The relapsing polychondritis damage index (RPDAM): Development of a disease-specific damage score for relapsing polychondritis

Mertz, Philippe; Belot, Alexandre; Cervera, Ricard; Chuah, Tyng Yu; Dagna, Lorenzo; Damian, Laura; Danda, Debashish; D’Cruz, David; Espinosa, Gerard; Françès, Camille; Jayne, David; Ooi, Kong Kok; Kucharz, Eugene J.; Lebovics, Robert S.; Marie, I.; Moulis, Guillaume; Peng, Stanford L.; Sharma, Aman; Suzuki, Noboru; Tanaka, Toshio; Vollenhoven, Ronald van; Sibilia, Jean; Gottenberg, Jacques Éric; Chasset, François; Arnaud, Laurent

doi:10.1016/j.jbspin.2018.11.001

Cited by 16 publications

(5 citation statements)

References 15 publications

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“…Also, we have not routinely used IV Cyclophosphamide for induction, but tended to use it when other agents have failed. Disease activity and damage scores have been developed [16,35] and are of use in documenting response to treatment; and also serve as a reminder of the various manifestations of this rare illness. Multiple biological agents have been tried, but due to the rarity of the condition, there are no randomised controlled trials in this field.…”

Section: Discussionmentioning

confidence: 99%

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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Section: Discussionmentioning

confidence: 99%

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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“…In patients with multiple remissions and relapses, the remission with the longest duration was used in the analysis of duration of remission. RP induced damage was assessed using the Relapsing Polychondritis Damage Index (RPDAM) 9 .…”

Section: Methodsmentioning

confidence: 99%

A multicenter study of long-term outcomes of relapsing polychondritis in Iran

Jafarpour,

Saberivand,

Saemi

et al. 2024

Sci Rep

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Relapsing polychondritis (RP) is a systemic immune mediated disease characterized by recurrent episodes of inflammation in various cartilage-rich areas. RP may cause extensive tissue destruction and is associated with significant morbidity and mortality. In this multicenter study, we considered the remission status and long-term outcomes of RP in patients who were followed-up in six referral rheumatology centers in Iran. Outcomes of disease was assessed by remission status and RP induced damage. A total of 29 patients with RP were examined for enrollment in the study, and 26 patients with a minimum follow-up period of 6 months were included in the RP outcome analysis. Median time to control of symptoms and sustained remission were 5 and 23 weeks, respectively. Prednisolone was discontinued in 8 (30.8%) patients and medication-free remission was achieved in 7 (23.1%) patients. Regarding the disease course, 34.6% of patients had a relapsing–remitting course, 42.3% had a monophasic course, and 23.1% had an always-active course. Despite extensive treatment with immunosuppressive medications, RP induced damage was developed in 21 (80.8%) patients. Ear deformity and osteoporosis were the most common RP induced damage. Long-term remission and medications-free remission in RP is accessible. However, RP related damage occur in majority of patients.

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“…Разработан индекс степени поражения при РПХ (Relapsing Polychondritis Damage Index, RPDAM). Он содержит 3 дополнительных респираторных пункта: постоянные деформации грудной клетки, дисфония и обструктивный синдром (гортани, трахеи и бронхов) [31,32].…”

Section: лечение легочной патологии при рпхunclassified

Recurrent polychondritis: a review of the literature and a clinical case description

Denisov¹,

Виноградова

Bakhtina

2023

Sovremennaâ revmatologiâ

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Relapsing polychondritis (RPC) is a rare disease, its diagnosis presents certain difficulties. This is due to the absence of characteristic clinical manifestations at the initial stages of the disease, late diagnosis and difficulties in selecting adequate therapy.The article presents a review of the literature on the diagnosis and treatment of RPC, as well as a clinical case with tracheobronchial tree and other organ systems involvement in the absence of classical auricular involvement.

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The relapsing polychondritis damage index (RPDAM): Development of a disease-specific damage score for relapsing polychondritis

Cited by 16 publications

References 15 publications

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

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

A multicenter study of long-term outcomes of relapsing polychondritis in Iran

Recurrent polychondritis: a review of the literature and a clinical case description

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