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
Specialty: Immunology/Rheumatology