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High prevalence of fractures and osteoporosis in patients with indolent systemic mastocytosis

机译：惰性系统性肥大细胞增多症患者的骨折和骨质疏松症高发

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Background: Indolent systemic mastocytosis (ISM) is a rare disease characterized by accumulation of abnormal mast cells in various tissues, including bone marrow. Symptoms are usually related to release of mast cell mediators. The aims are to establish the prevalence of osteoporotic fractures in ISM and to investigate the association with serum tryptase and the urinary histamine metabolites, methylhistamine (MH), and methylimidazole acetic acid. Methods: The fracture prevalence in 157 patients (65 men; 92 women), mean age 54 ± 12 years, was assessed by vertebral morphometry and data from patient records, supplemented by a questionnaire. Bone mineral density (BMD) of lumbar spine and femoral neck was measured, and tryptase and histamine metabolites were analysed. Results: We registered 235 lifetime fractures in 154 patients, including 140 osteoporotic (low-energy trauma) fractures, of which 62% were vertebral, 1% hip and 36% other nonvertebral fractures. Osteoporotic fractures and osteoporosis were found in 37% and 28% of the patients, respectively. In men, the prevalence of these osteoporotic manifestations (46% <50 years; 73% ≥50 years) was much higher compared with women (18% <50 years; 58% ≥50 years). Older age, male gender, and higher urinary MH were independently related to the osteoporotic manifestations. Conclusions: This first publication about prevalence of fractures and osteoporosis in patients with ISM shows that the risk of osteoporotic fractures is high, especially in men. Higher urinary MH levels are associated with a higher risk of osteoporotic manifestations. Routine measurements of BMD and vertebral morphometry are warranted in these patients for early detection of osteoporosis.
机译：背景：惰性系统性肥大细胞增多症（ISM）是一种罕见的疾病，其特征是异常的肥大细胞在包括骨髓在内的各种组织中积累。症状通常与肥大细胞介质的释放有关。目的是确定ISM中骨质疏松性骨折的患病率，并探讨其与血清类胰蛋白酶和尿组胺代谢产物，甲基组胺（MH）和甲基咪唑乙酸的关系。方法：通过椎体形态测量和患者病历数据，并补充问卷，对157例患者的骨折发生率进行了评估（男65例;女92例），平均年龄54±12岁。测量腰椎和股骨颈的骨矿物质密度（BMD），并分析类胰蛋白酶和组胺代谢产物。结果：我们登记了154例患者中的235例终生骨折，包括140例骨质疏松（低能量创伤）骨折，其中62％为椎骨，1％髋关节和36％其他非椎骨骨折。分别在37％和28％的患者中发现骨质疏松性骨折和骨质疏松症。在男性中，这些骨质疏松表现的患病率（46％<50岁; 73％≥50岁）比女性（18％<50岁; 58％≥50岁）高得多。年龄，男性性别和较高的尿中MH与骨质疏松表现独立相关。结论：这本有关ISM患者骨折和骨质疏松症患病率的首次出版物显示，骨质疏松性骨折的风险很高，尤其是男性。较高的尿中MH水平与骨质疏松表现的较高风险有关。这些患者应常规进行BMD和椎体形态测量，以早期发现骨质疏松症。

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