Fantò M, Salemi S, Socciarelli F, Bartolazzi A, Natale G A, Casorelli I, Pavan A, Vaglio S, Di Rosa R, D'Amelio R
Department of Allergy, Clinical Immunology and Rheumatology, S. Andrea Hospital, Sapienza University of Rome, Italy.
Case Rep Rheumatol. 2013;2013:857694. doi: 10.1155/2013/857694. Epub 2013 Jul 28.
A 30-year-old woman affected by Mixed Connective Tissue Disease with scleroderma spectrum developed a facial eruption, a clinical and histological characteristic of subacute cutaneous lupus erythematosus (SCLE). Speckled anti-nuclear antibodies, high-titer anti-ribonucleoprotein1, anti-Sm, anti-Cardiolipin (aCL) IgG/IgM, and anti-Ro/SSA antibodies were positive. SCLE was resistant to Azathioprine, Hydroxychloroquine, and Methotrexate while Mycophenolate Mofetil was suspended due to side effects. Subsequently, the patient was treated with three cycles of therapeutic plasma exchange (TPE) followed, one month after the last TPE, by the anti-CD20 antibody Rituximab (RTX) (375 mg/m(2) weekly for 4 weeks). Eight and 16 months later the patient received other two TPE and RTX cycles, respectively. This therapeutic approach has allowed to obtain a complete skin healing persistent even after 8-month follow-up. Moreover, mitigation of Raynaud's phenomenon, resolution of alopecia, and a decline of aCL IgG/IgM and anti-Ro/SSA antibodies were observed.
一名患有硬皮病谱系混合性结缔组织病的30岁女性出现了面部皮疹,这是亚急性皮肤型红斑狼疮(SCLE)的临床和组织学特征。斑点型抗核抗体、高滴度抗核糖核蛋白1、抗Sm、抗心磷脂(aCL)IgG/IgM以及抗Ro/SSA抗体均呈阳性。SCLE对硫唑嘌呤、羟氯喹和甲氨蝶呤耐药,而霉酚酸酯因副作用停药。随后,患者接受了三个疗程的治疗性血浆置换(TPE),在最后一次TPE一个月后,接受了抗CD20抗体利妥昔单抗(RTX)治疗(375mg/m²,每周一次,共4周)。8个月和16个月后患者分别又接受了两个TPE和RTX疗程。这种治疗方法使皮肤完全愈合,即使在8个月的随访后仍持续存在。此外,还观察到雷诺现象减轻、脱发症状缓解以及aCL IgG/IgM和抗Ro/SSA抗体水平下降。