Alvin Cheah is a Junior Medical Officer currently working in Manning Base Hospital, New South Wales. He obtained his MBBS degree from Monash University. His interests include gastroenterology, cardiology and medical oncology.
Fever, Rash And Aches That Wont Go Away
A 76-year-old female represented to hospital with a 3-week history of daily high grade fevers with late evening peaks, night sweats, polyarthralgia, myalgia, sore throat, back pain and weight loss. This was on a background of well-controlled essential hypertension and stable chronic kidney disease. Physical examination revealed a pink macular rash over her back. Initial investigations identified neutrophilic leucocytosis, elevated Erythrocyte Sedimentation Rate(ESR) and high C-Reactive Protein(CRP) levels. Urine culture was positive for Eschericha coli(E.coli). Despite appropriate antibiotic treatment, her fevers and arthralgia persisted. She subsequently underwent further investigations to exclude underlying infections, malignancy and rheumatic disorders. Blood cultures were negative for bacterial growth. However, she surprisingly had markedly elevated ferritin levels (>40,000 ng/ml) and abnormal liver enzymes. Hepatitis B and Hepatitis C serologies were negative. Autoantibody testing for Anti-Nuclear Antibody(ANA), Anti-double stranded DNA antibody(anti-dsDNA), Anti-neutrophil cytoplasmic antibodies(ANCA), Extractable Nuclear Antigen(ENA) Antibody, Rheumatoid Factor (RF) and Anti-cyclic citrullinated peptide(anti-CCP) Antibody were also negative. Abdominal imaging was unremarkable. Based on the Yamaguchi criteria, she met the diagnosis for Adult Onset Still’s Disease(AOSD). Following treatment with prednisolone and Nonsteroidal Anti-Inflammatory Drugs(NSAIDs), her fevers and arthralgia improved together with her biochemical markers.
AOSD is a systemic inflammatory disorder of unknown aetiology. There are no specific tests available. The diagnosis is based on the Yamaguchi criteria and our patient met three minor and four major criteria for AOSD. Patients present with a clinical triad of fever, rash and arthritis, along with biochemical triad of leucocytosis, elevated ferritin levels and deranged liver enzymes. The treatment options include NSAIDs, steroids, Disease-modifying antirheumatic drugs(DMARDs) and biological agents.
Conclusion and clinical practice point:
Patients with AOSD present with diverse clinical manifestations. It is the most common rheumatological cause for fevers of unknown origin. Being rare, a high index of suspicion is required for early recognition and prompt treatment.