{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,3]],"date-time":"2026-10-03T02:21:01Z","timestamp":1790994061079,"version":"4.1.0"},"reference-count":48,"publisher":"MDPI AG","issue":"11","license":[{"start":{"date-parts":[[2024,10,24]],"date-time":"2024-10-24T00:00:00Z","timestamp":1729728000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Medicina"],"abstract":"<jats:p>Background: Mastocytosis (MS) is a rare disease that can involve various organs, including the bone. Given the incidence of the disease in the global population, MS poses a challenge for physicians, and early therapeutic intervention in the initial stages could significantly impact the quality of life of affected patients. Objective: The aim of this scoping review was to provide an overview of secondary osteoporosis in systemic mastocytosis (SM), focusing on the heterogeneity of its manifestations, the benefits of early diagnosis, and appropriate pharmacological treatment. Design: A technical expert panel (TEP) consisting of 8 physicians with expertise in metabolic bone diseases conducted the review following the PRISMA-ScR model. A strength of this study is that it provides various therapeutic approaches for patients with bone involvement in SM, although the limited available literature on the topic constituted a limitation. The TEP sought evidence regarding the following diagnostic and therapeutic modalities in the management of SM: \u201cbisphosphonate therapy\u201d, \u201czoledronic acid therapy\u201d, \u201cdenosumab therapy\u201d, \u201cIFN-alpha therapy\u201d, and \u201cIFN-alpha therapy in combination with pamidronate\u201d. Results: Clinical data showed a correlation between densitometric outcomes, serum tryptase levels, and mast cell infiltration in the bone marrow, between increased bone mineral density and the presence of osteosclerosis in cases of advanced SM, between the severity of osteoporosis and hypertryptasemia, and also provided results on the long-term effects of bisphosphonate therapy, the therapeutic efficacy of zoledronic acid administration, the positive effect of denosumab on the reduction of serum tryptase levels (even if is proved in a limited numbers of cases) and the prevention of new fractures, and the effect of IFN-alpha in more severe cases of SM, either alone or in combination with pamidronate. Conclusions: Studies have demonstrated the effectiveness of various treatments depending on the form of mastocytosis, whether indolent systemic or advanced systemic, in the prognosis of the disease. However, this role should be further investigated in additional clinical studies, considering the limited data on the use of these interventions.<\/jats:p>","DOI":"10.3390\/medicina60111752","type":"journal-article","created":{"date-parts":[[2024,10,25]],"date-time":"2024-10-25T03:46:04Z","timestamp":1729827964000},"page":"1752","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["Osteoporosis in Systemic Mastocytosis: A Scoping Review"],"prefix":"10.3390","volume":"60","author":[{"given":"Giulia","family":"Letizia Mauro","sequence":"first","affiliation":[{"name":"Precision Medicine in the Medical, Surgical and Critical Care Areas, University of Palermo, 90100 Palermo, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jessica","family":"Accomando","sequence":"additional","affiliation":[{"name":"Section of Pharmacology, Department of Biomedical and Biotechnological Sciences, University of Catania, 37100 Catania, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sofia","family":"Tomasello","sequence":"additional","affiliation":[{"name":"Neuromotor and Cognitive Rehabilitation Research Center, Physical and Rehabilitation Medicine Section, Department of Neurosciences, Biomedicine and Movement Sciences, University of Verona, 37100 Verona, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adele","family":"Duca","sequence":"additional","affiliation":[{"name":"Section of Pharmacology, Department of Biomedical and Biotechnological Sciences, University of Catania, 37100 Catania, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria Silvia","family":"Mangano","sequence":"additional","affiliation":[{"name":"Section of Pharmacology, Department of Biomedical and Biotechnological Sciences, University of Catania, 37100 Catania, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5541-8346","authenticated-orcid":false,"given":"Alessandro","family":"de Sire","sequence":"additional","affiliation":[{"name":"Physical and Rehabilitative Medicine, Department of Medical and Surgical Sciences, University of Catanzaro \u201cMagna Graecia\u201d, 88100 Catanzaro, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5254-3283","authenticated-orcid":false,"given":"Michele","family":"Vecchio","sequence":"additional","affiliation":[{"name":"Section of Pharmacology, Department of Biomedical and Biotechnological Sciences, University of Catania, 37100 Catania, Italy"},{"name":"Rehabilitation Unit, AOU Policlinico Vittorio Emanuele, 95123 Catania, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5035-2288","authenticated-orcid":false,"given":"Dalila","family":"Scaturro","sequence":"additional","affiliation":[{"name":"Precision Medicine in the Medical, Surgical and Critical Care Areas, University of Palermo, 90100 Palermo, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2024,10,24]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","unstructured":"Gaudio, A., Xourafa, A., Rapisarda, R., Zanoli, L., Signorelli, S.S., and Castellino, P. 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