Table 2

The Relapsing Polychondritis Disease Activity Index (RPDAI), adapted from Arnaud et al 9

PointsPoints
Constitutional symptomsCutaneous manifestations
2 Fever (>38°C/100.4°F)3 Purpura
Rheumatological manifestationsRenal manifestations
1 Arthritis4 Haematuria
Chondritides6 Proteinuria
3 Manubriosternal chondritis17 Renal failure
4 Sternoclavicular chondritisCardiovascular manifestations
4 Costochondritis9 Pericarditis
9 Auricular chondritis (can be unilateral or bilateral)16 Large-sized and/or medium-sized vessel involvement
9 Nasal chondritis17 Myocarditis
Ophthalmological manifestations18 Acute aortic or mitral insufficiency
5 EpiscleritisNeurological manifestations
9 Scleritis12 Motor or sensorimotor neuropathy
9 Uveitis22 Encephalitis
11 Corneal ulcerRespiratory manifestations
14 Retinal vasculitisRespiratory chondritis (laryngeal and/or tracheal and/or bronchial chondritis)
Biological data14 Without acute respiratory failure
3 Raised C reactive protein (>20 mg/L)24 With acute respiratory failure
ENT manifestations Other (please explain):
8 Sensorineural deafness 
12 Vestibular dysfunction 
 TOTAL RPDAI SCORE

  • The scoring should only take into account manifestations attributable to relapsing polychondritis and present during the last 28 days.

  • ENT, ear, nose and throat.