide

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Prednisone

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The following are recognised adverse effects of TNF inhibitors, except:

Irreversible lupus like syndrome

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Demyelinating diseaae

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Cardiac failure

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Reactivation of latent TB

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Infusion reaction

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What is the mechanism of action of abatacept?

Interferes with T-cell co-stimulation and activation

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TNF inhbitor

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Anti-CD20 monoclonal antibody

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Monoclonal antibody against the IL-6 receptor

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IL-1 receptor antagonist

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Which of the following statements regarding the use of JAK inhibitors in the treatment of rheumatoid arthritis is correct?

JAK inhibitors are recommended as first-line therapy before any biologic DMARD

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JAK inhibitors are consistently less effective than biologic DMARDs for active rheumatoid arthritis

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Tofacitinib is usually taken at a dose of 25 mg twice daily

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JAK inhibitors are generally used only after an inadequate response or intolerance to at least one TNF inhibitor

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Peficitinib is a highly selective JAK1 inhibitor

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Mock Rheumatology Module 2

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