Predictors of new fragility fractures after diagnosis of indolent systemic mastocytosis.

BACKGROUND: Fragility fractures (FFxs) and osteoporosis occur frequently in patients with indolent systemic mastocytosis (ISM), even before 50 years of age. OBJECTIVE: We sought to develop a prediction model to identify individual patients with ISM at risk of new FFxs. METHODS: Data on lifetime fractures and trauma circumstances were collected from vertebral morphometry, patients' records, and questionnaires. Clinical, lifestyle, and bone characteristics were measured. Patients receiving treatment for osteoporosis before ISM diagnosis or with missing bone data were excluded from FFx risk assessment. RESULTS: In total, 389 lifetime fractures occurred in 127 of the 221 patients with ISM (age, 19-77 years), including 90 patients with 264 FFxs. Median follow-up after diagnosis was 5.4 years (range, 0.4-15.3 years), with 5- and 10-year FFx risks of 23% ± 3% and 31% ± 4%, respectively. Male sex, high levels of bone resorption (serum type I collagen C-telopeptide), low hip bone mineral density, absence of urticaria pigmentosa, and alcohol intake at the time of ISM diagnosis were independent predictors of future FFxs. The MastFx score, a prediction model using these 5 characteristics, showed good accuracy (area under the curve, 0.80) to determine the risk of new FFxs. QFracture, a validated risk scoring tool for persons aged 30 to 99 years, was not useful in patients with ISM. CONCLUSION: The MastFx score distinguishes patients with ISM at high, intermediate, and low risk of new FFxs. The included characteristics sex, serum type I collagen C-telopeptide, hip bone mineral density, urticaria pigmentosa, and alcohol intake are easy to collect in clinical practice. The high occurrence of FFxs in patients with ISM underlines the importance of optimizing bone quality and early start of therapeutic prevention in patients at risk.