Description

Mertz et al reported the Relapsing Polychondritis Damage Index (RPDAM) as a disease-specific damage score. This can help to monitor the patient and the response to therapy. The authors are from multiple institutions from around the world.


Domains:

(1) ear

(2) nose

(3) eye

(4) respiratory tract

(5) cardiovascular

(6) hematological

(7) treatment related

 

Damage was defined as an irreversible disease manifestation that is unlikely to benefit from disease escalation AND present for >= 3 months.

 

The onset of damage should be after the diagnosis of onset of relapsing polychondritis.

 

Domain

Damage Item

ear

permanent auricular cartilage deformities

 

sensorineural hearing loss

 

conductive hearing loss

 

permanent vestibular syndrome

nose

nasal bridge collapse or septal perforation

eye

scleromalacia (scleral thinning)

 

blindness in one or both eyes

 

orbital wall destruction

respiratory tract

permanent chest wall deformities

 

dysponia

 

obstructive syndrome (laryngeal, tracheal, bronchial)

cardiovascular

aortic root dilatation

 

left ventricular dysfunction

 

major tissue loss

hematological

myelodysplasia with transfusion dependency

treatment-related

osteoporosis with fractures or vertebral collapse

 

avascular necrosis

 

damage index =

= SUM(number of items present)

 

Interpretation:

• minimum index: 0

• maximum index: 17


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