Table 5. [Clinical Staging of Fibrodysplasia Ossificans Progressiva]. - GeneReviews®

Feature	Clinical Stage
History of flare-ups	None; or if present, limited to scalp, neck, or back	Mostly limited to axial regions & upper limbs	In any location	In any location	In any location
Body regions affected	Neck, back, upper limbs	Neck, back, chest, upper & lower limbs	Neck, back, chest, upper & lower limbs, jaw	Neck, back, chest, upper & lower limbs, jaw & distal limbs (wrists & ankles)	Ankyloses of most or all joints
Thoracic insufficiency (TI)		Limited chest expansion	Rigid chest wall, no chest expansion, diaphragmatic breathing	Symptomatic TI syndrome (pulmonary hypertension & right-sided heart failure)	SymptomaticTI syndrome (pulmonary hypertension & right-sided heart failure)
Other complications				Pneumonia, pressure ulcers	Recurrent respiratory infections
ADL	No or minimal assistance required due to mild joint limitations or physical delay in developmental milestones	Some assistance required	Assistance needed for most activities	Dependent for all ADL	Dependent for all ADL
Ambulation	Unaffected or cannot evaluate due to very young age	Walks; may use wheelchair in extenuating circumstances (e.g., long distances)	Walks w/assistive device &/or uses wheelchair	Wheelchair bound	Mostly bed bound
CAJIS	≤4	5-18	19-24	≥24	≥28

Feature

Clinical Stage

Early/Mild

Moderate

Late/Severe

Profound

End Stage

History of flare-ups

None; or if present, limited to scalp, neck, or back

Mostly limited to axial regions & upper limbs

In any location

Body regions affected

Neck, back, upper limbs

Neck, back, chest, upper & lower limbs

Neck, back, chest, upper & lower limbs, jaw

Neck, back, chest, upper & lower limbs, jaw & distal limbs (wrists & ankles)

Ankyloses of most or all joints

Thoracic insufficiency (TI)

Limited chest expansion

Rigid chest wall, no chest expansion, diaphragmatic breathing

Symptomatic TI syndrome (pulmonary hypertension & right-sided heart failure)

SymptomaticTI syndrome (pulmonary hypertension & right-sided heart failure)

Other complications

Pneumonia, pressure ulcers

Recurrent respiratory infections

ADL

No or minimal assistance required due to mild joint limitations or physical delay in developmental milestones

Some assistance required

Assistance needed for most activities

Dependent for all ADL

Ambulation

Unaffected or cannot evaluate due to very young age

Walks; may use wheelchair in extenuating circumstances (e.g., long distances)

Walks w/assistive device &/or uses wheelchair

Wheelchair bound

Mostly bed bound

CAJIS

≤4

5-18

19-24

≥24

≥28

From: Fibrodysplasia Ossificans Progressiva

GeneReviews^® [Internet].

Adam MP, Feldman J, Mirzaa GM, et al., editors.

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