Description

ter Haar et al developed the Autoinflammatory Disease Damage Index (ADDI) to evaluate a patient with an autoinflammatory disease. The authors are from multiple hospitals from around the world.


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.


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