再発性多発軟骨炎

Aortopathies in autoimmune rheumatic diseases (ARD) include a spectrum of aortic pathologies-including aortitis, aneurysms, dissections, and insufficiency-primarily caused by systemic inflammation. This comprehensive review investigates the clinical manifestations, pathophysiology, diagnostic modalities, and management strategies across various rheumatic diseases associated with aortopathies such as large vessel vasculitis (e.g., Takayasu arteritis, giant cell arteritis), connective tissue diseases (e.g., systemic lupus erythematosus, rheumatoid arthritis, ankylosing spondylitis, systemic sclerosis) and less common conditions (e.g., relapsing polychondritis, Cogan's syndrome, Behçet's disease, IgG4-related disease). Disease-specific pathophysiologic mechanisms of aortic wall inflammation and remodeling, including granulomatous and lymphoplasmacytic patterns and mixed inflammatory infiltrates, are described. Diagnostic imaging modalities-such as CTA, MRI, and PET/CT-are evaluated for their roles in detecting active inflammation, assessing structural complications, and guiding clinical decision-making. Histopathological findings provide insight into disease-specific vascular changes. Management strategies focus on the use of glucocorticoids, disease-modifying antirheumatic drugs (DMARDs), and biologics, including IL-6 and TNF-α inhibitors, with an emphasis on patient-centered approaches, multidisciplinary care, and timely surgical intervention for complications. Evidence gaps include optimal screening intervals and the role of novel biomarkers in risk stratification and in monitoring disease progression, highlighting the need for early recognition, frequent monitoring, and aggressive management of aortic involvement in rheumatic diseases to prevent life-threatening complications.

Relapsing polychondritis is a rare chronic autoimmune inflammation of the cartilage associated with life-threatening respiratory complications. Currently, no clear role of imaging modalities such as 18F-fluorodeoxyglucose (FDG) PET/computed tomography (CT) is defined in the literature. This systematic review and meta-analysis provide current evidence on the PET-positivity rate and utility in relapsing polychondritis. Prospective or retrospective studies with more than five patients of suspected relapsing polychondritis who underwent 18F-FDG PET/CT during their management and reported a PET-positivity rate were included. Low-sample-size studies describing chondritis due to other aetiologies or utilizing PET-based radiopharmaceuticals other than FDG were excluded. A systematic search using relevant keywords was conducted across four databases (PubMed, Embase, Scopus and Web of Science) to include studies up to 25 April 2025. The Joanna Briggs Institute critical appraisal tools were used for risk-of-bias analysis. Data were analysed using the R software package (v4.3.1; 2023). Out of 962 articles, three with a total of 97 patients were included. With a pooled PET-positivity rate of 94% [95% confidence interval (CI): 73-99%, I2 = 0%, P = 0.76] and a pooled baseline SUVmax of 4.0 (95% CI: 3.5-4.6, I2 = 32%, P = 0.23), 18F-FDG PET identified asymptomatic cartilage involvement in more than 25% patients and PET parameters correlated well with inflammatory markers. It had a higher positivity rate for inaccessible sites, such as peripheral airways, and was crucial in treatment monitoring. The pooled PET-positivity rate of 18F-FDG PET in relapsing polychondritis is high but requires prospective large-sample-size studies to explore the diagnostic accuracy and prognostic implications of 18F-FDG PET in relapsing polychondritis.

A 59-year-old man presented with sore throat and cough, and chest computed tomography and bronchoscopy revealed airway stenosis. Four McAdam's criteria supported the diagnosis of relapsing polychondritis. High-dose prednisolone and cyclophosphamide improved symptoms and airway lesions, highlighting the importance of early recognition and serial airway evaluation.

AIMS: To analyze the clinical characteristics and identify factors associated with cardiovascular involvement in patients with relapsing polychondritis (RP). METHODS: A total of 105 RP patients were enrolled and divided into cardiac and non-cardiac groups. Demographic, clinical, and serological data were collected and analyzed comparatively. Univariate and Firth's logistic regression analyses were performed to identify independent factors associated with cardiac involvement. Hierarchical clustering was applied to explore phenotypic patterns. RESULTS: Among the RP cohort, 29.5% (31/105) exhibited cardiovascular involvement, with a male-to-female ratio of 16:15 and a mean age of 57.87 ± 12.06 years. Manifestations included aortic root dilatation (11.4%), conduction abnormalities (8.6%), myocarditis (7.6%), pericarditis (5.7%), and valvular dysfunction (3.8%). Patients with cardiac involvement exhibited a significantly lower prevalence of ocular involvement (12.9 vs. 36.5%; P = 0.016) and a non-significant trend toward lower frequency of costochondritis (9.7 vs. 24.3%; P = 0.087). Notably, low-density lipoprotein (LDL) levels were significantly lower in the cardiac involvement group [2.60(1.00, 4.37) vs. 2.84(1.45, 5.30) mmol/L; P = 0.041]. Firth's logistic regression identified the ocular involvement (OR: 0.22, 95% CI: 0.06-0.78; P = 0.010), costochondritis (OR: 0.26, 95% CI: 0.07-0.99; P = 0.029), and LDL levels (OR: 0.51, 95% CI: 0.27-0.93; P = 0.017) as independent factors associated with cardiac involvement. Clustering analysis identified five phenotypic groups, with the "tracheobronchi-cardiac" cluster showing the lowest LDL levels and the smallest proportions of ocular and costal cartilage involvement. CONCLUSIONS: Cardiac involvement in RP is not rare and is independently associated with lower LDL levels and reduced frequencies of ocular and costal cartilage involvement. These findings may help identify RP patients at risk for cardiac manifestations and suggest potential protective roles of certain clinical features.