Abstract Background/Aims Relapsing polychondritis (RP) is a rare, systemic inflammatory condition that primarily affects cartilaginous structures involving the ears, nose, large airways and joints. It can also affect the cardiac valves and large vessels; the aorta is the most frequently involved vessel. The presentation is variable and diagnosis is often delayed due to symptom overlap with other conditions. It can be life-threatening when the airway or cardiovascular system are affected. We present the case of an 81-year-old man whose RP diagnosis emerged after a prolonged hospital admission. This case exemplifies the importance of multidisciplinary collaboration, and careful exclusion of other conditions. Methods An 81-year-old man with a history of uveitis and multiple co-morbidities was admitted following a femoral fracture. Bilateral optic disc swelling prompted rheumatology assessment. Giant cell arteritis was excluded clinically and by temporal artery ultrasound. After initial discharge, he was re-admitted with confusion, reduced mobility, and elevated inflammatory markers. Infectious causes were investigated and treated empirically with broad-spectrum antibiotics. Persisting fever and delirium triggered repeat imaging, revealing peri-aortic changes suspicious for large vessel vasculitis (LVV). The rheumatology team were consulted. Key findings included uveitis, new confusion and erythema with structural changes to the right ear which had developed over several months before the fracture. Differential diagnoses included RP, VEXAS syndrome, cerebral vasculitis, and other autoimmune conditions. Investigations identified an elevated dsDNA at 53IU/ml. Screening for infectious causes, including syphilis, were unremarkable. Referrals were made to neurology, dermatology and haematology given the complexity of the case. Dermatology arranged an auricular biopsy, and haematology initiated genetic testing for VEXAS and conducted a bone marrow biopsy. Results PET-CT confirmed active inflammation of the ascending aorta consistent with LVV. Auricular biopsy demonstrated chronic inflammation with hyper-eosinophilic chondrocytes, supporting RP diagnosis. Genetic and infectious workups were negative. Treatment comprised intravenous methylprednisolone (100 mg daily for 3 days), followed by oral prednisolone (60 mg daily) and introduction of mycophenolate mofetil as a steroid-sparing agent considering the patient’s frailty. This regimen led to rapid cognitive and systemic improvement, resolution of delirium, and gradual recovery in mobility. The prolonged hospital stay of four months was complicated by hospital-acquired infections, including COVID-19 and RSV, but coordinated multidisciplinary care enabled successful discharge and ongoing rehabilitation. Conclusion This case underscores the complexity of diagnosing RP with LVV in older patients with multiple comorbidities and nonspecific symptoms. Auricular changes were a key clinical clue amid diagnostic uncertainty. Advanced imaging, particularly PET-CT, was essential in identifying life-threatening aortic involvement. Early, tailored immunosuppressive therapy facilitated significant clinical recovery despite frailty and infection risks. Multidisciplinary collaboration remains crucial in managing complex autoimmune vasculitis presentations. Further research is needed to optimize immunosuppressive strategies in frail patients with RP and large-vessel involvement. Disclosure Z. M. Iftikhar: None. K. Dasigan: None. H. Butt: None. J. Stanway: None. S. Kerr: None. A. Mustafa: None. N. Thalayasingam: None.
Iftikhar et al. (Wed,) studied this question.