Murphy E, Freaney R, Bresnihan B, McKenna M, FitzGerald O
Department of Rheumatology, St. Vincent's University Hospital, Dublin, Ireland.
Calcif Tissue Int. 2003 Nov;73(5):433-40. doi: 10.1007/s00223-002-2127-x. Epub 2003 Sep 10.
The aim of this study was to evaluate strategies to halt the progression of severe dystrophic calcification in a patient with progressive systemic sclerosis (PSS) and to monitor serial changes in biochemical markers of bone resorption and indices of calcium (Ca) metabolism in response to therapy. The relationship of bone turnover to the extent of dystrophic calcification was also investigated in a number of additional patients with varying degrees of calcinosis. Serial markers of bone turnover and indices of Ca metabolism were measured over a 3-year period in one patient with PSS and severe dystrophic calcification. Bone mineral density (BMD) was assessed by dual-energy x-ray absorptiometry (DXA). Bone turnover in this patient and two additional patients with PSS or dermatomyositis (DM) and severe dystrophic calcification (Group A, n = 3) was compared with that in patients with connective tissue disease with little or no evidence of dystrophic calcification (Group B, n = 13). Serial data on one patient with severe progressive calcinosis showed increased bone resorption markers, which remained high over a 3-year period despite antiresorptive therapy. BMD was low. Patients with PSS/DM with severe dystrophic calcification had higher bone resorption than those with minimal or no evidence of calcification. Mean serum ionized Ca and urinary Ca excretion were both lower in those with severe calcinosis. Bone resorption was increased in patients with connective tissue disease and severe dystrophic calcification. Several antiresorptive agents were shown to be ineffective in limiting either bone turnover or clinical progression in one patient.
本研究的目的是评估阻止一名进行性系统性硬化症（PSS）患者严重营养不良性钙化进展的策略，并监测骨吸收生化标志物和钙（Ca）代谢指标在治疗后的系列变化。还在一些钙化程度不同的其他患者中研究了骨转换与营养不良性钙化程度之间的关系。在一名患有PSS和严重营养不良性钙化的患者中，对骨转换的系列标志物和Ca代谢指标进行了为期3年的测量。通过双能X线吸收法（DXA）评估骨密度（BMD）。将该患者以及另外两名患有PSS或皮肌炎（DM）和严重营养不良性钙化的患者（A组，n = 3）的骨转换情况与结缔组织病且几乎没有或没有营养不良性钙化证据的患者（B组，n = 13）进行比较。一名严重进行性钙化患者的系列数据显示骨吸收标志物增加，尽管进行了抗吸收治疗，但在3年期间一直居高不下。BMD较低。患有严重营养不良性钙化的PSS/DM患者的骨吸收高于钙化程度轻微或无钙化证据的患者。严重钙化患者的平均血清离子钙和尿钙排泄均较低。结缔组织病和严重营养不良性钙化患者的骨吸收增加。在一名患者中，几种抗吸收剂在限制骨转换或临床进展方面均无效。
