Adult Onset Still's Disease

What is adult onset Still's disease?

Adult-onset Still’s disease is a rare immune-mediated, multisystem inflammatory disorder indicated by the “Still’s triad” of high spiking fevers, rash and arthritis (joint pain). It is frequently underdiagnosed and one of the main reasons for hospital admissions due to fever of unknown origin. The disease characteristically affects younger adults, with three quarters of the patients reporting disease onset between 16 and 35 years of age.

This syndrome was previously thought to occur only in children and was known as "Still’s disease," so named for English physician Sir George Frederic Still (1861 to 1941) who first described the condition. Today, pediatric Still's disease is known as systemic-onset juvenile idiopathic arthritis (sJIA). In 1971, the name "adult onset Still's disease" was established for adult patients who did not meet the criteria for classic rheumatoid arthritis (RA) but who displayed features similar to those described in pediatric sJIA. There is no known cure for the disease, but there are treatments available to control the reactions and symptoms.

What are the symptoms of adult onset Still's disease?

Characteristic symptoms include daily spiking fevers, evanescent (or short term) rashes, and arthritis, each occurring in about 75 to 95 percent of patients. Patients with adult Still's disease (ASD) will be affected one of three ways (though the first two might evolve into the chronic pattern):

• Monophasic (or monocyclic): the disease course lasts weeks to months, completely resolving within a year
• Intermittent: the disease appears in unpredictable episodes or flares with remissions lasting weeks to years in between
• Chronic: persistent and active, this type of ASD is most destructive to joints

Other less common, but more serious symptoms can include myalgia, inflammatory myopathy, liver abnormalities, pleuritis, pericarditis, splenomegaly, pericardial tamponade, myocarditis, pulmonary fibrosis, pleural effusions, adult respiratory distress syndrome, interstitial nephritis, subacute glomerulitis, renal amyloidosis, collapsing glomerulopathy, thrombotic thrombocytopenic purpura, pure red cell aplasia, cranial nerve palsies, seizures, aseptic meningoencephalitis, and Miller-Fisher syndrome.

Life-threatening complications such as macrophage activation syndrome may occur and need to be diagnosed and treated promptly.

How is adult onset Still's disease diagnosed?

Diagnosis is made after:

• a thorough examination and consideration of symptoms and medical history
• the exclusion of mimickers of infectious, autoimmune or neoplastic causes
• consideration of non-specific laboratory abnormalities such as peripheral leukocytosis and elevation of serum ferritin and other acute phase reactants

What is the treatment for adult onset Still's disease?

The disease manifestations can change frequently and can include diverse complications, affecting multiple organ systems. Moreover, the severity of the organ involvement can vary considerably, representing a wide spectrum from the self-limited to severe. The mainstay of therapy has evolved from the traditional use of corticosteroids and oral immunosuppressants such as methotrexate to the newer biologic agents targeted at specific inflammatory molecules (interleukins), such as anakinra, canakinumab, and tocilizumab. These medications are given by injection or IV infusion.

Posted: 12/3/2018