Still’s Disease: Long-term Sequellae

Still’s Disease: Long-term Sequellae

Case report

Synopsis: Systemic onset Still’s disease is associated with long-term disability.

Among the many unusual diseases that infectious disease consultants encounter in the evaluation of patients with fever of unknown origin (FUO) is Still’s disease, a rare rheumatologic condition associated with recurrent episodes of fever, evanescent rash, and often, arthritis. Patients also have leukocytosis and unusually dramatic elevations in ferritin. I just had the opportunity to see my second case of Still’s disease in three years, in an 18-year-old with fever, a brilliant confluent erythematous rash (which is somewhat atypical for this disease), severe limiting arthralgias, and a ferritin level of about 26,000 mg/dL. A clue to the diagnosis was her history of two similar episodes during the previous year, both of which spontaneously resolved, and one of which required hospitalization and was attributed to a viral syndrome.

Because these kind of patients are often referred to a rheumatologist once the diagnosis is made, I wasn’t sure what her long-term outcome might be. Well, here is the answer.

Ten patients with juvenile-onset Still’s disease (occurring before the age of 15), and eight patients with adult Still’s were followed for at least 10 years after their initial presentation.1 Both groups had similar initial manifestations and joint involvement, and equally severe long-term sequellae. All 18 patients experienced multiple, recurrent flare-ups of disease requiring large doses of corticosteroids. Patients with juvenile-onset Still’s generally had few or no sequellae until after age 15. Five of 10 (50%) of the pediatric patients subsequently developed progressive narrowing of joint spaces and articular destruction, and four required bilateral total hip replacements. Three pediatric patients developed ankylosis of the spine, including one who developed severe scoliosis. The articular changes in these patients generally occurred within 5-7 years of presentation, while most were still young adults. Two patients had severe growth retardation as a result of their bone and joint involvement.

A similar frequency (50%) of joint destruction occurred in adult-onset Still’s subjects. Joint disease occurred within 1-8 years of presentation in four of eight adult subjects, and progressed rapidly despite the administration of aggressive anti-inflammatory agents and corticosteroids. Within 10 years, three patients required five total hip replacements, and another had severe subchondral erosion of the hip and shoulder joints.

Comment by Carol A. Kemper, MD

While most of the systemic manifestations of disease responded fairly well to therapy, these data show that the joint destruction was very difficult to control in about one-half of the patients. In particular, the initial cardiopulmonary manifestations (e.g., pericarditis) of disease did not recur. However, three of the patients with adult Still’s developed amyloidosis, resulting in renal failure in two and gastrointestinal bleeding in one. Two of these three patients died as a consequence of their amyloidosis. Iatrogenic complications were also frequent, including aseptic arthritis and gastrointestinal bleeding from high-dose anti-inflammatory agents. Because the use of high-dose anti-inflammatory agents and corticosteroids did not halt the progression of joint destruction, which progressed fairly rapidly once it appeared, Cabane and colleagues propose the additional use of other agents, such as methotrexate.

Infectious disease consultants may only see a handful of Still’s disease cases in their careers, usually in the evaluation of FUO, and we are seldom involved in the long-term care of these patients once the diagnosis is made. Many of these patients do extremely poorly, and half are at risk for severe joint destruction within 10 years of presentation, irrespective of the age of onset of disease. In addition, these data suggest that amyloidosis may occur in up to one-third of patients with adult-onset Still’s, often with a fatal outcome.

Reference

1. Cabane J, et al. Comparison of long-term evolution of adult onset and juvenile onset Still’s disease, both followed up for more than 10 years. Ann Rheum Dis 1990;49:283-285.

Infectious disease consultants usually encounter Still’s disease in the evaluation of patients with:

a. leukocytosis.

b. arthritis.

c. fever of unknown origin (FUO).

d. dramatic elevations in ferritin.

The most common condition that occurred in patients with juvenile-onset Still’s disease and those with adult-onset Still’s disease is:

a. pericarditis.

b. amyloidosis.

c. iatrogenic complications.

d. joint destruction.

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