Stanford School of Medicine, Division of Immunology &Rheumatology, 1000 Welch Road, Suite 204, Palo Alto, 94304, California, USA.
University of Washington, Department of Medicine, Division of Rheumatology, 750 Republican Street, Seattle, WA, 98109, USA.
Best Pract Res Clin Rheumatol. 2022 Jun;36(2):101768. doi: 10.1016/j.berh.2022.101768. Epub 2022 Jul 5.
Calcinosis, insoluble calcium compounds deposited in skin and other tissues, is a crippling sequela of dermatomyositis. Prolonged disease associated with ongoing inflammation, ischemia, repetitive trauma, and certain autoantibodies are associated with calcinosis. Herein, we describe potential pathogenic mechanisms including the role of mitochondrial calcification. There are no widely effective treatments for calcinosis. We review available pharmacologic therapies for calcinosis including those targeting calcium and phosphorus metabolism; immunosuppressive/anti-inflammatory therapies; and vasodilators. Mounting evidence supports the use of various formulations of sodium thiosulfate in the treatment of calcinosis. Although the early institution of aggressive immunosuppression may prevent calcinosis in juvenile dermatomyositis, only limited data support improvement once it has developed. Minocycline can be useful particularly for lesions associated with surrounding inflammation. Powerful vasodilators, such as prostacyclin analogs, may have promise in the treatment of calcinosis, but further studies are necessary. Surgical removal of lesions when amenable is our treatment of choice.
钙沉积,即不溶性钙化合物在皮肤和其他组织中的沉积,是皮肌炎的一种致残后遗症。与持续性炎症、缺血、反复创伤和某些自身抗体相关的长期疾病与钙沉积有关。在此,我们描述了潜在的发病机制,包括线粒体钙化的作用。目前还没有针对钙沉积的广泛有效治疗方法。我们综述了现有的针对钙沉积的药物治疗方法,包括针对钙磷代谢的治疗方法、免疫抑制/抗炎治疗方法和血管扩张剂。越来越多的证据支持使用各种硫代硫酸钠制剂治疗钙沉积。虽然早期积极的免疫抑制治疗可能预防幼年皮肌炎中的钙沉积,但只有有限的数据支持在其发展后改善。米诺环素尤其对伴有周围炎症的病变有用。强力血管扩张剂,如前列环素类似物,在治疗钙沉积方面可能有一定前景,但还需要进一步的研究。当病变适合手术切除时,我们选择手术切除病变。