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Difficulty: MediumRheumatology and Immunology

A 28-year-old man presents to the emergency department with a 3-week history of daily spiking fevers, reaching up to 39.6°C (103.3°F), typically occurring in the late afternoon. He reports a severe sore throat, profound fatigue, and worsening pain and swelling in his bilateral wrists and knees. He notes a faint, salmon-colored rash on his trunk that seems to appear only during his febrile episodes and resolves when his temperature normalizes. He had an uncomplicated appendectomy 4 weeks ago. He is also stressed because his employer is demanding his specific medical diagnosis to approve his prolonged sick leave.

Vital signs show a temperature of 39.5°C (103.1°F), blood pressure of 120/78 mm Hg, heart rate of 110/min, and respiratory rate of 16/min. Physical examination reveals swollen, tender wrists and a macular erythematous rash on the chest. The lungs are clear to auscultation. Laboratory studies reveal:

TestResult
Leukocyte count22,000/mm322,000/\text{mm}^3 (88%88\% neutrophils)
Hemoglobin11.2 g/dL11.2 \text{ g/dL}
Erythrocyte sedimentation rate110 mm/h110 \text{ mm/h}
Serum ferritin9,200 ng/mL9,200 \text{ ng/mL}

Rheumatoid factor and antinuclear antibody (ANA) tests are negative. Blood cultures drawn 3 days ago in the outpatient clinic are sterile. Which of the following is the most appropriate next step in the management of this patient?

  1. A
    Administer broad-spectrum intravenous antibiotics
  2. B
    Contact the patient's employer directly to disclose the suspected diagnosis
  3. Initiate systemic corticosteroid therapyAnswer
  4. D
    Perform an abdominal computed tomography scan
  5. E
    Administer intravenous furosemide and obtain an echocardiogram

Answer

The correct approach is to initiate systemic corticosteroid therapy.
This patient's presentation of quotidian (daily spiking) fevers, a transient salmon-colored rash appearing at the height of the fever, polyarthritis, significant leukocytosis, and markedly elevated serum ferritin is the classic presentation of adult-onset Still's disease (AOSD). The negative rheumatoid factor, negative ANA, and sterile blood cultures are typical and help rule out other connective tissue diseases or sepsis. For moderate-to-severe presentations of AOSD, systemic corticosteroids are the mainstay of initial therapy to suppress the severe autoinflammatory response.

Step-by-Step Solution

1
Analyze the clinical presentation.
The patient is a young adult presenting with quotidian (daily spiking) fevers, an evanescent salmon-colored rash, polyarthritis, marked leukocytosis, and extreme hyperferritinemia.
Identifying these key features is essential to form a differential diagnosis.
2
Formulate the most likely diagnosis.
The constellation of symptoms and lab findings represents the classic triad for adult-onset Still's disease (AOSD).
Negative sterile blood cultures, negative ANA/RF, and a remote surgical history make infectious, autoimmune, and postoperative etiologies highly unlikely.
3
Determine the appropriate management.
Systemic corticosteroids (or high-dose NSAIDs) should be initiated.
AOSD is an autoinflammatory disorder. For moderate-to-severe systemic manifestations (debilitating arthritis, prolonged high fevers), systemic corticosteroids are the standard first-line treatment to rapidly control inflammation.

Key Concept

Adult-onset Still's disease (AOSD) is a systemic autoinflammatory condition classically presenting with quotidian fevers, a transient salmon-colored maculopapular rash, polyarthritis, marked leukocytosis, and severe hyperferritinemia. Treatment requires immunosuppression.
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Topics

Rheumatology and Immunology

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