Autoinflammatory Disease Damage Index (ADDI) of ter Haar et al

Patient selection: autoinflammatory disease

 

Parameters:

(1) fertility unrelated to partner

(2) amenorrhea if female

(3) amyloidosis

(4) proteinuria (persistent urinary protein to creatinine ratio > 20 mg/mmol in the morning void; daily protein excretion > 0.3 g per 24 hours; urine albumin to creatinine ratio > 15 mg/mmol)

(5) renal insufficiency

(6) growth failure (>= 2 of the following: lower than 3rd percentile height for age; growth velocity over 6 months less than 3rd percentile for age; crossing at leaset two centiles (5%, 10%, 25%, 50%, 75%, 90%, 95%) on growth chart

(7) puberty delay (Tanner stage below -2 SD for age)

(8) serosal scarring (adhesions or fibrosis)

(9) developmental delay (failure to reach age-appropriate developmental milestones)

(10) cognitive impairment

(11) elevated intracranial pressure

(12) central nervous system involvement

(13) hearing loss of better ear

(14) ocular involvement of better eye

(15) joint restriction (fixed limitation in normal range of motion)

(16) bone deformity or overgrowth

(17) osteoporosis (confirmed by densitometry or fracture)

(18) musculoskeletal pain (non-inflammatory, impairs activities of daily living)

 

Parameter	Findings	Points
fertility	normal	0
	subfertility	2
	infertility	2
amenorrhea if female	no	0
	yes	1
amyloidosis	none	0
	limited	2
	extensive	3
proteinuria	absent	0
	present	1
renal insufficiency	none	0
	mild	0
	moderate (GFR 15 to 60 mL per min per 1.73 sq m)	2
	severe (GFR < 15 mL per min per 1.73 sq m or dialysis or transplant)	3
growth failure	no	0
	yes	2
puberty delay	no	0
	yes	1
serosal scarring	absent	0
	present	1
developmental delay	absent	0
	present	2
cognitive impairment	no	0
	yes	3
elevated intracranial pressure	no	0
	yes	2
central nervous system involvement	absent	0
	present	3
hearing loss of better ear	none	0
	mild	0
	moderate	1
	severe	2
ocular involvement of better eye	none	0
	mild	1
	moderate	2
	severe	3
joint restriction	no	0
	yes	2
bone deformity	absent	0
	present	2
osteoporosis	absent	0
	present	1
musculoskeletal pain	absent	0
	present	1

 

subscore for reproductive system =

= (points for fertility) + (points for amenorrhea)

 

subscore for renal system or amyloidosis =

= MIN(6, (points for amyloidosis) + (points for proteinuria) + (points for renal insufficiency))

 

subscore for developmental system =

= (points for growth failure) + (points for puberty delay)

 

subscore for serosal system =

= (points for serosal scarring)

 

subscore for neurological system =

= MIN(6, (points for developmental delay) + (points for cognitive impairment) + (points for elevated intracranial pressure) + (points for CNS involvement))

 

subscore for ears =

= (points for hearing loss)

 

subscore for ocular system =

= (points for ocular involvement)

 

subscore for musculoskeletal system =

= MIN(4, (points for joint restriction) + (points for bone deformity) + (points for osteoporosis) + (points for musculoskeletal pain))

 

total score =

= SUM(points for all 8 subscores)

 

Interpretation:

• minimum score: 0

• maximum score: 28

• The higher the score the greater the damage.