title: New onset of myasthenia gravis after treatment of systemic sclerosis by autologous hematopoietic stem cell transplantation: sustained autoimmunity or inadequate reset of tolerance?
abstract: Autologous hematopoietic stem-cell transplantation (HSCT) showed promising results for the treatment of primary severe autoimmune diseases (ADs). In this context, development of secondary AD after HSCT has exceptionally been observed, further questioning the roles of patient propensity for AD and of the HSCT procedure. Herein, we report new onset of myasthenia gravis 3 years after successful HSCT in a patient with severe systemic sclerosis, while in complete remission from her first AD. The de novo occurrence of secondary AD (myasthenia gravis) after HSCT was accompanied by the appearance of clonal T-cell expansions measured by the "immunoscope" technique in the context of an ongoing T-cell immune reconstitution. Secondary ADs are increasingly recognized after HSCT for AD. In our case, development of myasthenia followed clonal T-cell expansion. Detailed T-cell repertoire analysis may shed light on autoreactivity mechanisms after HSCT and may help to identify patients at risk.
authors.affiliation: Department of Internal Medicine and Rheumatology, La Meynard Hospital, Fort de France, France.
authors.forename: Christophe
authors.initials: C
authors.lastname: Deligny
authors.forename: Emmanuel
authors.initials: E
authors.lastname: Clave
authors.forename: David
authors.initials: D
authors.lastname: Sibon
authors.forename: Thomas
authors.initials: T
authors.lastname: Daikeler
authors.forename: Homah
authors.initials: H
authors.lastname: Keshmandt
authors.forename: Maryvonnic
authors.initials: M
authors.lastname: Carmagnat
authors.forename: Corinne
authors.initials: C
authors.lastname: Douay
authors.forename: Serge
authors.initials: S
authors.lastname: Arfi
authors.forename: Bernard
authors.initials: B
authors.lastname: Clair
authors.forename: Antoine
authors.initials: A
authors.lastname: Toubert
authors.forename: Dominique
authors.initials: D
authors.lastname: Farge
mesh_terms: D015551:Autoimmunity; D049109:Cell Proliferation; D002478:Cells, Cultured; D004247:DNA / Q000032:analysis*; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D007108:Immune Tolerance; D008875:Middle Aged; D009157:Myasthenia Gravis / Q000175:diagnosis* / Q000209:etiology* / Q000235:genetics / Q000503:physiopathology; D057089:Pathology, Molecular; D011183:Postoperative Complications; D011948:Receptors, Antigen, T-Cell / Q000096:biosynthesis / Q000235:genetics* / Q000276:immunology; D012074:Remission Induction; D012595:Scleroderma, Systemic / Q000150:complications / Q000628:therapy*; D013601:T-Lymphocytes / Q000276:immunology / Q000378:metabolism* / Q000473:pathology; D014182:Transplantation, Autologous
chemical_list: D011948:Receptors, Antigen, T-Cell; D004247:DNA