Y Degboé1,2, M Eischen3, P A Apoil4, C Mailhol5, P Dubreuil6, O Hermine7, C Paul8, C Bulai Livideanu8, M Laroche3. 1. Rheumatology Centre, Pierre Paul Riquet Hospital, Toulouse University Hospital & Paul Sabatier University, 1 Place du Dr Baylac, 31059, Toulouse, France. degboe.y@chu-toulouse.fr. 2. Center for Pathophysiology of Toulouse Purpan, INSERM UMR 1043, CHU Purpan, Toulouse, France. degboe.y@chu-toulouse.fr. 3. Rheumatology Centre, Pierre Paul Riquet Hospital, Toulouse University Hospital & Paul Sabatier University, 1 Place du Dr Baylac, 31059, Toulouse, France. 4. Department of Immunology, Rangueil Hospital, Toulouse University Hospital, Toulouse, France. 5. Department of Pneumo-allergology, Larrey Hospital, Toulouse University Hospital, Toulouse, France. 6. Cancer Research Center of Marseille, INSERM , Institut Paoli Calmettes & CNRS, CEREMAST, Label Ligue Contre le Cancer, Aix Marseille University, Marseille, France. 7. Department of Hematology, CEREMAST, Label Ligue Contre le Cancer, Université Paris Descartes & Hôpital Necker Enfants Malades & Assistance Publique Hôpitaux de Paris, Paris, France. 8. Department of Dermatology, Mastocytosis Expert Centre of Midi-Pyrénées, Toulouse University Hospital & Paul Sabatier University, Toulouse, France.
Abstract
Little is known about osteoporosis in mast cell disorders (MCDs) not related to systemic mastocytosis. We described osteoporosis and fractures in MCDs and showed that systemic mastocytosis was the only studied MCDs associated with osteoporotic vertebral fractures. INTRODUCTION: To describe osteoporosis (OP) and fragility fractures in mast cell disorders (MCDs). METHODS: We retrospectively analyzed data concerning all successive patients with systemic mastocytosis (SM), cutaneous mastocytosis (CM), and mast cell activation syndromes (MCAS) diagnosed in our mastocytosis expert center between 2004 and 2015. We collected data concerning demographic profiles, clinical signs of MCD, osteoporosis, fractures, densitometry, and biological assessment of MCD. We compared CM and MCAS patients with SM patients with regard to the characteristics of OP and fragility fractures. RESULTS: We assessed 89 SM patients, 20 CM patients, and 20 MCAS patients. Osteoporosis was less frequent in CM (15.0%) and MCAS (10.0%) than in SM (44.9%). Similarly, fractures were less frequent in non-SM MCDs, respectively 5.0%, 5.0%, and 28.1%. SM patients displayed high prevalence of vertebral fractures (22.5%), mostly multiple. Conversely, in non-SM patients, vertebral fractures appeared to be uncommon (5%) and more frequently associated with risk factors for osteoporosis. CONCLUSIONS: SM is associated with multiple vertebral osteoporotic fractures, whereas CM and MCAS do not appear to be associated with this phenotype.
Little is known about osteoporosis in mast cell disorders (MCDs) not related to systemic mastocytosis. We described osteoporosis and fractures in MCDs and showed that systemic mastocytosis was the only studied MCDs associated with osteoporotic vertebral fractures. INTRODUCTION: To describe osteoporosis (OP) and fragility fractures in mast cell disorders (MCDs). METHODS: We retrospectively analyzed data concerning all successive patients with systemic mastocytosis (SM), cutaneous mastocytosis (CM), and mast cell activation syndromes (MCAS) diagnosed in our mastocytosis expert center between 2004 and 2015. We collected data concerning demographic profiles, clinical signs of MCD, osteoporosis, fractures, densitometry, and biological assessment of MCD. We compared CM and MCAS patients with SM patients with regard to the characteristics of OP and fragility fractures. RESULTS: We assessed 89 SM patients, 20 CMpatients, and 20 MCAS patients. Osteoporosis was less frequent in CM (15.0%) and MCAS (10.0%) than in SM (44.9%). Similarly, fractures were less frequent in non-SM MCDs, respectively 5.0%, 5.0%, and 28.1%. SM patients displayed high prevalence of vertebral fractures (22.5%), mostly multiple. Conversely, in non-SM patients, vertebral fractures appeared to be uncommon (5%) and more frequently associated with risk factors for osteoporosis. CONCLUSIONS: SM is associated with multiple vertebral osteoporotic fractures, whereas CM and MCAS do not appear to be associated with this phenotype.