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[...]the observation period was only 5–8 months, and the long-term efficacy and side effects of TCZ after further reduction of PSL are unknown. [...]TCZ itself may induce cytopenia6 and might exacerbate the risk for cytopenia of MDS associated with VEXAS syndrome. [...]the mechanism by which UBA1 variants induce organ inflammation is not fully understood, and it is not clear whether IL-6 inhibition can suppress all the pathologies underlying VEXAS syndrome. [...]a previous report showed only a transient benefit of TCZ in four patients, with a median duration of 8 months.3 It took 30 months to diagnose VEXAS syndrome in RP16, which may be related to the accumulation of UBA1 mutant cells and failure of TCZ treatment, implying the importance of early diagnosis.

Relapsing polychondritis (RP) is an immune-mediated disorder that primarily involves the targeting of cartilaginous tissues for inflammation and destruction. Limbic encephalitis (LE) is a rare central nervous system (CNS) manifestation of RP. We report the case of a 39-year-old man who was diagnosed with RP complicated by anti-gamma-aminobutyric acid B receptor (anti-GABABR) antibody−associated LE and presented with recurrent headaches, fever, bilateral auricular swelling, scleral injection, and cognitive impairment. Laboratory tests revealed positive anti-GABABR IgG antibodies in both the serum (titer 1:100) and the cerebrospinal fluid (CSF) (titer 1:1), along with CSF lymphocytic pleocytosis. A brain MRI revealed bilateral frontal and parietal subcortical and periventricular T2-weighted fluid-attenuated inversion recovery (T2-FLAIR) hyperintensities. Immunosuppressive therapy with high-dose methylprednisolone and cyclophosphamide induced rapid symptom resolution, and no relapse occurred during a follow-up period of 1 year. This case expands the spectrum of RP-associated LE, emphasizes the necessity of neuronal autoantibody screening in RP patients with neurological symptoms, and suggests potential pathogenic links involving antigenic cross-reactivity between cartilage and neural tissues and GABAergic metabolism dysregulation.

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. 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. We also reviewed 13 publications on drug-induced RP in the context of cancer and analyzed the pathogenesis, ancillary tests, treatment, and prognosis of the cases described therein. Including our case, 14 drug-related RP cases with a tumor background were analyzed. Patients usually develop related symptoms 3-5 months after initiating medication. The primary tumor involvement sites included the hematological system (5/14, 35.71%), upper digestive tract (4/14, 28.57%), skin (2/14, 14.29%), reproductive system (2/14, 14.29%), bone (1/14,7.14%), and lung (1/ 14, 7.14%). 18F-FDG PET/CT plays a crucial role in diagnosing RP caused by PD-1 monoclonal antibodies. Early detection and the prompt administration of corticosteroids are crucial in effectively controlling the progression of RP, helping to alleviate symptoms and prevent further complications.

BackgroundDue 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.MethodsA 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.ResultsA 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.ConclusionsThe reviewed articles described classical clinical manifestations, but few articles reported data on other manifestations and comorbidities that can occur in RP. Our study provided new insights by mapping symptom evaluations, thereby enhancing the understanding of disease evolution. Understanding and characterizing RP will allow for better assistance in its diagnosis and follow-up.

Relapsing polychondritis is a rare multisystem disease involving the cartilaginous and proteoglycan rich structures. The spectrum of clinical presentations may vary from intermittent episodes of painful and often disfiguring auricular and nasal chondritis, to occasional organ or even life-threatening manifestations like airway collapse. There is lack of awareness about this disease due to its rarity. Relapsing polychondritis disease activity index has recently been validated and may help in clinical decision making and research. This article reviews the literature on this disease entity.

We conducted retrospective cohort studies of patients with relapsing polychondritis (RP) twice in 2009 and 2019, using a physician questionnaire. We compared the patients’ clinical statuses between the years. Age and gender were comparable between the two surveys. Mean disease duration was longer in 2019 survey (8.3 years) than that in 2009 survey (4.8 years, P < 0.001). The mortality rate declined in 2019 survey compared with those in 2009 survey (from 9.2 to 1.6%, P < 0.001). Incidence of airway involvement decreased in 2019 survey compared with that in 2009 survey (from 49 to 37%, P = 0.012). In 2019 survey, we found more frequent use of biological agents and immunosuppressants in patients with airway involvement. When we focused on RP patients with airway involvement, physicians in 2019 chose methotrexate and calcineurin inhibitors preferentially, compared with azathioprine and cyclophosphamide. Of note is that increased use of infliximab was observed in RP patients with airway involvement, but not in those without. Reduction of airway involvement and mortality in patients with RP was observed in 2019 survey. The reduction may associate with the frequent use of biologics including infliximab in RP patients with airway involvement.

To identify the clinical characteristics, treatment, and prognosis of relapsing polychondritis patients with airway involvement. Twenty-eight patients with relapsing polychondritis, hospitalised in the First Hospital of Shanxi Medical University between April 2011 and April 2021, were retrospectively analysed. Fifty per cent of relapsing polychondritis patients with airway involvement had a lower risk of ear and ocular involvement. Relapsing polychondritis patients with airway involvement had a longer time-to-diagnosis ( < 0.001), a poorer outcome following glucocorticoid combined with immunosuppressant treatment ( = 0.004), and a higher recurrence rate than those without airway involvement ( = 0.004). The rates of positive findings on chest computed tomography and bronchoscopy in relapsing polychondritis patients with airway involvement were 88.9 per cent and 85.7 per cent, respectively. Laryngoscopy analysis showed that 66.7 per cent of relapsing polychondritis patients had varying degrees of mucosal lesions. For relapsing polychondritis patients with airway involvement, drug treatment should be combined with local airway management.

This work is aimed to study the clinical and prognostic features of relapsing polychondritis (RP) in China. A total of 158 RP cases from 1985 to 2013 in China were included and compared with international case series in terms of clinical features, systemic involvement, differential diagnosis and prognosis. (1) The average age at the onset was 45.3 years old, the average age for initial symptoms was 14.4 months, female/male ratio was 0.7:1 and misdiagnosis rate was 47 %. (2) The incidence of arthritis was lower than that in Caucasians. The incidences of auricular chondritis (68 %: 84–95 %), ocular inflammation (44 %: 49–65 %) and renal involvement (3 %: 7–26 %) were lower, and laryngotracheal symptoms (69 %: 31–67 %), skin (46 %: 4–38 %) and neurological involvement (12 %: 2–8 %) were higher during the follow-up period. The proportion of associated autoimmune disease and systemic vasculitis were 5 and 3 %, respectively, similar to that in Japanese (4 and 2 %), but less than that in Caucasians (12–31 and 8–18 %) except the Francès’s study (7 and 3 %). The primary death cause is respiratory failure due to RP, followed by lung infections and cardiovascular events. (3) Juvenile RP (onset ≤18 years) was more severe than adults, similar to results from the Caucasians. However, Chinese juvenile RP had more severe ocular inflammation (57 %: 40–47 %), arthritis (100 %: 71–90 %), cardiovascular (14 %: 3–10 %) and skin involvement (20 %: 10–11 %) than Caucasian juvenile RP. Although sharing most of the clinical features with case series in previous literature, Chinese patients with RP have its unique characteristics.

SummaryNivolumab, a programmed death 1 blockade drug, is used in various types of cancers and can cause a unique immune-related adverse event (irAE). Relapsing polychondritis (RP) is a rare autoimmune disease that mainly involves inflammation of the auricle, nose and airway cartilage. A 72-year-old man with mandibular cancer received nivolumab after surgery for the primary lesion and radiation therapy for lung metastases. He then developed radiation pneumonitis, and prednisolone (PSL) was started. During the tapering of PSL, he developed exertional dyspnea and cough. The condition of mandibular cancer and radiation pneumonitis had not deteriorated. Fluorodeoxyglucose (FDG)-PET/CT showed a thickening of and abnormal FDG uptake in the tracheobronchial and nasal septum cartilage. These characteristic findings were not observed before nivolumab was initiated; thus, we clinically diagnosed the patient as having RP induced by nivolumab. Since the symptoms were mild, the patient’s condition was carefully managed with inhaled corticosteroids, and the RP has not progressed thus far. Physicians should be aware that RP can occur as an irAE because RP may progress to serious respiratory symptoms.

Purpose To report a rare case of relapsing polychondritis (RP) presenting initially as bilateral angle-closure glaucoma due to supraciliary effusion, highlighting the diagnostic challenges when classical features like perichondritis appear late. Methods A 54-year-old male presented with bilateral eye pain, redness, and elevated intraocular pressure. Clinically there was presence of bilateral angle closure glaucoma and peripheral choroidal detachment in left eye. Ultrasound biomicroscopy revealed bilateral supraciliary effusion. Systemic symptoms including intermittent fever, sensorineural hearing loss, nasal tenderness, and hoarseness of voice were noted. Infectious and autoimmune evaluations were inconclusive. Indocyanine green angiography excluded Vogt-Koyanagi-Harada syndrome. The patient was managed with corticosteroids and antiglaucoma medications. Later development of auricular chondritis confirmed the diagnosis of RP using Modified Michet’s criteria. Results The patient experienced recurrent bilateral angle closure and anterior uveitis despite immunosuppression. Classical signs of RP such as perichondritis and laryngotracheal involvement manifested late, allowing retrospective correlation of earlier ocular and systemic signs. The patient showed clinical improvement with high-dose corticosteroids and azathioprine. Multidisciplinary collaboration was essential in establishing the diagnosis and guiding management. Conclusion This case expands the clinical spectrum of RP by presenting with bilateral angle-closure glaucoma prior to typical cartilage involvement. It emphasizes the importance of considering RP in patients with unexplained ocular inflammation and cartilage-related systemic symptoms. Early recognition, even in the absence of overt perichondritis, is key to timely diagnosis and effective management. A multidisciplinary approach remains crucial in addressing the systemic and relapsing nature of RP.