Medicine (Baltimore) 1984 Mar;63(2):82-91

Adult Still's disease. Evolution of a clinical syndrome and diagnosis,
treatment, and follow-up of 17 patients.

Larson EB

Adult Onet Still's Disease has evolved into a well-characterized
nosologic entity. This categorization allows physicians to place a
unifying label on the rare, puzzling patient who presents with a
systemic illness characterized by high spiking fever of unknown cause
associated with intense arthralgias or arthritis, an evanescent
erythematous macular or maculopapular rash, and other less constant
features of systemic illness including lymphadenopathy,
hepatosplenomegaly, sore throat, leukocytosis, anemia and elevated
concentration of hepatic enzymes. The diagnosis of Adult SD is
syndromic, based solely on compatible clinical findings; serologic or
other diagnostic tests do not aid in diagnosis. The diagnostic problem
presented by these patients with such severe systemic illness and the
insecurities inherent in diagnosis based solely on clinical features
make the availability of the diagnosis, Adult SD, useful in patient
care. The cause of Adult SD is unknown. Some have speculated that the
disease has features of non-necrotizing immune complex vasculitis.
Rubella infection has been reported to be associated with Adult SD, but
no clear-cut etiologic relationship has been established. Neither
rubella infection nor any other potential antigen has been identified
consistently in association with the disease. Management of patients
with the disease depends on the correct diagnosis. Diagnosis should
include recognition of the syndrome as well as exclude other possible
diseases. Control of systemic manifestations may require unusually high
doses of aspirin, indomethacin or other non-steroidal anti-inflammatory
drugs, prednisone or combinations of these drugs. Some adults appear to
require both high-dose prednisone and indomethacin to control disease
manifestations. Fortunately, systemic attacks are usually episodic;
steroid toxicity can be minimized by use of alternate day doses and
attempts to discontinue steroids between episodes. The current series
and other reports of long-term follow-up indicate that Adult SD may be
more disabling than was originally reported. At least three patterns of
recurrences occur: 1) systemic attacks with or without arthritis, 2)
pauciarticular disease, and 3) disabling deforming chronic arthritis,
which may require surgery and long-term anti-inflammatory, gold, or
cytotoxic therapy.