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.

[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.