Osteoporosis in Systemic Mastocytosis: A Scoping Review
<i>Background</i>: 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 impac...
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2024-10-01
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| Online Access: | https://www.mdpi.com/1648-9144/60/11/1752 |
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| author | Giulia Letizia Mauro Jessica Accomando Sofia Tomasello Adele Duca Maria Silvia Mangano Alessandro de Sire Michele Vecchio Dalila Scaturro |
| author_facet | Giulia Letizia Mauro Jessica Accomando Sofia Tomasello Adele Duca Maria Silvia Mangano Alessandro de Sire Michele Vecchio Dalila Scaturro |
| author_sort | Giulia Letizia Mauro |
| collection | DOAJ |
| description | <i>Background</i>: 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. <i>Objective</i>: 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. <i>Design</i>: 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: “bisphosphonate therapy”, “zoledronic acid therapy”, “denosumab therapy”, “IFN-alpha therapy”, and “IFN-alpha therapy in combination with pamidronate”. <i>Results</i>: 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. <i>Conclusions</i>: 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. |
| format | Article |
| id | doaj-art-3521e1d4c94c4ca2b9d2e86cfa9a1270 |
| institution | OA Journals |
| issn | 1010-660X 1648-9144 |
| language | English |
| publishDate | 2024-10-01 |
| publisher | MDPI AG |
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| series | Medicina |
| spelling | doaj-art-3521e1d4c94c4ca2b9d2e86cfa9a12702025-08-20T02:05:04ZengMDPI AGMedicina1010-660X1648-91442024-10-016011175210.3390/medicina60111752Osteoporosis in Systemic Mastocytosis: A Scoping ReviewGiulia Letizia Mauro0Jessica Accomando1Sofia Tomasello2Adele Duca3Maria Silvia Mangano4Alessandro de Sire5Michele Vecchio6Dalila Scaturro7Precision Medicine in the Medical, Surgical and Critical Care Areas, University of Palermo, 90100 Palermo, ItalySection of Pharmacology, Department of Biomedical and Biotechnological Sciences, University of Catania, 37100 Catania, ItalyNeuromotor and Cognitive Rehabilitation Research Center, Physical and Rehabilitation Medicine Section, Department of Neurosciences, Biomedicine and Movement Sciences, University of Verona, 37100 Verona, ItalySection of Pharmacology, Department of Biomedical and Biotechnological Sciences, University of Catania, 37100 Catania, ItalySection of Pharmacology, Department of Biomedical and Biotechnological Sciences, University of Catania, 37100 Catania, ItalyPhysical and Rehabilitative Medicine, Department of Medical and Surgical Sciences, University of Catanzaro “Magna Graecia”, 88100 Catanzaro, ItalySection of Pharmacology, Department of Biomedical and Biotechnological Sciences, University of Catania, 37100 Catania, ItalyPrecision Medicine in the Medical, Surgical and Critical Care Areas, University of Palermo, 90100 Palermo, Italy<i>Background</i>: 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. <i>Objective</i>: 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. <i>Design</i>: 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: “bisphosphonate therapy”, “zoledronic acid therapy”, “denosumab therapy”, “IFN-alpha therapy”, and “IFN-alpha therapy in combination with pamidronate”. <i>Results</i>: 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. <i>Conclusions</i>: 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.https://www.mdpi.com/1648-9144/60/11/1752systemic mastocytosissecondary osteoporosisBMDosteoporotic fracturesbone densitometryradiography |
| spellingShingle | Giulia Letizia Mauro Jessica Accomando Sofia Tomasello Adele Duca Maria Silvia Mangano Alessandro de Sire Michele Vecchio Dalila Scaturro Osteoporosis in Systemic Mastocytosis: A Scoping Review Medicina systemic mastocytosis secondary osteoporosis BMD osteoporotic fractures bone densitometry radiography |
| title | Osteoporosis in Systemic Mastocytosis: A Scoping Review |
| title_full | Osteoporosis in Systemic Mastocytosis: A Scoping Review |
| title_fullStr | Osteoporosis in Systemic Mastocytosis: A Scoping Review |
| title_full_unstemmed | Osteoporosis in Systemic Mastocytosis: A Scoping Review |
| title_short | Osteoporosis in Systemic Mastocytosis: A Scoping Review |
| title_sort | osteoporosis in systemic mastocytosis a scoping review |
| topic | systemic mastocytosis secondary osteoporosis BMD osteoporotic fractures bone densitometry radiography |
| url | https://www.mdpi.com/1648-9144/60/11/1752 |
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