Fibrous Dysplasia of Bone

A disease of bone marked by thinning of the cortex by fibrous tissue containing bony spicules, producing pain, disability, and gradually increasing deformity. Only one bone may be involved (FIBROUS DYSPLASIA, MONOSTOTIC) or several (FIBROUS DYSPLASIA, POLYOSTOTIC).

Also Known As:

Fibrocartilaginous Dysplasia of Bone; Fibrocystic Dysplasia of Bone; Jaffe-Lichtenstein Disease; Bone Fibrocartilaginous Dysplasia; Bone Fibrocartilaginous Dysplasias; Bone Fibrocystic Dysplasia; Bone Fibrocystic Dysplasias; Bone Fibrous Dysplasia; Bone Fibrous Dysplasias; Jaffe Lichtenstein Disease; Osteitis Fibrosa Disseminata

Networked: 78 relevant articles (2 outcomes, 6 trials/studies)

Relationship Network

Disease Context: Research Results

Musculoskeletal Diseases: 719
- Bone Diseases: 5863
  - Developmental Bone Diseases: 153
    - Osteochondrodysplasias: 592
      - Fibrous Dysplasia of Bone: 78
        Polyostotic Fibrous Dysplasia: 407
        Cherubism: 108
        Craniofacial Fibrous Dysplasia: 37
        Monostotic Fibrous Dysplasia: 25
        Florid cemento-osseous dysplasia: 4
        Camptodactyly, fibrous tissue hyperplasia, and skeletal dysplasia
        Lichtenstein syndrome
        Panostotic fibrous dysplasia

Related Diseases

1.	Pain (Aches)
2.	Polyostotic Fibrous Dysplasia (McCune Albright Syndrome)
3.	Neoplasms (Cancer)
4.	Precocious Puberty
5.	Osteoporosis

Experts

1.	Collins, Michael T: 9 articles (01/2019 - 09/2003)
2.	Chapurlat, Roland: 5 articles (01/2022 - 09/2008)
3.	Riminucci, Mara: 5 articles (10/2017 - 09/2003)
4.	Robey, Pamela G: 5 articles (10/2017 - 04/2004)
5.	Boyce, Alison M: 4 articles (01/2019 - 11/2014)
6.	Bianco, Paolo: 4 articles (11/2014 - 09/2003)
7.	Lala, R: 4 articles (08/2002 - 02/2000)
8.	Matarazzo, P: 4 articles (08/2002 - 02/2000)
9.	Bastepe, Murat: 3 articles (01/2018 - 02/2011)
10.	Lala, Roberto: 3 articles (01/2018 - 05/2006)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Fibrous Dysplasia of Bone:

1.	Denosumab FDA Link 01/01/2023 - "Safety and Efficacy of Denosumab for Fibrous Dysplasia of Bone." 02/01/2014 - "Rapid biochemical response to denosumab in fibrous dysplasia of bone: report of two cases." 12/01/2014 - "Transient improvement of severe pain from fibrous dysplasia of bone with denosumab treatment." 10/01/2023 - "Letter regarding - Denosumab use in bone fibrous dysplasia refractory to bisphosphonate: A retrospective multicentric study." 01/01/2023 - "Denosumab use in bone fibrous dysplasia refractory to bisphosphonate: A retrospective multicentric study."
2.	pegvisomant FDA LinkGeneric 08/01/2006 - "We examined efficacy of the GH receptor antagonist, pegvisomant, in controlling gsp oncogene-mediated GH excess and skeletal disease (fibrous dysplasia of bone) associated with MAS. "
3.	Diphosphonates (Bisphosphonates)IBA 01/01/2021 - "Bisphosphonates for the treatment of fibrous dysplasia of bone." 02/01/2000 - "Little data have been collected regarding bisphosphonate treatment of bone fibrous dysplasia in paediatric patients with this syndrome. " 01/01/2019 - "Age-Related Changes and Effects of Bisphosphonates on Bone Turnover and Disease Progression in Fibrous Dysplasia of Bone." 10/01/2017 - "Bisphosphonates (BPs) are currently used in the treatment of diverse bone diseases including fibrous dysplasia of bone (FD). " 10/01/2017 - "Bisphosphonate-induced zebra lines in fibrous dysplasia of bone: histo-radiographic correlation in a case of McCune-Albright syndrome."
4.	Alendronate (Alendronate Sodium)FDA LinkGeneric 11/01/2014 - "A randomized, double blind, placebo-controlled trial of alendronate treatment for fibrous dysplasia of bone." 12/01/2006 - "In open studies, bisphosphonate therapy (pamidronate, alendronate) reduced bone pain associated with fibrous dysplasia of bone and was associated to some radiological improvement. "
5.	Pamidronate (Aredia)FDA LinkGeneric 09/01/2008 - "We report on a young boy initially diagnosed with fibrous dysplasia of bone, who underwent renal investigation because of treatment with pamidronate. " 07/01/2004 - "Treatment of fibrous dysplasia of bone with intravenous pamidronate: long-term effectiveness and evaluation of predictors of response to treatment." 10/01/1997 - "Long-term effects of intravenous pamidronate in fibrous dysplasia of bone." 04/16/1994 - "Long-term effects of intravenous pamidronate in fibrous dysplasia of bone." 01/01/2006 - "To examine outcomes of pamidronate treatment on fibrous dysplasia of bone in three children with McCune-Albright syndrome (MAS). "
6.	Interleukin-6 (Interleukin 6)IBA 01/01/2022 - "Inhibition of IL-6 in the treatment of fibrous dysplasia of bone: The randomized double-blind placebo-controlled TOCIDYS trial." 09/01/2003 - "Osteoclastogenesis in fibrous dysplasia of bone: in situ and in vitro analysis of IL-6 expression." 01/01/2022 - "Increased interleukin-6 (IL-6) has been observed in the bone tissue of fibrous dysplasia of bone/McCune-Albright syndrome (FD/MAS) and is possibly involved in the increased bone destruction and bone pain characterizing this disease. "
7.	MicroRNAs (MicroRNA)IBA 10/01/2020 - "A Signature of Circulating miRNAs Associated With Fibrous Dysplasia of Bone: the mirDys Study."
8.	Phosphates (Orthophosphate)IBA 09/01/2003 - "FGF-23 in fibrous dysplasia of bone and its relationship to renal phosphate wasting." 05/01/2006 - "Additionally, there are non-endocrine manifestations, such as fibrous dysplasia of bone (FD), renal phosphate wasting, and skin hyperpigmentation, i.e. " 09/01/2003 - "Renal phosphate wasting occurs in approximately 50% of patients with McCune-Albright syndrome (MAS) and fibrous dysplasia of bone (FD), which result from postzygotic mutations of the GNAS1 gene. " 05/01/2001 - "Renal phosphate wasting in fibrous dysplasia of bone is part of a generalized renal tubular dysfunction similar to that seen in tumor-induced osteomalacia." 01/01/2012 - " We describe a boy with a molecularly confirmed MAS, clinically evident with congenital café-au-lait spots, bone fibrous dysplasia, hyperthyroidism, and renal phosphate wasting syndrome. "
9.	GTP Phosphohydrolases (GTPases)IBA 06/01/2002 - "Mutations of Gs(alpha) residues involved in the GTPase reaction that lead to constitutive activation are present in endocrine tumors, fibrous dysplasia of bone, and McCune-Albright syndrome. " 11/04/2011 - "Although isoproterenol-induced cAMP response was not prolonged by XLαs expression, a GTPase-deficient XLαs mutant found in certain tumors and patients with fibrous dysplasia of bone and McCune-Albright syndrome generated more basal cAMP accumulation in HEK293 cells and caused more severe impairment of osteoblastic differentiation of MC3T3-E1 cells than the cognate Gαs mutant (gsp oncogene). " 12/01/2004 - "Somatic activating G(s)alpha mutations, which alter key residues required for the GTPase turn-off reaction, are present in various endocrine tumors and fibrous dysplasia of bone, and in a more widespread distribution in patients with McCune- Albright syndrome. " 10/01/2001 - "Mutations of specific G(s)alpha residues (Arg(201) or Gln(227)) that are critical for the GTPase reaction lead to constitutive activation of G(s)-coupled signaling pathways, and such somatic mutations are found in endocrine tumors, fibrous dysplasia of bone, and the McCune-Albright syndrome. "
10.	Growth Hormone (Somatotropin)IBA 11/15/2018 - "Mosaic Effects of Growth Hormone on Fibrous Dysplasia of Bone." 03/01/1999 - "The potential role of chronic growth hormone overproduction in the occurrence of malignant transformation and the possible value of bisphosphonates in the treatment of bone fibrous dysplasias are discussed." 01/01/2018 - "Growth hormone-Insulin-like growth factor 1 axis hyperactivity on bone fibrous dysplasia in McCune-Albright Syndrome." 02/01/2002 - "To date, mutations at the codon encoding R201, typically underlying the McCune-Albright syndrome and isolated fibrous dysplasia of bone, have been demonstrated only in growth hormone secreting pituitary adenomas. "

Therapies and Procedures

1.	Therapeutics 12/01/2006 - "Medical therapy in adults with fibrous dysplasia of bone." 04/28/2022 - "Effects of zoledronic acid therapy in fibrous dysplasia of bone: a single-center experience." 06/01/1948 - "Testosterone therapy in a case of fibrous dysplasia of bone with hypogenitalism." 11/01/1947 - "Testosterone therapy in a case of fibrous dysplasia of bone with hypogenitalism." 06/01/2017 - "Novel systemic therapies are available for fibrous dysplasia and GCT of the bone: Fibrous dysplasia can be treated with bisphosphonates, and GCT responds to denosumab. "
2.	Bone Transplantation (Bone Grafting) 03/01/1977 - "Pathologic subtrochanteric fracture in a femur rarefied by fibrous dysplasia of bone was successfully treated with condylocephalic nailing and bone grafting. " 01/01/2001 - "From January 1994 to June 1999, 148 cases of bone defect were repaired by embryonic bone transplantation following alcohol treatment, there were 63 cases with bone cyst, 42 cases with fibrous dysplasia of bone, 26 cases with giant cell tumor of bone, and 17 cases with enchondroma among them. "
3.	Curettage 03/01/2000 - "Intralesional curettage and reconstruction with autogenous iliac crest graft in fibrous dysplasia of bone leads to a high recurrence rate. " 04/01/2008 - "From January 2003 to December 2005, 61 cases of various bone defects following the curettage of the benign bone tumors were treated with interporous TCP, with 33 males and 28 females, including bone fibrous dysplasia in 8 cases, bone cyst in 23 cases, eosinophilic granuloma in 12 cases, enchondroma in 13 cases, non-ossifying fibroma in 2 cases, and osteoblastoma in 3 cases. "
4.	Radiotherapy 01/01/2011 - "Radiation therapy (RT) efficaciously controlled GH hypersecretion, however, it may have been the cause of the malignant transformation of the dysplastic bone tissue, which eventually caused brain hernia and death; autopsy demonstrated that the cranium had significant thickening (as much as 10 cm), the pathological diagnosis was fibrous dysplasia of bone associated with chondrosarcoma; and undifferentiated chondrosarcoma with malignant fibrous histocytoma subtype in the sellar region; nodular goiter with the thyroid gland, one nodus was pathologically demonstrated as papillary carcinoma. "
5.	Ovariectomy (Oophorectomy) 08/01/2010 - "McCune-Albright syndrome (MAS): early and extensive bone fibrous dysplasia involvement and "mistaken identity" oophorectomy."