Background Relapsing polychondritis (RP) is a rare autoimmune disorder characterized by recurrent inflammation of cartilage and connective tissue.1 The pathogenesis revolves around an immune reaction to type II collagen, which is abundant in cartilage and sclera.1 Ocular involvement occurs in up to 65% of patients2 and often manifests as scleritis, a potentially eye-threatening condition characterized by the infiltration of sclera by inflammatory cells. Prompt immunosuppressive therapy is needed to avoid debilitating sequelae. Topical tacrolimus showed promising results in the treatment of several ocular inflammatory conditions,3 but the efficacy of oral tacrolimus in the treatment of scleritis is undetermined.
Objectives To describe the efficacy of oral tacrolimus in 2 patients with RP and active resistant scleritis.
Methods Two patients with RP and active scleritis in spite of previous treatments with steroids, methotrexate, and systemic cyclosporineA (discontinued due to side effects), were administered tacrolimus (0.08-0.1 mg/kg daily, 2.5mg twice daily) for one year. The efficacy of the treatment was assessed by periodic immuno-ophtalmic examinations.
Results A complete, sustained resolution of the ocular inflammation was observed in both cases shortly after tacrolimus initiation. Corticosteroids were tapered and discontinued without the appearance of disease flares. In the following year, patients remained on tacrolimus, and experienced no recurrence of symptoms.
Conclusions Scleritis is a chronic, progressive inflammation associated with several autoimmune and vasculitic diseases. Immunosuppressive therapy is needed to avoid ocular complications potentially leading to visual impairment or visual loss. Tacrolimus is an immunosuppressive drug which has an analogous mechanism of action to cyclosporinA (CyA): both inhibit calcineurin and dampen signal transduction downstream of the T-cell receptor, thus inhibiting the transcription of IL-2.4 CyA shows moderate efficacy in the management of ocular inflammation.5 In our 2 patients with refractory scleritis, we opted for a treatment with oral tacrolimus, also based on the encouraging results of tacrolimus ointment in ocular inflammation.3
In both patients, we observed a complete, sustained, relapse-free resolution of the ocular inflammation. Although this observation needs to be confirmed in larger studies, oral tacrolimus seems to be effective for refractory ocular inflammation and scleritis associated with active RP.
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Liu J, et al. Calcineurin is a common target of cyclophilin-cyclosporin A and FKBP-FK506 complexes. Cell 1991;66:807–15.
Kaçmaz RO, et al. Cyclosporine for ocular inflammatory diseases. Ophthalmology 2010;117:576-584.
Disclosure of Interest None Declared