The latest advances in the use of biological DMARDs to treat Still’s disease

ABSTRACT

Introduction

Currently, the therapeutic management of Still’s disease, a multisystemic inflammatory rare disorder, is directed to target the inflammatory symptoms and signs of patients. The treatment varies from glucocorticoids to disease-modifying antirheumatic drugs (DMARDs), both conventional synthetic and biological (bDMARDs). Usually, in refractory patients, bDMARDs are administered.

Areas covered

Among bDMARDs, IL-1 and IL-6 inhibitors are frequently used, as data reported from both clinical trials and ‘real-life’ experiences. Recently, innovative therapeutic strategies have suggested an early administration of bDMARDs to increase the rate of clinical response and drug-free remission. Some new targets have been also proposed targeting IL-18, IFN-γ, and JAK/STAT pathway, which could be applied to Still’s disease and its life-threatening evolution.

Expert opinion

Many lines of evidence improved the knowledge about the therapeutic management of Still’s disease with bDMARDs. However, many unmet needs may be still highlighted which could provide the basis to arrange further specific research in increasing the rate of clinical response. In fact, Still’s disease remains a highly heterogeneous disease suggesting possible diverse underlying pathogenic mechanisms, at least partially, and consequent different therapeutic strategies. A better patient stratification may help in arranging specific studies to improve the long-term outcome of Still’s disease.

KEYWORDS:

• Still’s disease
• systemic juvenile idiopathic arthritis
• adult-onset Still’s disease
• therapy
• biologic DMARDs
• precision medicine

Article highlights

• The treatment of Still’s disease varies from glucocorticoids to disease-modifying antirheumatic drugs (DMARDs), both conventional synthetic and biological (bDMARDs). Usually, in refractory patients, bDMARDs are administered.

• IL-1 and IL-6 inhibitors are the most frequently used bDMARDs administered in patients with Still’s disease, supported by data reported from both clinical trials and ‘real-life’ experiences.

• Recently, innovative therapeutic strategies have suggested an early administration of bDMARDs to increase the rate of clinical response and drug-free remission over time, possibly modifying the disease course.

• Some new targets have been also proposed targeting IL-18, IFN-γ, and JAK/STAT pathway, which could be applied to Still’s disease and its life-threatening evolution.

• Many lines of evidence improved the knowledge about the therapeutic management of Still’s disease with bDMARDs, but several unmet needs are still highlighted which could provide the basis to arrange further specific research in increasing the rate of clinical response improving the long-term outcomes of these patients.

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.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Author contributions

Both authors made substantial contributions to the conception or design of the work, the acquisition and interpretation of data. Both authors contributed to the critical review and revision of the manuscript and approved the final version. Both authors agreed to be accountable for all aspects of the work.

Additional information

Funding

This paper was not funded.