Rheumatology Module 3

A 35-year-old man presents to your dermatology clinic with a 6-month history of progressive muscle weakness and skin rash. The rash is violaceous in color and is distributed over his eyelids, nose, and knuckles. He also reports difficulty swallowing and a 15-pound weight loss over the past 3 months.

Physical examination reveals a heliotrope rash on his eyelids, Gottron’s papules on his knuckles, and a V-shaped rash on his chest.

In addition, you also noticed some abnormalities on his fingernails.

Which of the following fingernail manifestations would be most characteristic of dermatomyositis?

A 32-year-old male presents to the clinic complaining of recurrent painful oral ulcers, genital ulcers, and recent onset of blurred vision. On examination, you note several shallow ulcers on the buccal mucosa and scrotum. He also reports a history of episodes of painful red eyes and arthritis that affects his knees and ankles. An ophthalmologic examination confirms uveitis. There are no significant findings in his family history, and he denies any recent travel or risky sexual behaviors.

Based on the clinical presentation, what is the most likely diagnosis?

The following genes are associated with risk of developing rheumatoid arthritis, EXCEPT?

The following cells are predominantly found in RA synovial tissue except?

Which of the following cytokine is NOT of key importance in the pathogenesis of rheumatoid arthritis?

Bony erosion seen in rheumatoid arthritis is typically mediated by which cell?

The following are required to support osteoclast development in RA, except?

Which one of the following condition causes high titers of rheumatoid factor?

What is the most common cause for false positive rheumatoid factor?

An asymptomatic patient is found to have anti-CCP positivity.

What is the biggest risk factor for the development of RA in the future?

The following factors are predictors of RA severity, EXCEPT?

A 62-year-old female was diagnosed with Rheumatoid Arthritis (RA) 2 years ago with stable symptoms on oral Methotrexate 10 mg/week for the past 18 months.

She now presents with a 3-week history of progressive breathlessness and a nonproductive cough. She reports no recent infections or changes in her RA symptoms.

On examination, she is mildly tachypneic, oxygen saturation at 92% on room air, fine crackles heard at the lung bases bilaterally. No signs of active joint inflammation

Her investigations:

1. Chest X-Ray: Shows diffuse bilateral interstitial opacities.
2. High-Resolution Computed Tomography (HRCT) of the Chest: Reveals patchy areas of ground glass attenuation and small areas of consolidation, primarily in the lower lung zones.
3. Pulmonary Function Tests: Indicate a restrictive pattern with reduced diffusing capacity for carbon monoxide (DLCO).
4. Laboratory Tests:
   • Complete blood count (CBC): Normal white blood cell count.
   • Peripheral blood eosinophilia is noted.
   • Routine bacterial cultures and viral serology: Negative.
5. Bronchoalveolar Lavage (BAL): Shows lymphocytic predominance with an increase in the number of CD4 lymphocytes.

Apart from stopping her Methotrexate, which of the following intervention would be most useful in this situation?

A 45 year old woman with a background of poorly controlled rheumatoid arthritis, unresponsive to standard DMARDs is found to have latent TB on routine work up.

Her chest x-ray is unremarkable and she is clinically asymptomatic from a respiratory point of view. She is New Zealand born and as far as she is aware, has had no previous TB contact.

Her rheumatologist is planning to start her on Infliximab.

What should be done prior to treatment?

A 68-year-old man with a long-standing history of severe rheumatoid arthritis (RA) presents to the emergency department with a 3-day history of fever and rigors. He has been on a stable regimen of adalimumab, methotrexate, and prednisone for the management of his RA. His medical history is also significant for hypertension and type 2 diabetes mellitus, which are controlled with medication. He has no known drug allergies and does not smoke or consume alcohol.

On examination, his temperature is 38.7°C (101.7°F), blood pressure is 130/85 mmHg, heart rate is 102 beats per minute, and respiratory rate is 22 breaths per minute. Physical examination reveals no obvious source of infection. His joints show chronic deformative changes without signs of acute inflammation. Lung auscultation reveals fine crackles at the bases bilaterally. The rest of the examination, including a neurological assessment, is unremarkable.

Laboratory investigations show a white blood cell count of 12,000/µL with a left shift, C-reactive protein is elevated, and chest X-ray demonstrates bilateral interstitial infiltrates. The patient is hypoxic with an oxygen saturation of 92% on room air.

Given the patient’s immunosuppressed state due to his RA treatment, particularly with adalimumab, methotrexate, and prednisone, and the clinical presentation, a differential diagnosis of opportunistic infections should be considered.

Based on the clinical presentation and the patient’s background, what is the most likely causative organism?

What is the mechanism of action of Rituximab?

All of the following factors are targets for DMARDs except?

Which of the following group of patients should not be considered for biological DMARDs?

A 47-year-old man presents to the Emergency Department with a complaint of shortness of breath. He has a notable medical history of chronic sinusitis, which has been recurrent and difficult to manage. On examination, he appears fatigued and reports a recent unintentional weight loss. He also mentions occasional haemoptysis and a persistent dry cough over the past few weeks.

Initial investigations reveal the following:

• Hemoglobin (Hb): 104 g/L (indicating mild anemia)
• Platelet count: 477 x 10^9/L (thrombocytosis)
• White Cell Count (WCC): 14.3 x 10^9/L (leukocytosis)
• Erythrocyte Sedimentation Rate (ESR): 92 mm/h (significantly elevated)
• Urea: 20 mmol/L (elevated)
• Creatinine: 198 µmol/L (elevated, indicating renal impairment)
• Urine dipstick: Blood +++ (indicating haematuria)

Based on the clinical presentation and laboratory findings, which of the following is the most likely diagnosis?

Which of the following features are not typically seen in a patient with adult onset Still’s disease?

Which one of the following statements concerning discoid lupus is correct?

Which one of the following is the most common ocular manifestation of rheumatoid arthritis?

Which one of the following is least recognised as a risk factor for developing osteoporosis?

Each of the following feature may be seen in reactive arthritis, except:

Which imaging modality would be useful as a guide for muscle biopsy in dermatomyositis?

Which of the following is the most useful test for assessment of disease activity in systemic lupus erythematosus?

A 32-year-old female presents to the emergency department with a 2-week history of high-grade fevers, reaching 39.5°C, unresponsive to over-the-counter antipyretics. She reports significant fatigue, a 5 kg weight loss over the last month, and a diffuse maculopapular rash. Her medical history is notable for a recent diagnosis of adult onset Still’s disease (AOSD), for which she started receiving immunosuppressive therapy three weeks ago.

On examination, she appears acutely ill. She has marked hepatomegaly and splenomegaly, as well as generalized lymphadenopathy. Neurological examination reveals mild confusion and disorientation, which her family states is new and progressive over the past few days.

Initial laboratory tests reveal extremely elevated ferritin levels (>5000 micrograms per liter), significant transaminitis, and thrombocytopenia with a platelet count of 70,000/uL. Her white blood cell count is 12,000/uL, but her family reports that it has been higher in previous blood tests. Erythrocyte sedimentation rate (ESR) is 30 mm/hr, which is lower than expected for her degree of systemic inflammation. Serum triglycerides are elevated at 400 mg/dL.

What should be the next step in investigation?

A 56-year-old male accountant with a background history of mild asthma and occasional allergic rhinitis presents with a 4-month history of progressive, painless swelling of both parotid glands. He reports bilateral enlargement causing mild discomfort and cosmetic concern. The patient denies any tenderness in the salivary glands. He also mentions occasional episodes of dry eyes and mild difficulty in swallowing, but no significant change in vision.

He has noticed weight loss of approximately 10kg over the past 6 months without intentional dieting or exercise changes.

On examination, he was slight jaundiced. He had bilaterally enlarged, nontender parotid glands but no palpable lymphadenopathy. The rest of the examination was fairly unremarkable.

Blood test shows mildly elevated bilirubin and alkaline phosphatase, raised serum IgG4 levels of 3.2g/L (1.35g/l) with normal white cell count and hemoglobin.

Abdominal Ultrasound showed a ‘sausage-shaped’ pancreas and dilated intrahepatic bile ducts and abdominal MRI showed diffuse enlargement of the pancreas and narrowing of the distal common bile duct.

CT of the neck and chest showed bilateral enlargement of the parotid glands but with no significant lymphadenopathy.

What is the underlying diagnosis?

A 45-year-old male presents to the clinic with a 6-month history of intermittent ear pain and redness. He describes the pain as sharp and more pronounced in the right ear, with the ear appearing reddish-purple at times but sparing the earlobes.

He also reports occasional difficulty hearing and episodes of tinnitus in the affected ear. Over the last two months, he has experienced two episodes of fever and malaise, each lasting for a few days.

On examination, the right ear shows signs of inflammation but no signs of infection. He also complains of mild joint pain in his wrists and knees, which he attributes to overuse.

He denies any respiratory symptoms, eye problems, or changes in his nasal structure. However, he mentions a history of unexplained chest pain a year ago, which was not thoroughly investigated. His family history is unremarkable.

Based on the above clinical scenario, which of the following is the most likely diagnosis?

A 54-year-old Thai man was seen in the rheumatology clinic 3 weeks ago with a 2-year history of recurrent acute gout and a serum urate of 540 µmol/L (8.9 mg/dL). He has a background of hypertension and stage 3 chronic kidney disease (eGFR 52 mL/min/1.73m²). He was started on allopurinol 100 mg daily without any pre-treatment investigations.

He now presents to the emergency department with a 3-day history of fever, malaise, and a rapidly spreading painful rash. On examination, he is febrile at 38.9°C. There are widespread dusky erythematous macules with early blistering over his trunk and face, and painful erosions of the oral and conjunctival mucosa. Nikolsky’s sign is positive, with epidermal detachment affecting approximately 8% of his body surface area.

His allopurinol is stopped immediately, and he is transferred to a burns unit for supportive care.

Which of the following investigations, if performed before starting his urate-lowering therapy, would most likely have prevented this presentation?