Rheumatology Centre, Pierre Paul Riquet Hospital, Toulouse University Hospital and Paul Sabatier University, Toulouse, France.
Rheumatology Centre, Pierre Paul Riquet Hospital, Toulouse University Hospital and Paul Sabatier University, Toulouse, France.
Bone. 2017 Dec;105:219-225. doi: 10.1016/j.bone.2017.09.005. Epub 2017 Sep 14.
Systemic mastocytosis (SM) is characterized by the accumulation of mast cells in tissues other than the skin. Bone involvement although frequent has not been thoroughly evaluated. Primary objective was to determine risk factors associated with fragility fractures (FF) in SM. Secondary objectives were to evaluate the ability of bone marrow tryptase (BMT) level to identify patients with FF, and to describe bone involvement in SM.
We analyzed retrospectively all consecutive patients seen in our expert center, with a diagnosis of SM according to the 2001 WHO criteria, and with complete bone assessment. We collected data about lifetime fractures, types of cutaneous manifestations, degranulation symptoms, blood and BMT levels, bone mineral density assessed by densitometry and KIT mutation. We performed a univariate analysis investigating the factors associated with FF and then a logistic multivariable regression analysis. We assessed the ability of bone marrow tryptase to identify patients with FF.
Eighty-nine patients with SM were included. Thirty-six patients (40.4%) suffered from osteoporosis and twenty-five (28.1%) experienced lifetime FF. Univariate analysis identified age at diagnosis and disease onset, presence of telangiectasia macularis eruptiva perstans, digestive symptoms, mast cells activation symptoms, elevated BMT, low femoral and lumbar BMD, as associated with FF. Multivariate analysis identified elevated BMT, low femoral T score and older age at diagnosis as independently associated with FF.
Low femoral T-score, BMT level, and older age at diagnosis are markers associated with FF in SM. BMT may represent an important biomarker to predict FF in SM patients.
系统性肥大细胞增多症（SM）的特征是组织中除皮肤以外的肥大细胞积累。尽管骨骼受累很常见，但尚未得到充分评估。主要目的是确定与 SM 脆性骨折（FF）相关的危险因素。次要目的是评估骨髓胰蛋白酶（BMT）水平识别 FF 患者的能力，并描述 SM 中的骨骼受累。
我们回顾性分析了所有在我们的专家中心就诊的、根据 2001 年 WHO 标准诊断为 SM 且具有完整骨骼评估的连续患者。我们收集了关于终身骨折、皮肤表现类型、脱颗粒症状、血液和 BMT 水平、通过骨密度仪评估的骨矿物质密度和 KIT 突变的数据。我们进行了单因素分析，调查与 FF 相关的因素，然后进行逻辑多元回归分析。我们评估了骨髓胰蛋白酶识别 FF 患者的能力。
共纳入 89 例 SM 患者。36 例（40.4%）患有骨质疏松症，25 例（28.1%）经历过终身 FF。单因素分析确定了诊断时和发病时的年龄、存在持久性皮疹性毛细血管扩张症、消化症状、肥大细胞激活症状、BMT 升高、股骨和腰椎 BMD 降低与 FF 相关。多因素分析确定了 BMT 升高、股骨 T 评分低和诊断时年龄较大与 FF 独立相关。
股骨 T 评分低、BMT 水平和诊断时年龄较大是 SM 中 FF 的相关标志物。BMT 可能是预测 SM 患者 FF 的重要生物标志物。
