Atypical Progressive Multifocal Leukoencephalopathy in a Patient with Antisynthetase Syndrome

Abstract

Antisynthetase syndrome is a disorder belonging to the dermatomyositis/polymyositis group, with high rates of morbidity and mortality. We herein present the case of a 71-year-old man who was diagnosed with antisynthetase syndrome and treated with rituximab. Almost three years later, the patient showed right-sided hemiparesis that ultimately progressed to complete hemiplegia and advancing cognitive deterioration with a poor clinical outcome. The neuropathological diagnosis was progressive multifocal leukoencephalopathy. Treatment with rituximab for antisynthetase syndrome itself plays a fundamental role in the development of infectious complications.

The use of rituximab in idiopathic inflammatory myopathies: description of a monocentric cohort and review of the literature

“…A higher prevalence of these AEs in IIM patients was also reported by other authors; in particular, infusion reactions (35,39,41) and infections have been correlated to treatment with RTX. Although infections during treatment with RTX in IIM patients were usually mild (39), rare cases of severe AEs have been reported including viral and bacterial infections (41,42) and few cases of multifocal leukoencephalopathy (43,44).…”

Autoimmune Myopathies: Updates on Evaluation and Treatment

“…Our practice is to recommend prophylaxis to patients receiving a daily dose of prednisone greater than 20 mg/day together with a second immunosuppressive agent. Finally, progressive multifocal leukoencephalopathy (PML) due to reactivation of the JC virus has been reported in myositis patients during treatment with rituximab, azathioprine, mycophenolate, cyclosporine, and cyclophosphamide and IVIG [163][164][165]. The incremental risk associated with any one agent remains uncertain in this population, as many cases occurred in patients treated with multiple agents and PML has also been reported in myositis patients on prednisone monotherapy and even without immunosuppressive therapy [163].…”

Rituximab in the Treatment of Interstitial Lung Disease Associated with Antisynthetase Syndrome: A Multicenter Retrospective Case Review

“…[20,21,28–36] Adverse events mainly included infectious complications, such as P. jirovecii pneumonia, which were occasionally fatal, as well as rash, arrhythmia, and serum sickness. [21,30,31,33,34,37]…”