Orsolini Giovanni, Viapiana Ombretta, Rossini Maurizio, Bonifacio Massimiliano, Zanotti Roberta
Rheumatology Unit, University of Verona, Verona, Italy.
Rheumatology Unit, University of Verona, Verona, Italy.
Immunol Allergy Clin North Am. 2018 Aug;38(3):443-454. doi: 10.1016/j.iac.2018.04.013.
Systemic mastocytosis can give very different bone pictures: from osteosclerosis to osteoporosis. Osteoporosis is one of the most frequent manifestations particularly in adults and the most clinical relevant. It is often complicated by a high recurrence of mainly vertebral fragility fractures. The main factor of bone loss is the osteoclast with a relative or absolute predominance of bone resorption. The RANK-RANKL pathway seems of key importance, but histamine and other cytokines also play a significant role in the process. The predominance of resorption made bisphosphonates, as anti-resorptive drugs, the most rational treatment of bone involvement in systemic mastocytosis.
从骨硬化到骨质疏松。骨质疏松是最常见的表现之一,尤其是在成年人中,也是临床上最相关的表现。它常因主要是椎体脆性骨折的高复发率而复杂化。骨质流失的主要因素是破骨细胞,其骨吸收相对或绝对占优势。RANK-RANKL通路似乎至关重要,但组胺和其他细胞因子在这一过程中也发挥着重要作用。由于骨吸收占优势,双膦酸盐作为抗吸收药物,成为系统性肥大细胞增多症骨受累最合理的治疗方法。