Epidemiology

Frequency

Demographics of FOP

ü The prevalence of Fibrodysplasia Ossificans Progressiva (FOP) has been estimated at 1 case per 2 million persons.

ü 700 confirmed cases across the globe, from an estimated 2500.

ü  285 known cases in the United States and 45 in the UK.

Mortality/Morbidity

In most cases FOP (Fibrodysplasia Ossificans Progressiva) usually starts in early infancy with episodes of soft tissue swelling, although skeletal deformations are present at birth. The prognosis is poor because of the involvement of thoracic muscles, leading to restrictive lung disease. Most of the patients become bedridden by time they are in their 30’s.

Race

FOP mainly occurs in whites, but it is also reported in blacks.

Sex

FOP is more common in females than in males. The observed male-to-male transmission of the disorder excludes X-linked inheritance. Because few individuals who are affected choose to have children, most patients are considered to have new mutations.

Age

FOP usually starts in early infancy; however, reports exist of in utero involvement and skeletal deformations are present at birth.