Frédérique Lefrançois, Tanveer Towheed
Background Anifrolumab, an anti-interferon I receptor monoclonal antibody, is the second biologic molecule to be recognized for treatment of active systemic lupus erythematosus (SLE). Jaccoud’s arthropathy (JA) a reducible, non-erosive subtype of musculoskeletal involvement in SLE patients, and Chilblain Lupus Erythematosus (CHLE), a subtype of cutaneous involvement presenting as a spectrum of acral cold-induced lesions that can progress to painful ulcerations, are 2 rare manifestations of SLE with treatment guidelines mostly relying on clinical cases and experts’ opinion. Case Report We report a case of a patient diagnosed with coexisting SLE, CHLE requiring amputation and mutilating JA. Clinical response on all aspects remained suboptimal after multiple lines of treatment including Hydroxychloroquine, high dose Prednisone, Mycophenolate mofetil, Ustekinumab, Azathioprine, Belimumab. She showed positive clinical response to Anifrolumab, with significant reduction of arthralgia and complete resolution of chilblains lesions. This was maintained at 1 year post introduction of Anifrolumab with only complication attributable to this treatment being a cutaneous abscess treated with topical and systemic antibiotics. Following this improvement, we decided to proceed to a narrative review of literature regarding management of both CHLE and JA. Guidelines did not recommend Anifrolumab directly for any of those 2 subtypes of SLE. The line of treatment after Belimumab in the most up to date Cases of both presentations were compiled, with specific interest in patients reported to have received Anifrolumab as part of their treatment regimen. We collected 10 cases of CHLE treated with Anifrolumab. We also found 1 case of JA that demonstrated improvement of arthralgia on Anifrolumab. Most of the patients had also failed to respond to multiple lines of treatment including trial of Belimumab and/or Rituximab. All cases responded completely to Anifrolumab within 8 to 20 weeks. We did not find reports of cases followed for longer intervals. Conclusion With our case, we are participating in the growing weight of evidence that Anifrolumab is a safe and potent treatment option that should be considered in refractory cutaneous lupus, including CHLE, and potentially in refractory arthralgia, even in JA subtypes.