Table 1.

Intraclass correlation coefficient (ICC) of the total ADDI score in all patients (overall), the ICC of the ADDI in the four different diseases and separate ICCs for each category and damage item. Numbers in brackets indicate the 95% CI

	ICC (95% CI)
Overall	0.84 (0.78 to 0.89)
Per disease
CAPS	0.82 (0.71 to 0.91)
TRAPS	0.62 (0.39 to 0.80)
FMF	0.84 (0.72 to 0.92)
MKD	0.73 (0.55 to 0.86)
Per category
Reproductive	0.67 (0.59 to 0.76)
Sub/infertility	0.72 (0.63 to 0.79)
Amenorrhea	0.57 (0.46 to 0.67)
Renal/amyloidosis	0.88 (0.84 to 0.92)
Amyloidosis	0.76 (0.69 to 0.82)
Proteinuria	0.80 (0.73 to 0.85)
Renal insufficiency	0.84 (0.78 to 0.88)
Developmental	0.54 (0.43 to 0.64)
Growth failure	0.57 (0.46 to 0.67)
Puberty delay	0.29 (0.16 to 0.43)
Serosal	0.64 (0.54 to 0.72)
Serosal scarring	0.64 (0.54 to 0.72)
Neurological	0.75 (0.67 to 0.81)
Developmental delay	0.48 (0.37 to 0.60)
Cognitive impairment	0.54 (0.43 to 0.65)
Elevated ICP	0.65 (0.56 to 0.74)
CNS involvement	0.67 (0.58 to 0.75)
Ears	0.86 (0.82 to 0.90)
Hearing loss	0.86 (0.82 to 0.90)
Ocular	0.74 (0.66 to 0.80)
Ocular damage	0.74 (0.66 to 0.80)
Musculoskeletal	0.73 (0.64 to 0.80)
Joint restriction	0.52 (0.41 to 0.63)
Bone deformity	0.74 (0.66 to 0.81)
Osteoporosis	0.86 (0.81 to 0.90)
Musculoskeletal pain	0.47 (0.35 to 0.58)

ADDI, Autoinflammatory Disease Damage Index; CAPS, cryopyrin-associated periodic syndrome; CNS, central nervous system; FMF, familial Mediterranean fever; ICP, intracranial pressure; MKD, mevalonate kinase deficiency; TRAPS, tumour necrosis factor receptor-associated periodic syndrome.