Efficacy and safety of biologics in relapsing polychondritis: a French national multicentre study

Moulis, Guillaume; Pugnet, G.; Costédoat-Chalumeau, Nathalie; Mathian, Alexis; Leroux, Gaëlle; Boutémy, Jonathan; Espitia, O.; Bouillet, Laurence; Berthier, S.; Gaultier, J.-B.; Jeandel, Pierre-Yves; Konaté, Amadou; Mékinian, A.; Solau-Gervais, Élisabeth; Terrier, Benjamin; Wendling, Daniel; Andry, F.; Garnier, Camille; Cathébras, P.; Arnaud, Laurent; Palmaro, Aurore; Cacoub, P.; Amoura, Zahir; Piette, Jean‐Charles; Arlet, P.; Lapeyre‐Mestre, Maryse; Sailler, L.

doi:10.1136/annrheumdis-2017-212705

Cited by 46 publications

(45 citation statements)

References 13 publications

(10 reference statements)

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“…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

162

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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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

162

129

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“…We favor the long‐term use of MMF in case of severe respiratory involvement, although in France it remains off‐label for RP, as for systemic lupus erythematosus, but the drug is reimbursed by Social Security when prescribed by referral centers. Biotherapies are gradually growing in their importance as treatment for RP . We now empirically propose adalimumab to patients with predominant rheumatologic manifestations unresponsive to methotrexate.…”

mentioning

confidence: 99%

News on Relapsing Polychondritis: The Patient's Experience

Piette

Dion

Costédoat-Chalumeau

2018

Arthritis Care & Research

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“…Multiple biological agents have been tried, but due to the rarity of the condition, there are no randomised controlled trials in this field. A French national study looking at biologics in RP did not demonstrate any clear trends that would help guide use of biological agents [36].…”

Section: Discussionmentioning

confidence: 97%

Respiratory subtype of relapsing polychondritis frequently presents as difficult asthma: a descriptive study of respiratory involvement in relapsing polychondritis with 13 patients from a single UK centre

et al. 2021

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IntroductionRelapsing Polychondritis (RP) is a rare multisystem vasculitis characterised by recurrent cartilage inflammation. Respiratory involvement, of which tracheobronchomalacia (TBM) is the commonest form, is difficult to treat and is linked to increased mortality. We describe 13 patients with respiratory involvement.MethodsThis is a retrospective study of all the patients with RP at University Hospitals Coventry and Warwickshire NHS Trust (UHCW), a secondary care provider for ∼500 000. Only patients with respiratory involvement were included in this study.ResultsWe identified 13 patients who fulfilled the inclusion criteria. Most patients were identified from the “difficult asthma” clinic. TBM was seen in 11 patients, whilst 2 patients had pleural effusions which resolved with immunosuppression and 1 patient had small airways disease. CT scans (inspiratory and expiratory) and bronchoscopy findings were useful in diagnosing TBM. Pulmonary function testing revealed significant expiratory flow abnormalities. All patients were treated with corticosteroids/disease modifying anti rheumatic drugs (DMARDs) and some were given Cyclophosphamide or biological agents although the response to Cyclophosphamide (1 out of 4) or biologicals (2 out of 4) was modest in this cohort. Ambulatory continuous positive airway pressure (CPAP) ventilation was successful in 4 patients.ConclusionsRP may be overlooked in “difficult asthma” clinics with patients having TBM (not asthma) and other features of RP. Awareness of this condition is crucial to enable early diagnosis and interventions to reduce the risk of life threatening airway collapse. A number of patients respond well to DMARDs and are able to minimise corticosteroid use.

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Efficacy and safety of biologics in relapsing polychondritis: a French national multicentre study

Abstract: This study describes the efficacy of biologics for refractory RP. However, the number of complete responses was low and there were concerns about the risk of ADRs, particularly infections.

Cited by 46 publications

References 13 publications

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

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

News on Relapsing Polychondritis: The Patient's Experience

Respiratory subtype of relapsing polychondritis frequently presents as difficult asthma: a descriptive study of respiratory involvement in relapsing polychondritis with 13 patients from a single UK centre

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