An update: current and prospective treatments for adult-onset Still’s disease

ABSTRACT

Introduction: Adult-onset Still’s disease (AOSD) is an uncommon systemic inflammatory disease characterized by a spiking fever, an evanescent skin rash, polyarthralgia, and hepatosplenomegaly. Although most patients have a benign course, severe manifestations such as macrophage activation syndrome and fulminant hepatitis may occur. These complications could be potentially life-threatening. Therefore, appropriate diagnosis and measurement of disease activity is necessary for the successful treatment of AOSD.

Areas covered: Since the disease is very rare, a clinical randomized study cannot be conducted on AOSD. Nevertheless, physicians should be aware of the treatment for AOSD, as the disease can be associated with serious complications and cause mortality. Therefore, here, we aim to provide the appropriate management of patients with AOSD.

Expert opinion: Several conventional disease-modifying anti-rheumatic drugs and biologics have been used for patients with AOSD in case series or by observational study. The initial treatment is administration of a corticosteroid for controlling systemic manifestations in AOSD. Methotrexate, cyclosporine, and biologic agents, such as IL-1 antagonists, IL-6 receptor blockers, and TNF blockers, could be considered for treating patients with AOSD refractory to corticosteroids. Ongoing research on the pathogenesis of AOSD can assist in new drug development through the use of new powerful technologies.

KEYWORDS:

• Adult-onset Still’s disease
• biologic agent
• corticosteroid
• disease modifying anti-rheumatic drug
• treatment

Article highlights

• Adult-onset Still’s disease (AOSD) is an uncommon systemic inflammatory disease characterized by a high spiking fever, sore throat, an evanescent skin rash, polyarthralgia, lymphadenopathy, and hepatosplenomegaly.

• The most commonly used laboratory markers for evaluating disease activity for AOSD have included erythrocyte sedimentation rate, C-reactive protein, and ferritin.

• AOSD disease activity could be evaluated according to Pouchot et al.’s method, which assigns a score from 0 to 12.

• The initial treatment is a corticosteroid for controlling systemic manifestations in AOSD, and methotrexate and cyclosporine are effective drugs in the treatment of both systemic manifestations and chronic articular involvement.

• Biologic agents could be considered for treatment of patients with AOSD refractory to corticosteroids and conventional disease modifying anti-rheumatic drugs.

• Interleukin-1 (IL-1) antagonists and IL-6 receptor blockers seem to be effective for treating systemic manifestations. Additionally, IL-6 receptor blockers and tumor necrosis factor blockers may be more effective in treating the chronic articular pattern of AOSD.

This box summarizes key points contained in the article.

Declaration of interest

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Additional information

Funding

This article was supported by a grant of the Korea Health Technology R&D Project through the Korea Health Industry Development Institute, funded by the Ministry of Health & Welfare, Republic of Korea (grant number: HI16C0992).