Zoledronic acid, bisphosphonates, interferon (IFN)-alpha therapy, and denosumab may successfully prevent fracture risk and increase bone mineral density in patients with systemic mastocytosis (SM)-associated osteoporosis, according to a recently published literature review in Medicina.
A technical expert panel (TEP) of eight physicians, two physiatrists specializing in metabolic bone diseases and two experts in review methodology examined the results of an extensive search in renowned medical databases to provide an overview of the available therapeutic alternatives for SM-associated osteoporosis.
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“There is still no consensus on specific anti-osteoporotic treatment in patients with systemic mastocytosisMastocytosis Rare disease caused by the buildup of mast cells. Cutaneous mastocytosis primarily affects the skin and is more common in children, while systemic mastocytosis affects internal organs and is more common in adults.,” the authors wrote. “In this context, with this scoping review, we aimed to provide an overview of secondary osteoporosis in systemic mastocytosis in order to define early and appropriate pharmacological treatment.”
The search yielded nine observational clinical studies that met the previously established inclusion criteria. Six were about pharmacological treatments’ clinical efficacy in patients with SM osteoporosis.
Results showed that patients with indolent SMIndolent SM A subtype of nonadvanced SM caused by the abnormal accumulation of mast cells in the bone marrow and other organs. Indolent SM accounts for around 90% of SM cases. benefited from treatment with bisphosphonates in terms of increased bone density. However, in patients with a history of prior fractures, fractures can still occur frequently. Zoledronic acid was also effective; however, many patients reported an acute reaction towards the first dose.
Denosumab, a monoclonal antibody used in patients intolerant to bisphosphonates, showed promising results regarding increased BMD and decreased bone turnover markers with minimal adverse effects.
Cytoreductive therapies such as IFN therapy are reserved for patients with advanced forms of SM or those refractory to other treatments. The literature review showed three cases where IFN therapy led to BMD increase and mast cellMast cell A type of white blood cell produced in the bone marrow. They help defend against infections and play a key role in allergic reactions. In SM, mast cells become overactive and build up throughout the body. decrease in the bone marrow.
“The literature demonstrates that there are several effective therapeutic options, which can lead to an increase in bone mineral density (BMD) and a reduction in fracture risk, thereby improving the patient’s quality of life,” the authors wrote. Finally, since this is a rare disease that requires a multidisciplinary approach, it is essential for patients to be followed in specialized centers for mastocytosis to receive comprehensive and coordinated care.”
