Prevalence, pathogenesis, and treatment options for mastocytosis-related osteoporosis
| Autor: | Vidya Satheesn Kunnathully, Roberta Zanotti, Maurizio Rossini, Ombretta Viapiana, Alberto Zamò, Davide Gatti, Silvano Adami, Giovanni Orsolini, Gaia Tripi, Luca Idolazzi, Patrizia Bonadonna |
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| Jazyk: | angličtina |
| Rok vydání: | 2016 |
| Předmět: |
0301 basic medicine
Pathology medicine.medical_specialty Endocrinology Diabetes and Metabolism medicine.medical_treatment Osteoporosis Osteoclasts Zoledronic Acid Bone resorption Mast cell proliferation Bone remodeling 03 medical and health sciences 0302 clinical medicine Osteoclast Bone Density Bone mineral density medicine Prevalence Humans Bisphosphonate Systemic mastocytosis Bone Density Conservation Agents Diphosphonates Receptor Activator of Nuclear Factor-kappa B business.industry Bone turnover markers Denosumab Mastocytosis Zoledronate RANK Ligand Imidazoles denosumab medicine.disease osteoporosis 030104 developmental biology medicine.anatomical_structure 030220 oncology & carcinogenesis Immunology Cytokines business medicine.drug Histamine Signal Transduction |
| Popis: | Mastocytosis is a rare condition characterized by abnormal mast cell proliferation and a broad spectrum of manifestations, including various organs and tissues. Osteoporosis is one of the most frequent manifestations of systemic mastocytosis, particularly in adults. Osteoporosis secondary to systemic mastocytosis is a cause of unexplained low bone mineral density that should be investigated when accompanied by suspicious clinical elements. Bone involvement is often complicated by a high recurrence of fragility fractures, mainly vertebral, leading to severe disability. The mechanism of bone loss is the result of different pathways, not yet fully discovered. The main actor is the osteoclast with a relative or absolute predominance of bone resorption. Among the stimuli that drive osteoclast activity, the most important one seems to be the RANK-RANKL signaling, but also histamine and other cytokines play a significant role in the process. The central role of osteoclasts made bisphosphonates, as anti-resorptive drugs, the most rational treatment for bone involvement in systemic mastocytosis. There are a few small studies supporting this approach, with large heterogeneity of drug and administration scheme. Currently, zoledronate has the best evidence in terms of gain in bone mineral density and bone turnover suppression, two surrogate markers of anti-fracture efficacy. |
| Databáze: | OpenAIRE |
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