Refractory Adult-Onset Still’s Disease: A Case Report

When clinicians encounter patients with pyrexia of unknown cause, they are frequently faced with a difficulty and a conundrum. Several tests to discover the underlying reason may yield inconclusive findings. We report a 52-year-old man who has had an undulating fever for over ten months, which is consistent with pyrexia of unknown aetiology (PUO). It was linked to chronic hyperferritinemia, elevated C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR), leukocytosis, thrombocytopenia, hepatosplenomegaly, hypertriglyceridemia, raised fibrinogen index, and ankle joint arthritis. Markers of serology, All other tests, including septic workouts, cerebrospinal fluid, and bone marrow and trephine biopsy (BMT), were negative. Apart from slight hepatosplenomegaly, the imaging examinations (computed tomography scan of the entire body) were normal. Polyarthritis is visible on a positron-emission tomography (PET) scan. Although the BMT findings were ambiguous, the diagnosis of refractory adult-onset Still’s disease complicated by haemophagocytic lymphohistiocytosis was determined based on these data. He succumbed to the condition despite corticosteroids, disease-modifying anti-rheumatic drugs (DMARDs), and immune-modulator therapy. This example highlights the diagnostic difficulty of adult-onset Still’s disease with a wide range of symptoms.

Author (S) Details

Wahinuddin Sulaiman
Faculty of Medicine, Universiti Kuala Lumpur Royal College of Medicine Perak, Malaysia.

Aris Chandran Abdullah
Faculty of Medicine, Universiti Kuala Lumpur Royal College of Medicine Perak, Malaysia and Department of Medicine, Hospital Raja Permaisuri Bainun, Ipoh, Malaysia.

Jerome Tan Tsen Chuen
Department of Medicine, Hospital Raja Permaisuri Bainun, Ipoh, Malaysia.

Shaffie Baba
Department of Radiology, Hospital Raja Permaisuri Bainun, Ipoh, Malaysia.

Norain Karim
Faculty of Medicine, Universiti Kuala Lumpur Royal College of Medicine Perak, Malaysia and Department of Pathology, Hospital Raja Permaisuri Bainun, Ipoh, Malaysia.

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