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Objectives: Relapsing polychondritis is a rare, multi-systemic and inflammatory condition of unknown origin. We currently lack a core set of measures to assess and follow damage in patients suffering from this condition. Our primary aim was to derive a disease-specific damage measuring tool for relapsing polychondritis, the Relapsing Polychondritis Damage Index (RPDAM).
Methods: We performed an international 4-round multicenter Delphi study during which experts were asked to rate the relevance of potential damage items for relapsing polychondritis (141 items were obtained from a literature review and 12 from expert suggestion), using a Likert Scale. The selection of items for each subsequent round was based on the median rating of each item.
Results: Twenty-four experts from 11 nationalities participated in round 1 and 22 in rounds 2, 3 and 4. From the initial 153 potential damage items, 44 items were selected during round 1, 30 items during round 2 and 16 during round 3. During round 4, we refined the index to a total of 17 items referring to ear nose and throat, eye, respiratory, cardiovascular and hematological systems as well as to treatment-related specific damage items.
Conclusion: We have developed by international consensus a scoring system to assess damage in patients with relapsing polychondritis. Following its validation, the RPDAM may contribute to improve the care of patients suffering from this rare condition as well as to standardize data collection for future clinical trials.
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http://dx.doi.org/10.1016/j.jbspin.2018.11.001 | DOI Listing |
Medicine (Baltimore)
September 2025
Department of Cardiac Surgery, Chest Hospital, Tianjin University, Tianjin, China.
Rationale: Tracheomalacia, typically seen in relapsing polychondritis,[1] is rarely reported in association with congenital heart disease (CHD). In patients with pulmonary hypoperfusion-type CHD, surgical repair results in a rapid increase in pulmonary blood flow, predisposing them to mucus retention, airway obstruction, and respiratory distress. We describe acute airway collapse in a patient with double outlet right ventricle and congenital bronchial stenosis following cardiac repair.
View Article and Find Full Text PDFClin Cosmet Investig Dermatol
August 2025
Department of Imaging, The Fifth People's Hospital of Hainan Province, Haikou, Hainan, People's Republic of China.
Relapsing polychondritis is a rare immunologic disorder that can involve all cartilage and proteoglycan-rich tissues. Clinical symptoms of relapsing polychondritis are often associated with recurrent inflammatory manifestations and functional impairment of such tissues. The disease has an insidious onset, and the first symptoms and clinical manifestations vary, making it easy to misdiagnose and miss the diagnosis.
View Article and Find Full Text PDFFront Immunol
September 2025
Respiratory and Critical Care Department, The First Hospital of China Medical University, Shenyang, China.
Background: Up to 22% of cancer patients treated with immune checkpoint inhibitors (ICIs) can experience immune-related adverse events (irAEs) that mimic rheumatic disease, such as relapsing polychondritis (RP), which is a rare autoimmune disease that mainly manifests as inflammation of airway cartilage.
Methods: We report a case of RP induced by humanized recombinant anti-PD-1 monoclonal antibody therapy (tislelizumab). 18F-Fluorodeoxyglucose positron emission tomography/ computed tomography (18F-FDG PET/CT) contributed to the diagnosis of RP, and methylprednisolone was used to effectively control its progression.
Int J Mol Sci
August 2025
Institute for Biomedical Research and Innovation (IRIB), National Research Council (CNR), 90146 Palermo, Italy.
VEXAS syndrome (Vacuoles, E1-enzyme, X-linked, Autoinflammation, and Somatic) is a recently identified late-onset autoinflammatory disorder characterized by a unique interplay between hematological and inflammatory manifestations. It results from somatic mutations in the gene, located on the short arm of the X chromosome. Initially, females were considered mere carriers, with the syndrome primarily affecting males over 50.
View Article and Find Full Text PDFMedicine (Baltimore)
August 2025
Department of Respiratory and Critical Care Medicine, Yichang Central People's Hospital, The First Clinical Medical College of China Three Gorges University, Yichang, China.
Rationale: Relapsing polychondritis is a rare autoimmune disease that commonly leads to tracheobronchial stenosis, presenting significant treatment challenges.
Patient Concerns: This case report describes a 64-year-old male with relapsing polychondritis who developed severe tracheobronchial stenosis.
Diagnoses: Computed tomography scanning revealed stenosis of the trachea and bronchi, while bronchoscopy confirmed occlusion or stenosis.