Jakez-Ocampo Juan, Atisha-Fregoso Yemil, Llorente Luis
From the *Department of Immunology and Rheumatology and †Medical Direction, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.
J Clin Rheumatol. 2015 Jan;21(1):31-2. doi: 10.1097/RHU.0000000000000210.
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.
原发性干燥综合征（PSS）是一种以干燥综合征及各种全身表现为特征的慢性自身免疫性疾病。尽管使用皮质类固醇治疗全身表现已被广泛接受，但关于难治性病例使用靶向治疗的信息却很少。我们描述了一例严重PSS患者，该患者对传统治疗无效，但对硼替佐米（一种常用于治疗多发性骨髓瘤的蛋白酶体抑制剂）有反应。使用硼替佐米后，患者的一般症状显著改善，尤其是疲劳，血清球蛋白水平和血清粘度降低。高球蛋白血症性紫癜消失，泼尼松减量成功。由于疾病的慢性特征，干燥症状未观察到临床变化。据我们所知，这是关于硼替佐米用于难治性PSS病例的首次报告。
