Category Ranking
98%
Total Visits
921
Avg Visit Duration
2 minutes
Citations
20
Article Abstract
Objectives: The lack of effective biomarkers for relapsing polychondritis (RP) poses a significant challenge in its early diagnosis and treatment. This study aimed to identify novel autoantibodies and elucidate the pathogenesis and molecular heterogeneity of RP.
Methods: Plasma samples from 467 RP patients, 164 healthy controls (HCs), and 186 disease controls (DCs) were analysed using 2 sequential microarrays and enzyme-linked immunosorbent assay to sequentially discover, validate, and verify new autoantibodies. Machine learning and differential analysis were used to identify diagnosis-specific autoantibodies and their correlation with disease activity, recurrence, and remission.
Results: The RP group had 1344 elevated autoantibodies, discriminating RP patients from HCs. These antigenic targets were associated with pathways involving autoimmune responses, infections, and cardiovascular lesions. Two molecular subtypes characterised by distinct organ involvement and prognosis highlighted the heterogeneity of RP. Notably, 14 new autoantibodies were identified, which differentiated RP versus HCs and DCs with a sensitivity of 41% and 49.7% and a specificity of 91.7% and 90.5%, respectively. Among them, 6 autoantibodies showed better diagnostic performance and were consistently verified. Specifically, anti-C4B was positively correlated with disease activity, and increased anti-KRT16 predicted RP recurrence within 1 year. In addition, anti-C4B, anti-FNBP4, and anti-KRT10 decreased from acute attack to remission. Furthermore, the deposition of C4B protein in tracheal tissues, coupled with its reduction in plasma of RP patients, indicated that abnormal complement activation might be related to the pathological mechanism of RP.
Conclusions: The 14 autoantibodies promoted a noninvasive early detection of RP, predicted disease recurrence and provided new insights into the understanding of RP pathogenesis.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1016/j.ard.2025.06.001 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Acute stent placement for dynamic airway collapse after cardiac surgery in congenital heart disease with bronchial stenosis: A case report.
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 PDFA Case of Relapsing Polychondritis with Multisystemic Involvement.
Clin 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 PDFRelapsing polychondritis following PD-1 blockade diagnosed via 18F-FDG PET/CT and improved by steroid administration: a case report and literature review.
Front 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.
VEXAS Syndrome: Genetics, Gender Differences, Clinical Insights, Diagnostic Pitfalls, and Emerging Therapies.
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 PDFBridging silicone and metallic stents for tracheobronchial stenosis in relapsing polychondritis: A case report.
Medicine (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.