Anakinra in relapsing polychondritis: a case report and review of the literature.

Relapsing polychondritis is rare and affects non-synovial fibrocartilage. Currently, there is a paucity of treatment algorithms, especially for those with refractory disease. A middle-aged man presented with polychondritis affecting the nose, ears, joints, and larynx. Two months prior, a diagnosis of non-arteritic ischemic optic neuropathy was made. Oral prednisolone was initiated, and over the following three years, he had several flares for which the following other treatments were given: moderate dose methotrexate (elevated liver enzymes), azathioprine (gastrointestinal intolerance), mycophenolate (ineffective), tocilizumab (widespread eruption), and tofacitinib (acute diverticulitis). Further investigations were unremarkable for malignancy and vasculitis. UBA1 mutation screening was negative. Given the limited therapeutic options, methotrexate at a lower dose was re-added, but he developed acute flare with laryngeal symptoms. Anakinra was initiated, prompting a successful prednisolone wean over the following weeks and disease remission. A literature review identified 11 publications comprising 25 patients. Of the 21 patients with anakinra response documented, six (28.6%) had symptomatic improvement. In one of these patients, there was co-administration of methotrexate. In summary, anakinra may remain as an option, only for those subsets of patients in whom many of the other more efficacious treatments have been tried to provide sustained disease control.