Abstract
One of the most common complications of fibrous dysplasia of bone (FD) is bone pain. Usual pain killers are often of inadequate efficacy to control this bone pain. The mechanism of bone pain in FD remains uncertain, but by analogy with bone tumors one may consider that ectopic sprouting and formation of neuroma-like structures by sensory and sympathetic nerve fibers also occur in the dysplastic skeleton. Bone pain has been reported in up to 81% of adults and 49% of children. It affects predominantly the lower limbs and the spine. The degree of pain is highly variable and adults reports more pain than children. Bisphosphonates have been shown to reduce bone pain in uncontrolled studies. Their influence on bone strength remains unknown. In a randomized trial testing alendronate, bone pain was not significantly improved. Another trial assessing the effect of risedronate is ongoing. Possible future therapies include tocilizumab, denosumab and drugs targeting nerve growth factor and its receptor TrkA.
Highlights
Fibrous dysplasia of bone (FD) is a rare disease responsible for bone deformities, fractures, nerve compression and bone pain
These newly sprouted nerve fibers are probably activated and sensitized by released nerve growth factor (NGF) and as such this truly ectopic and pathological reorganization of sensory and sympathetic nerve fibers may provide an anatomical substrate which drives skeletal pain. In support of this hypothesis, preventive treatment with an antibody that sequesters NGF, administered when prostate tumorinduced pain and bone remodeling were first observed, blocked this ectopic sprouting and significantly inhibited the development and severity of cancer pain [13]. While it is not known whether sprouting of sensory nerve fibers occurs in FD, this phenomenon has been observed in non-malignant skeletal pain states in human and animals
In a series of 6 adult patients who had been treated with pamidronate followed by alendronate or who had used alendronate alone, bone pain decreased substantially in response to therapy, bone resorption was reduced with intravenous pamidronate but not with oral alendronate, and four out of six patients exhibited radiological improvement [27]
Summary
Fibrous dysplasia of bone (FD) is a rare disease responsible for bone deformities, fractures, nerve compression and bone pain. These newly sprouted nerve fibers are probably activated and sensitized by released NGF and as such this truly ectopic and pathological reorganization of sensory and sympathetic nerve fibers may provide an anatomical substrate which drives skeletal pain In support of this hypothesis, preventive treatment with an antibody that sequesters NGF, administered when prostate tumorinduced pain and bone remodeling were first observed, blocked this ectopic sprouting and significantly inhibited the development and severity of cancer pain [13]. While it is not known whether sprouting of sensory nerve fibers occurs in FD, this phenomenon has been observed in non-malignant skeletal pain states in human and animals. Analgesic use and perceived relief information was obtained as part of the questionnaire, and confirmed during patient interviews
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