title: Refractory primary Sjögren syndrome successfully treated with bortezomib.
abstract: Primary Sjögren syndrome (PSS) is a chronic autoimmune disease characterized by sicca complex and various systemic manifestations. Although it is well accepted to use corticosteroids for the treatment of systemic manifestations, there is scarce information available regarding the use of targeted therapy for refractory cases. We describe a case of a severe PSS patient refractory to conventional treatment with a response to bortezomib, a proteasome inhibitor commonly used for the treatment of multiple myeloma. Bortezomib administration resulted in a notable improvement of the general symptoms, particularly fatigue, and a decrease in serum globulin levels as well as in serum viscosity. Hyperglobulinemic purpura disappeared, and prednisone tapering succeeded. Because of chronicity, no clinical changes were observed in sicca symptoms. As far as we know, this is the first report on the use of bortezomib in a refractory case of PSS.
authors.affiliation: From the *Department of Immunology and Rheumatology and †Medical Direction, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.
authors.forename: Juan
authors.initials: J
authors.lastname: Jakez-Ocampo
authors.forename: Yemil
authors.initials: Y
authors.lastname: Atisha-Fregoso
authors.forename: Luis
authors.initials: L
authors.lastname: Llorente
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D001897:Boronic Acids / Q000009:adverse effects / Q000627:therapeutic use*; D000069286:Bortezomib; D004351:Drug Resistance; D005221:Fatigue / Q000188:drug therapy / Q000453:epidemiology / Q000209:etiology; D005260:Female; D005916:Globulins / Q000378:metabolism; D006801:Humans; D015994:Incidence; D061988:Proteasome Inhibitors / Q000009:adverse effects / Q000627:therapeutic use*; D011719:Pyrazines / Q000009:adverse effects / Q000627:therapeutic use*; D012720:Severity of Illness Index; D012859:Sjogren's Syndrome / Q000150:complications / Q000188:drug therapy* / Q000378:metabolism; D016896:Treatment Outcome
chemical_list: D000305:Adrenal Cortex Hormones; D001897:Boronic Acids; D005916:Globulins; D061988:Proteasome Inhibitors; D011719:Pyrazines; D000069286:Bortezomib