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Refractory Primary Sjögren Syndrome Successfully Treated With Bortezomib

Jakez-Ocampo, Juan MD*; Atisha-Fregoso, Yemil MD; Llorente, Luis MD*

JCR: Journal of Clinical Rheumatology: January 2015 - Volume 21 - Issue 1 - p 31–32
doi: 10.1097/RHU.0000000000000210
Case Reports

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

From the *Department of Immunology and Rheumatology and †Medical Direction, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.

The authors declare no conflict of interest.

Correspondence: Luis Llorente, MD, Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Vasco de Quiroga 15, Tlalpan, 14000 México, D. F. Mexico. E-mail:

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