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Published on: September 20, 2018
Refractory Adult Onset Still's Disease
Wahinuddin Sulaiman1, Aris Chandran Abdullah2, Jerome Tan Tsen Chuen3
1Medicine, Faculty of Medicine, Universiti Kuala Lumpur Royal College of Medicine Perak.
This case study highlights a rare, fatal instance of Adult Onset Still's Disease (AOSD) complicated by hemophagocytic lymphohistiocytosis (HLH). Despite extensive treatment, the patient succumbed, underscoring the challenges in managing refractory AOSD and HLH.
Area of Science:
- Rheumatology
- Hematology
- Internal Medicine
Background:
- Pyrexia of unknown origin (FUO) presents diagnostic challenges.
- Persistent hyperferritinemia can indicate underlying inflammatory or malignant conditions.
Observation:
- A 52-year-old male presented with a 10-month history of undulating fever.
- Immunological and serological markers were negative.
- The patient exhibited persistent hyperferritinemia.
Findings:
- The patient received corticosteroids, disease-modifying anti-rheumatic agents, and immune modulators without improvement.
- A diagnosis of refractory Adult Onset Still's Disease (AOSD) complicated by hemophagocytic lymphohistiocytosis (HLH) was established.
- The patient ultimately succumbed to the illness.
Implications:
- Refractory AOSD poses a significant clinical challenge.
- HLH is a severe complication that can arise in AOSD.
- This case emphasizes the need for heightened awareness and timely diagnosis of AOSD and its complications.
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