Monique’s fevers blasted through multiple courses of antibiotics that were prescribed empirically. We had just two laboratory clues to work with: a marked leukocytosis and repeatedly elevated liver enzymes.
About 10 days following her admission, we were provided with a third clue, a pain in her groin. Monique had trouble walking to the bathroom, and she winced when we tried to flex or rotate her right hip. In those prehistoric days prior to the advent of magnetic resonance imaging or computed tomography, advanced hip imaging consisted of simple dye-contrast X-ray arthrography. We were astonished when the surprisingly crisp silhouette images confirmed that the typically smooth articular surface of a 22-year-old femoral head had been irreparably damaged. All in a matter of a few weeks.
A needle biopsy of the hip joint yielded inflamed, but sterile, synovial tissue and fluid. We were frustrated. How could a microbe resist an impressive array of antibiotics, destroy Monique’s hip and not leave a trace of its identity behind?
Our team was running out of ideas. Then, one afternoon, as we hovered over her bedside, someone spotted a faint salmon-colored rash spreading over Monique’s torso. It seemed to coincide with her fever spike, which occurred around 5 p.m. on most days. Suddenly, everything seemed to fit. The quotidian fever spike, the faint rash, the sterile monoarthritis, the abnormal lab results and the negative microbial cultures. We now knew the truth. Monique’s fever was merely a red herring. Just as the pungent odor of this fish was used to mislead the scent hounds on a hunt, so too, did her high fever throw all of her doctors off course. We were all focused on the likely diagnosis of an infection, but in reality, her illness had little to do with microbes and more to do with how the body can mistakenly activate aberrant pathways of inflammation.
Adults Behaving Like Children
Until the 1970s, the illness named for the British physician Dr. George Still was considered to be a disease of childhood. Looking back, it’s apparent that isolated adult cases of Still’s disease had sporadically been described in some published series of patients with fevers of unknown origin.2 In parts of Europe, the condition was known as subsepsis allergica or Wissler-Fanconi syndrome. The term adult Still’s disease was first used by the eminent British rheumatologist, Eric Bywaters, MB BCh, of the Royal Postgraduate Medical School in London, England, to describe a series of 14 adult patients seen over the course of several decades.3 Whether in young children or adults, it is the quotidian spiking fever, usually peaking in the late afternoon or evening, that distinguishes Still’s disease. The absence of any significant articular complaints in some patients may mislead treating physicians. As Dr. Still presciently described:4