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Relapsing polychondritis (RP) is a rare systemic inflammatory disease primarily affecting the ears, nose and tracheobronchial tree cartilage, but also the cardiovascular system. Cardiovascular complications are the second cause of mortality in RP. We report the case of a woman with a corticosteroid-resistant RP-associated aortitis, who was successfully treated with tocilizumab (TCZ). The FDG-PET/CT was a useful tool for diagnosing aortitis and assessing the effect of biotherapy. We conducted a systematic literature review confirming this is the first case of rapid and sustained remission in a patient with corticosteroid-resistant RP-associated aortitis after TCZ treatment administered as a first-line immunotherapy. However, further studies are needed to confirm the beneficial effect of TCZ used in this life-threatening condition.

To assess the usefulness of FDG-PET/CT as a potential diagnostic tool for detecting underlying systemic diseases (SD) in patients with orbital inflammatory disorders (OID). All consecutive patients managed for new-onset OID between 2011 and 2018 in a tertiary referral center, who underwent FDG-PET/CT as part of the etiological diagnostic workup were evaluated. To quantify the incremental value of FDG-PET/CT over standard diagnostic workup, the Net Reclassification Index (NRI) and Integrated Discrimination Index (IDI) were used. Among the 22 patients enrolled, 11 (50%) had a positive FDG-PET/CT. After clinicobiological evaluation, FDG-PET/CT correctly reclassified 4(29%) of 14 patients with SD (p =.04) and 1(13%) of 8 with idiopathic orbital inflammation syndrome (p =.32). NRI and IDI were 0.41 ± 0.17 (p =.03) and 0.38 ± 0.08 (p <.001), respectively. FDG-PET/CT successfully detected asymptomatic lesions in all (n = 4) patients with lymphoma. FDG-PET/CT enabled accurate reclassification of more than one-quarter of patients with SD, especially extraorbital lymphomas. [ABSTRACT FROM AUTHOR]