Adult Onset Still’s Disease in an Elderly Patient

• Published in: Pediatrics (Evanston) 2019-08, Vol.144 (2_MeetingAbstract), p.589-589
• Main Authors: Halpern, Danielle, Fagan, Mark, Duh, Erica
• Format: Article
• Language: English
• Subjects: Age composition; Arm; Arthritis; Autoimmune diseases; Bilirubin; Cell activation; Chills; Diarrhea; Differential diagnosis; Exanthema; Ferritin; Fever; Geriatrics; Hepatitis; Inflammatory diseases; Juvenile rheumatoid arthritis; Laboratories; Leukocytes (neutrophilic); Leukocytosis; Macrophages; Older people; Pain; Parvoviruses; Patients; Pediatrics; Pelvis; Penicillin; Pharyngitis; Pharynx; Plaques; Rheumatoid factor; Substance use
• ISSN: 0031-4005; 1098-4275
• DOI: 10.1542/peds.144.2MA6.589

A 67-year old female with PMH of HTN presented with daily fevers for 17 days and eight days of rash, joint pain, and diarrhea. She presented to the ED twice and was discharged with presumed viral illness. One week into her illness, the patient developed a migratory polyarthritis. The patient additionally noticed a relapsing remitting pruritic rash that spread from her right arm to her trunk, legs, and buttocks. She took unprescribed penicillin with no improvement of symptoms. She endorsed diarrhea, dizziness, chills, night sweats, and a sore throat. Patient denied any history of recurrent fevers, arthritis, morning stiffness and rash. She denies any recent travel. She denies any sick contacts, substance use or sexual activity. In the ED she was febrile to 103.7°F with HR 115 and BP 118/68. She had a rash with scattered blanchable erythematous papules and plaques (Figure 1). Patient denied any extremity weakness, joint tenderness to palpation, swelling, or warmth. Patient's left hand was limited in range of motion due to pain. Remainder of the examination was unremarkable. Laboratory investigation showed a leukocytosis of 18.8 x 10^9 / L, with 87% neutrophils, normal BMP, ESR 108, CRP 201 mg/L, LDH 580 U/L, AST 101 U/L, ALT 140 U/L with normal bilirubin levels. TSH was normal. BCx, CMV, EBV, HIV, parvovirus and a hepatitis panel were all negative. CXR was normal. CT chest abdomen pelvis showed no acute findings. Echo was normal. Ferritin was 4978 ng/mL (NR 10-120 ng/mL). ANA and rheumatoid factor were negative. The patient's history, clinical course, and laboratory results led to the diagnosis of adult onset Still's Disease (AOSD) based upon the Yamaguchi criteria (Figure 2). She was started on PO prednisone 60 mg daily on hospital day four, after which her fevers and other symptoms resolved. By a week later her ferritin had dropped to 1938 ng/mL and CRP to 28.86 mg/L. The patient was afebrile and rash-free since discharge. AOSD was first described by Eric Bywaters in 1971 in regards to 14 female patients ages 17-35 who developed evanescent rash, fever and arthritis similar to the classic juvenile chronic polyarthritis, or Still's disease. It is a multisystemic inflammatory disorder with large scale macrophage activation. Due to its nonspecific symptoms it is subject to a wide differential diagnosis. The Yamaguchi criteria were first proposed in 1992. They have been shown to be 93.5% sensitive for the detection of Still's disease. They rely upon the exclusi