Acute Flaccid Paralysis Accompanying West Nile Meningitis Ahmed ...

A New Diagnosis of Adult Onset Still’s Disease
Graef, E, OMS III; Cormier, D, DO, DPT; Verma, P, MD; McCormack, E., MD.
Roger Williams Medical Center, Internal Medicine, Providence, RI.
Introduction: Adult Onset Still’s Disease (AOSD) is a systemic inflammatory disease of
unknown etiology with an estimated prevalence rate less than 1/100,000. The
characteristic symptom triad includes daily spiking fevers, evanescent erythematous
rash, and arthritis. Diagnosis may be delayed as it requires exclusion of infections,
neoplasms, and other autoimmune disorders.
Case: A 58 year old Caucasian woman with a history of polymyalgia rheumatica on
tapering prednisone, hypothyroidism, and type 2 diabetes mellitus was admitted after 16
hours of fever, vomiting, and non-bloody diarrhea. On admission, review of systems was
positive only for 4 days of left arm pain without recent trauma. Examination revealed vital
signs within normal limits and decreased range of motion in the left upper extremity
secondary to pain. Laboratory studies were significant for WBC 17,100/uL with 9%
bands, hemoglobin 9.2g/dL, platelets 718,000/uL, ESR 128 mm/hour, C-reactive protein
19.908 mg/L. Peripheral smear showed toxic granulation and Dohle bodies. Records
reviewed from three outside hospitalizations within the previous 6 months revealed
similar musculoskeletal complaints, hematologic abnormalities, and elevated
inflammatory markers including ferritin levels between 575- 72,000. A recent outpatient
ferritin was 27,008 ng/mL.
While hospitalized, the patient developed recurring overnight fevers ranging from
101-103.5 degrees F. Rheumatology, infectious disease, hematology/oncology, and
gastroenterology were consulted. On hospital day 4, a transient erythematous
maculopapular rash was noted on her shoulders and back. The rash recurred daily with
intermittent pruritus and variable distribution over her back, shoulders, neck, and face.
Negative workup included shoulder and elbow imaging, shoulder aspirations, blood and
urine cultures, EGD, colonoscopy, HIDA scan, and abdominal ultrasound. CT
chest/abdomen/pelvis showed diffuse lymphadenopathy. Serology was negative for HIV,
tick borne illnesses, ANA, RF, or CCP. Thyroid screen was unremarkable. Iron studies
showed serum iron 38 mcg/dL, transferrin 123 mcg/dL, ferritin >1650 ng/mL, and soluble
transferrin receptor 2.6 mg/L. PPD was negative. By hospital day 8, the negative
infectious workup in combination with her persistent symptomatology and elevated
ferritin were deemed sufficient to proceed with an AOSD diagnosis. Prednisone was
increased to 45 mg daily and after remaining afebrile for 24 hours, she was discharged.
She continues to improve in rheumatology follow up.
Discussion: This case demonstrates the extensive clinical evaluations that may be
obtained before arriving at a diagnosis of AOSD given the broad and potentially
devastating differential. Diagnosis can be complicated by the disease’s rarity, variable
presentation, and lack of a specific diagnostic test. This patient had multiple recent
hospitalizations and interim outpatient monitoring involving symptoms and signs strongly
suggestive of AOSD. Markedly elevated serum ferritin levels are a notorious, yet nonspecific,
feature of AOSD. This case emphasizes the importance of considering AOSD
when a patient has a ferritin level greater than 5 x ULN.