Cafardi John M, Sami Naveed
The Christ Hospital and The University of Cincinnati Medical Center, USA.
Department of Dermatology, The University of Alabama at Birmingham, USA.
Open Rheumatol J. 2015 Nov 4;9:77-81. doi: 10.2174/1874312901409010077. eCollection 2015.
Amyopathic dermatomyositis (ADM) is a rare subtype of dermatomyositis which is often recalcitrant to immune suppressing treatments. Intravenous immunoglobulin (IVIG) has been used in the treatment of refractory dermatomyositis. We present two patients with severe ADM, who were treated with IVIG at 2 g/kg every four weeks. Both patients had a successful response and were able to taper the dosage of prednisone. We present both cases in describing IVIG as a rescue and maintenance steroid-sparing agent in the treatment of severe refractory ADM. We also review the treatment of refractory ADM with IVIg in the English literature.
无肌病性皮肌炎(ADM)是皮肌炎的一种罕见亚型,通常对免疫抑制治疗具有抵抗性。静脉注射免疫球蛋白(IVIG)已被用于治疗难治性皮肌炎。我们报告了两名严重ADM患者,他们接受了每四周2 g/kg的IVIG治疗。两名患者均取得了成功的治疗反应,并能够逐渐减少泼尼松的用量。我们通过这两个病例描述了IVIG作为一种挽救和维持药物,在治疗严重难治性ADM时可减少类固醇药物的使用。我们还回顾了英文文献中使用IVIg治疗难治性ADM的情况。