Neurol Neuroimmunol Neuroinflamm 2020 09 11;7(5). Epub 2020 Aug 11.
From the Department of Clinical Immunology and Internal Medicine (A.G., V.G, Y.D., V.P., T.M., A.-S.K.), National Reference Center for Systemic Autoimmune Diseases (CNR RESO), Tertiary Center for Primary Immunodeficiency, Strasbourg University Hospital; Université de Strasbourg (A.G., M.S., V.G., Y.D., T.M., A.-S.K.), INSERM UMR - S1109; Université de Strasbourg (A.G., M.S., Y.D., C.L., Y.H., T.M., A.-S.K.), Faculty of Medicine; Virology Laboratory (M.S.), Strasbourg University Hospital; Université de Strasbourg (V.G.), Faculty of Pharmacy, Illkirch, France; Internal Medicine and Intensive Care (C.K.), Strasbourg University Hospital; Department of Dermatology (C.L.), Strasbourg University Hospital; Department of Neurology (L.K.), INSERM U1119, Biopathologie de La Myéline, Neuroprotection et Stratégies Thérapeutiques, Université de Strasbourg, Fédération de Médecine Translationnelle de Strasbourg (FMTS); Université de Strasbourg (A.M., R.C.), INSERM UMR-S1109, GENOMAX Platform, Fédération Hospitalo-Universitaire OMICARE, Faculté de Médecine, Fédération de Médecine Translationnelle de Strasbourg (FMTS), LabEx TRANSPLANTEX; Department of Infectious Diseases (Y.H.), Strasbourg University Hospital; and Departement of Pneumology (S.H.), Strasbourg University Hospital, Strasbourg, France.
Objective: To report the association of JC virus infection of the brain (progressive multifocal encephalopathy [PML]) during the course of sarcoidosis and the challenging balance between immune reconstitution under targeted cytokine interleukin 7 (IL7) therapy for PML and immunosuppression for sarcoidosis.
Methods: Original case report including deep sequencing (whole-exome sequencing) to exclude a primary immunodeficiency (PID) and review of the literature of cases of PML and sarcoidosis.
Results: We report and discuss here a challenging case of immune reconstitution with IL7 therapy for PML in sarcoidosis in a patient without evidence for underling PID or previous immunosuppressive therapy.
Conclusions: New targeted therapies in immunology and infectiology open the doors of more specific and more specialized therapies for patients with immunodeficiencies, autoimmune diseases, or cancers. However, before instauration of these treatments, the risk of immune reconstitution inflammatory syndrome and potential exacerbation of an underlying disease must be considered. It is particularly true in case of autoimmune disease such as sarcoidosis or lupus.