[This corrects the article DOI: 10.3389/fimmu.2026.1866652.].

Relapsing polychondritis (RP) is a rare immune-mediated inflammatory disorder primarily affecting cartilaginous tissues throughout the body that may involve other connective tissues. The initial manifestation is often auricular chondritis, and cutaneous presentations are relatively uncommon. Approximately 30% of RP cases have an association with autoimmune diseases, but it is incredibly rare for there to be any coexistence with inflammatory bowel disease (IBD). Sweet syndrome (SS), which is also known as acute febrile neutrophilic dermatosis, also has an association with autoimmune disorders and is considered to be an incredibly rare extraintestinal IBD manifestation. In this article, a case of a patient simultaneously presenting with auricular chondritis, ulcerative colitis (UC), and SS, which had an effective response to systemic corticosteroid therapy is reported. As far as we are aware, this is the first reported case of the coexistence of these three disorders, and it will provide valuable insights into the diagnosis and management of RP that is complicated by UC and other associated conditions.

We report the case of a 30-year-old female patient with Crohn's disease who was receiving infliximab and mesalazine therapy. She presented with left ear pain accompanied by bloody diarrhea and abdominal pain. Initial treatment with ciprofloxacin and aluminum acetate was ineffective, requiring hospitalization. She was treated with intravenous meropenem, clindamycin, and prednisolone, which led to symptom resolution after 12 days. However, 7 months later, the patient developed similar symptoms in the right ear without intestinal involvement. She was hospitalized and treated with intravenous antibiotics, followed by prednisolone treatment.

VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a UBA1-driven hemato-inflammatory disorder that mimics relapsing polychondritis, vasculitis, and myelodysplastic syndromes, often causing diagnostic delay. We describe a case presenting with relapsing polychondritis-like features and summarize, through a focused literature review, its diagnostic pathway and therapeutic implications. A 74-year-old man presented with three weeks of fever, auricular and nasal chondritis, iritis, and pulmonary infiltrates. Inflammatory markers were elevated with macrocytic anaemia. Antimicrobial therapy failed, whereas glucocorticoids produced rapid defervescence and clinical improvement. Bone-marrow examination revealed cytoplasmic vacuoles in myeloid precursors with dysplastic features. Sanger sequencing of UBA1 exon 3 on peripheral-blood DNA identified the canonical variant c.122T > C, p.(Met41Thr) as a mixed C/T chromatogram peak at codon 41. At structured 6-month follow-up the patient remained relapse-free on glucocorticoids. The genetic finding nevertheless supported the diagnosis. Diagnostic difficulty arises from clinical heterogeneity. A practical pathway emerges: recognition of relapsing polychondritis and infection presentations, evaluation of macrocytosis and cytopenias, bone-marrow examination for precursor vacuoles, UBA1 sequencing with adequate analytic sensitivity, and exclusion of mimics. Glucocorticoids are first-line; steroid-sparing options-interleukin-1 and interleukin-6 inhibitors, Janus kinase inhibitors, and allogeneic hematopoietic stem cell transplantation-are selected by phenotype, severity, and transplant eligibility. VEXAS syndrome should be suspected in older adults with refractory inflammation and unexplained cytopenias. Early recognition of marrow vacuolization and UBA1 sequencing with adequate analytic sensitivity may shorten diagnostic delay and facilitate risk-adapted treatment.

Orbital inflammation is the most common presentation of VEXAS, with any orbital structure potentially affected. Non-sight-threatening and uncomplicated anterior non-granulomatous uveitis and anterior diffuse scleritis follow in frequency, along with episcleritis. Ophthalmic involvement was significantly associated with relapsing polychondritis (p = 0.014), with an increased chance of a fatal outcome (RR 5.87, p = 0.016) and independently predicts a higher mortality rate (OR 3.72, p = 0.026). Treatment of ophthalmic involvement showed full or partial response to glucocorticosteroids alone in 66.7% of cases. Ophthalmic involvement is common in VEXAS syndrome and signals a poorer prognosis in terms of mortality, highlighting the need for close monitoring.

Relapsing polychondritis (RP) is a rare inflammatory disease characterised by recurrent inflammation of cartilaginous structures, especially of the ears, nose and respiratory tract, with a broad spectrum of systemic features. Furthermore, cardiovascular involvement is a rare but potentially life-threatening complication. We report the case of a 28-year-old woman with RP and refractory disease activity complicated by an aortic aneurysm, severe aortic regurgitation and embolic strokes. Positron emission tomography/computed tomography (PET/CT) imaging confirmed large vessel vasculitis of the aorta. Despite multiple immunosuppressive therapies, including glucocorticoids, methotrexate, cyclophosphamide and biologics targeting tumour necrosis factor-alpha (TNF-α), interleukin (IL)-1 and IL-6, high disease activity persisted. Rescue therapy with bimekizumab, a dual inhibitor of IL-17A and IL-17F, was associated with the normalisation of inflammation markers, PET/CT features and sustained clinical remission. To the best of our knowledge, this is the first report of successful treatment of RP-associated aortitis with bimekizumab, highlighting the potential role of IL-17A and IL17F in RP pathogenesis. These findings provide a foundation for further exploration of IL-17 inhibition in RP-associated vasculitis.