Biologic therapy in relapsing polychondritis: navigating between options

ABSTRACT

Introduction

Relapsing polychondritis (RP) is a rare systemic inflammatory disease of unknown etiology, primarily affecting cartilaginous tissue and proteoglycan-rich structures. Clinical manifestations vary from mild symptoms to occasional organ or life-threatening complications. Treatment can be challenging and is mostly based on experience or case reports/series.

Areas covered

There is growing literature investigating the role of biologics in the management of RP. TNFα antagonists, abatacept, tocilizumab, rituximab, anakinra and tofacitinib have been prescribed in several RP patients, mainly as second-line treatment, after conventional immunosuppressive agents’ failure.

Expert opinion

Glucocorticoids represent the gold standard treatment of RP. Conventional immunosuppressants should be administered in refractory patients or when a glucocorticoid-sparing effect is needed. Biologic therapy should be used after failure of conventional treatments or in severe manifestations. TNFα inhibitors are the most prescribed biologic agent, with partial or complete response in several cases, but loss of efficacy may occur over time. Infliximab and adalimumab should be preferred among TNFα antagonists. Abatacept and tocilizumab proved to be effective as second-line biologic agents, but infections are reported with the former. Data on anakinra and rituximab are controversial, therefore they are not recommended as first-line biologic drugs. The use of JAK inhibitors is still anecdotal.

KEYWORDS:

• Relapsing polychondritis
• biological agents
• biologic therapy
• anti-tnfα
• infliximab
• rituximab
• tocilizumab
• anakinra

Article highlights

• The treatment of relapsing polychondritis (RP) could be challenging and is mostly based on experience and case reports/series.

• Glucocorticoids are the first-line treatment choice, while conventional immunosuppressive agents, such as methotrexate, could be used in relapsing patients or when a glucocorticoid-sparing effect is needed.

• TNFα inhibitors are the first-line biologics to be administered in RP patients. Infliximab and adalimumab should be preferred among the TNFα antagonists because their rapid onset of action, and higher retention rate.

• Tocilizumab is effective for almost all RP features, especially in patients with refractory RP-related aortitis.

• Abatacept may be a safe option in RP patients with high infection risk, presenting with refractory chondritis and joint symptoms.

• The use of anakinra and rituximab is controversial and these agents are not recommended as a first-line biologic drugs in treating RP refractory patients. The use of JAK inhibitors is still anecdotal.

Declaration of interest

F Schiavon has been principal investigator in a clinical trial sponsored by Roche and has received speaker’s honoraria from Roche and Novartis. A Doria has been principal investigator in clinical trials and has received speaker’s honoraria from GlaxoSmithKline, Pfizer, AstraZeneca, Celgene, Eli Lilly, Bristol-Meyers Squibb and Roche. The authors have no other 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 apart from those disclosed.

Reviewer disclosures

A reviewer on this manuscript has disclosed involvement with Eli Lilly, AbbVie, Boehringer-Ingelheim, Bristol-Myers Squibb, Gilead, Janssen-Cilag, Novartis, Otsuka, AstraZeneca, Pfizer, Biogen and Union Chimique Belge. Peer reviewers on this manuscript have no other relevant financial relationships or otherwise to disclose.