Bone Abstracts

Fibrous dysplasia of bone (FD) is a rare inherited but sporadic bone disease that can be responsible for bone pain, fracture and bone deformity. The prognosis may be difficult to establish because of the wide spectrum of disease severity, with patients benign forms of the disease and some others who are severely affected.

We have analyzed the data from the French National reference center for FD. We have established a database from electronic medical records. We have made descriptive statistics of the various forms of FD and examined the prognostic factors by multivariable logistic regression analysis, with a parcimonious stepwise method. The primary outcome was a clinically relevant composite index combining bone pain (analogic scale >3) and/or incident fracture.

In our modern cohort of 372 patients, the median age at diagnosis was 23. The revealing symptom (median age=18) was bone pain in 44% of patients, a fracture in 9% but the diagnosis was fortuitous in 25% of cases. Monostotic forms represented 58% of patients and polyostotic forms 42%. The femur was the most commonly affected bone (44% of patients), followed by the skull (38%). Twelve percent of patients had McCune-Albright syndrome (MAS). With a median duration of follow-up of 7 years among 211 patients, we observed an incidence of fracture of 17 and 51% of patients had no bone pain at the end of follow-up. In univariate analysis, younger age at diagnosis, renal phosphate wasting, a polyostotic form and prevalent fracture were predictors of a poorer prognosis, but in the multivariate model, the polyostotic form was the only significant predictor (OR=2.04 [1.29–3.27]).

In conclusion, in a national referral center for FD, one patient on follow-up out of six had incident fracture. A polyostotic form was the main risk factor of a poorer outcome.