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Fibrous Dysplasia in Hospitalized Patients in France: Prevalence and Associated Manifestations from a Health Database
Lucie Levaillant1,2, Régis Coutant1,3, Patrice Rodien2,3,4
1Department of Pediatric Endocrinology and Diabetology, University Hospital of Angers, Endo-ERN Center for Rare Endocrine Conditions, 49933 Angers Cedex 9, France.
Introduction:
Fibrous dysplasia (FD)/McCune-Albright syndrome (MAS) is a rare mosaic disorder involving the skeleton and/or extraskeletal manifestations, particularly endocrine features. Population-based estimates of prevalence and incidence are scarce, including in France, and data on associated manifestations mainly derive from specialized centres. This study aimed to estimate the prevalence and incidence of FD/MAS in hospitalized patients in France and to evaluate the frequency of associated manifestations.
Methods:
This retrospective cohort study used the French National Health Insurance Database, to identify all patients hospitalized between 2012 and 2024 with a primary or associated diagnosis of monostotic FD (MFD; ICD-10 M85.0) or polyostotic FD/MAS (PFD/MAS; ICD-10 Q78.1). Prevalence and incidence were calculated for 2024. Associated manifestations were assessed using hospitalization diagnoses and outpatient drug dispensing data.
Results:
Over the 2012-2024 period, 2249 patients were hospitalized in France; 60% had PFD/MAS, 63% were female, and 26% were children. The 2024 prevalence was 5.36 per 1,000,000 inhabitants (95% CI: 4.83-5.94), and the 2024 incidence was 2.58 patients per 1,000,000 inhabitants (95% CI: 2.21-2.99). Regarding associated manifestations, 27% of patients per year received bisphosphonates, 7.4% received phosphate supplements, 3.3% aromatase inhibitors, 8% pituitary treatments and 2.2% antithyroid agents.
Conclusion:
This nationwide study provides the first estimates of the prevalence and incidence of hospitalized FD/MAS in France, although estimates based on hospitalization data likely underestimate the true frequency of monostotic FD. Treatment-based proxies offer valuable insights into associated manifestations. Improved outpatient coding and integration of clinical data are needed to better capture the full spectrum of FD/MAS at the population level.
Insights
This study estimates the prevalence of Fibrous Dysplasia (FD)/McCune-Albright Syndrome (MAS) in France, finding 5.36 cases per million. Treatment data reveals common associated manifestations, highlighting the need for better data collection.
Area of Science:
- Endocrinology
- Genetics
- Public Health
Background:
- Fibrous dysplasia (FD)/McCune-Albright syndrome (MAS) is a rare genetic disorder affecting bones and endocrine systems.
- Existing data on FD/MAS prevalence and manifestations are limited, particularly in France.
Purpose of the Study:
- To estimate the prevalence and incidence of FD/MAS in hospitalized patients in France.
- To assess the frequency of associated manifestations in FD/MAS patients.
Main Methods:
- Retrospective cohort study using the French National Health Insurance Database (2012-2024).
- Identified patients with monostotic FD (MFD) or polyostotic FD/MAS (PFD/MAS) diagnoses.
- Calculated prevalence and incidence for 2024; analyzed manifestations via diagnoses and drug dispensing data.
Main Results:
- 2249 patients hospitalized between 2012-2024; 60% had PFD/MAS, 63% female, 26% children.
- 2024 prevalence: 5.36/million (95% CI: 4.83-5.94); 2024 incidence: 2.58/million (95% CI: 2.21-2.99).
- Annual treatment rates: bisphosphonates (27%), phosphate supplements (7.4%), aromatase inhibitors (3.3%), pituitary treatments (8%), antithyroid agents (2.2%).
Conclusions:
- First nationwide estimates of hospitalized FD/MAS prevalence and incidence in France.
- Hospitalization data likely underestimates monostotic FD frequency.
- Treatment data provides insights into manifestations; improved outpatient coding and clinical data integration are recommended.
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