OBJECTIVES: Relapsing polychondritis (RP) is characterised by recurrent flares, and comparative evidence for relapse prevention using biologics remains limited. We evaluated the association between biologic exposure and relapse incidence. METHODS: This single-centre retrospective cohort study (Kyoto University Hospital, 2000-2023) investigated adults with RP. Follow-up was divided into periods without biologics (No Bio), with TNF-α inhibitors (TNFi), or with IL-6 receptor inhibitor (IL-6Ri). Primary outcomes were RP relapse and hospitalised infection. Period-level incidence rate ratios were estimated using negative binomial regression with a log(person-time) offset and adjusted for prespecified covariates, and adjusted absolute event rates and rate differences were estimated by regression standardisation. RESULTS: Over 503.5 person-years, 55 patients were included (never-Bio, n = 28; ever-Bio, n = 27). Within the ever-Bio cohort, crude relapse rates were 46.9, 22.4, and 12.5 per 100 person-years during the No Bio, TNFi, and IL-6Ri periods, respectively. In adjusted models, both TNFi and IL-6Ri were associated with lower relapse rates than the No Bio period (TNFi: IRR 0.4, 95% CI 0.2-0.8; adjusted rate difference, -54.0 events per 100 person-years, 95% CI -141.5 to -5.5; IL-6Ri: IRR 0.2, 95% CI 0.1-0.4; adjusted rate difference, -68.3 events per 100 person-years, 95% CI -149.8 to -21.4). Hospitalised infection estimates were imprecise (TNFi: IRR 0.3, 95% CI 0.1-1.4; IL-6Ri: IRR 0.7, 95% CI 0.2-2.6). CONCLUSION: Biologic exposure was associated with lower relapse rates than during No Bio periods in RP, whereas estimates for hospitalised infection were imprecise.
A newsletter aborda o risco quase dobrado de câncer em pacientes com Síndrome VEXAS, destacando a importância da genotipagem de UBA1 e marcadores inflamatórios. Apresenta o novo guideline brasileiro para fibromialgia com recomendações atualizadas e analisa um ensaio clínico sobre o abatacepte em miopatias inflamatórias, que sugeriu benefício clínico em subtipos específicos como polimiosite e miopatia necrosante.
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. 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. 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. The 14 autoantibodies promoted a noninvasive early detection of RP, predicted disease recurrence and provided new insights into the understanding of RP pathogenesis.
Relapsing polychondritis (RP) is a rare autoimmune disorder primarily affecting cartilaginous structures. We aimed to characterise the clinical features and CT findings of laryngeal involvement in RP, hypothesising that specific CT patterns correlate with clinical manifestations. We retrospectively analysed 173 patients with confirmed RP. Demographic and clinical data were collected, and laryngeal, tracheal, and bronchial CT findings were reviewed. Statistical analyses identified factors associated with laryngeal involvement and airway stenosis. Notably, 66% of asymptomatic patients displayed CT evidence of airway damage, with laryngeal involvement in 41.1% (44/107), tracheal involvement in 80.9% (140/173), and bronchial involvement in 36.4% (63/107). Cricoid erosion and broadening with mucosal hyperplasia were the predominant laryngeal findings. Significant associations with laryngeal involvement included younger age at disease onset (p<0.05), longer disease duration (p<0.01) and multiorgan manifestations (p<0.01). Laryngeal involvement is common in RP patients with airway manifestations, second only to tracheal involvement. CT findings of cricoid erosion and broadening are characteristic. Vigilant clinical monitoring is recommended for RP patients with identified risk factors to facilitate early detection and management. Future research should focus on developing targeted interventions for this high-risk subgroup of RP patients.
Due to the rarity of relapsing polychondritis (RP), we described the demographic, clinical, treatment, outcomes, and comorbidities of patients with RP from our tertiary service. Additionally, a literature review was conducted. A total of 47 Brazilian patients with RP between 2000 and 2024 were analyzed. All patient data were collected from pre-parametrized and pre-standardized electronic medical records. A literature review using PubMed with "relapsing polychondritis" as the search term included 25 articles after applying the strict exclusion criteria. A total of 47 patients were evaluated. The median age was 40 (34-51) years, with a female-to-male ratio of 1.4:1, and 89.4% were of white ethnicity. The median time from symptom onset to diagnosis was 39 months and the median follow-up duration was 7 years. Ear cartilage biopsy was performed in 12.8% of cases. The clinical manifestations included auricular chondritis, arthralgia, and ocular involvement. Approximately half of the patients had hypertension and dyslipidemia, one-third had diabetes mellitus, and one-fifth had hypothyroidism. Tracheostomy and cochlear implantation were required in 12.8% and 6.4% of the patients, respectively. Disease outcomes showed that 46.8% of patients were in remission, 29.8% had active disease, and 25.5% were controlled with immunosuppressive therapy. Mortality occurred in 6.4% of the cases. In the literature review, 25 studies were analyzed, most of which originated in Asia. Studies have reported the classical manifestations of RP, such as auricular chondritis, arthritis, and ocular involvement. The median age of the patients with RP was similar across studies, averaging 46.4 years, with a predominance of female patients. A comparison with the literature showed consistency in clinical manifestations, particularly auricular chondritis and septum nasal chondritis, although few studies have explored comorbidities, disease evolution, and outcomes. The