Treatment of Relapsing Polychondritis with Tocilizumab

Wendling, Daniel; Godfrin-Valnet, Marie; Prati, Clément

doi:10.3899/jrheum.130371

Cited by 19 publications

(5 citation statements)

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“…Tocilizumab, has shown utility in patients with disease unresponsive to TNF antagonists [32]. Allogeneic and autologous hematopoietic stem cell transplantation remains a controversial subject, but may be of potential utility in refractory cases [33].…”

Section: Discussionmentioning

confidence: 99%

Relapsing polychondritis: clinical presentations, disease activity and outcomes

Sharma

Law

Bambery

et al. 2014

Orphanet J Rare Dis

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BackgroundRelapsing polychondritis is a rare disease characterised by inflammation of cartilaginous and proteoglycan rich structures. As there are only a few published single centre case series from all across the world, we describe our experience with 26 patients at a tertiary centre in north India.MethodsA retrospective study with all patients meeting Damiani and Levine’s modification of McAdam’s diagnostic criteria. Clinical details, investigations, disease activity assessment [(Relapsing Polychondritis Disease Activity Index (RPDAI)], treatment and outcomes were recorded.ResultsTen men and sixteen women (median age 45 years) met the diagnostic criteria. Auricular chondritis (96%), arthritis (54%), hearing impairment (42%), ocular (42%), dermal (26%), cardiovascular (11%) and laryngotracheal involvement (11%) characterized the clinical presentations. The median RPDAI was 31 (range 9-66). Two patients died during observation. Overall survival was 92.3% (median survival 13.5 years).ConclusionsApart from reduced laryngotracheal involvement, RP in India was clinically similar to recorded patterns elsewhere.

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Section: Discussionmentioning

confidence: 99%

Relapsing polychondritis: clinical presentations, disease activity and outcomes

Sharma

Law

Bambery

et al. 2014

Orphanet J Rare Dis

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“…RP is an orphan disease involving refractory inflammation in cartilage with no standardized treatment. Responses of RP patients to TCZ treatment have varied; in one case report, a sustained response was shown by two patients [ 159 ] and others have indicated its effectiveness [ 160 , 161 , 162 , 163 ] but one report showed it to be ineffective [ 164 ]. A French multicenter retrospective cohort study with 41 patients with RP who were exposed to 105 biologics reported the efficacy of TCZ (n = 17) as well as TNFα inhibitors (n = 60) but a low number of complete responses and concerns about the risk of infection were reported [ 165 ].…”

Section: Therapeutic Targeting Of Il-6 In Diseasesmentioning

confidence: 99%

The Two-Faced Cytokine IL-6 in Host Defense and Diseases

Narazaki

Kishimoto

2018

IJMS

169

129

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Interleukein-6 (IL-6), is produced locally from infectious or injured lesions and is delivered to the whole body via the blood stream, promptly activating the host defense system to perform diverse functions. However, excessive or sustained production of IL-6 is involved in various diseases. In diseases, the IL-6 inhibitory strategy begins with the development of the anti-IL-6 receptor antibody, tocilizumab (TCZ). This antibody has shown remarkable effects on Castleman disease, rheumatoid arthritis and juvenile idiopathic arthritis. In 2017, TCZ was proven to work effectively against giant cell arteritis, Takayasu arteritis and cytokine releasing syndrome, initiating a new era for the treatment of these diseases. In this study, the defensive functions of IL-6 and various pathological conditions are compared. Further, the diseases of which TCZ has been approved for treatment are summarized, the updated results of increasing off-label use of TCZ for various diseases are reviewed and the conditions for which IL-6 inhibition might have a beneficial role are discussed. Given the involvement of IL-6 in many pathologies, the diseases that can be improved by IL-6 inhibition will expand. However, the important role of IL-6 in host defense should always be kept in mind in clinical practice.

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“…It is also approved for use in children more than 2 years of age with the systemic form of juvenile idiopathic arthritis (JIA) , and for Castleman disease in Japan and India . Recent pilot studies have suggested that tocilizumab may have broad application for other chronic, immune‐mediated diseases such as systemic lupus erythematosus , systemic sclerosis , polymyositis , systemic vasculitis , Behçet's disease , Still's disease , Crohn's disease , relapsing polychondritis , polymyalgia rheumatica and uveitis and its associated ocular complications . It has been suggested that IL‐6 blockade may also be useful as a treatment of organ‐specific immune diseases such as acquired haemophilia A , autoimmune haemolytic anaemia , amyloid A amyloidosis and graft‐ versus ‐host disease , as well as other non‐organ specific autoimmune conditions .…”

Section: Tocilizumab: Current and Future Indicationsmentioning

confidence: 99%

Interleukin-6 blockade in ocular inflammatory diseases

Mesquida

Leszczynska

Llorenç

et al. 2014

Clinical and Experimental Immunology

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SummaryInterleukin-6 (IL-6) is a key cytokine featuring redundancy and pleiotropic activity. It plays a central role in host defence against environmental stress such as infection and injury. Dysregulated, persistent interleukin (IL)-6 production has been implicated in the development of various autoimmune, chronic inflammatory diseases and even cancers. Significant elevation of IL-6 has been found in ocular fluids derived from refractory/chronic uveitis patients. In experimental autoimmune uveitis models with IL-6 knock-out mice, IL-6 has shown to be essential for inducing inflammation. IL-6 blockade can suppress acute T helper type 17 (Th17) responses via its differentiation and, importantly, can ameliorate chronic inflammation. Tocilizumab, a recombinant humanized anti-IL-6 receptor antibody, has been shown to be effective in several autoimmune diseases, including uveitis. Herein, we discuss the basic biology of IL-6 and its role in development of autoimmune conditions, focusing particularly on non-infectious uveitis. It also provides an overview of efficacy and safety of tocilizumab therapy for ocular inflammatory diseases.

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Treatment of Relapsing Polychondritis with Tocilizumab

Cited by 19 publications

References 5 publications

Relapsing polychondritis: clinical presentations, disease activity and outcomes

Relapsing polychondritis: clinical presentations, disease activity and outcomes

The Two-Faced Cytokine IL-6 in Host Defense and Diseases

Interleukin-6 blockade in ocular inflammatory diseases

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