Skip to main content
. Author manuscript; available in PMC: 2021 Sep 2.
Published in final edited form as: Ann Rheum Dis. 2018 Aug 4;77(11):1599–1605. doi: 10.1136/annrheumdis-2018-213725

Table 2.

Definitive ADDI including glossary of terms

Damage item Grading* Points†
Reproductive Max 2
 Sub/infertility 2
 Amenorrhea 1
Renal/amyloidosis Max 6
 Amyloidosis Limited/extensive1 2/3
 Proteinuria 1
 Renal insufficiency Moderate/severe2 2/3
Developmental Max 3
 Growth failure 2
 Puberty delay 1
Serosal Max 1
 Serosal scarring 1
Neurological Max 6
 Developmental delay 2
 Cognitive impairment 3
 Elevated intracranial pressure 2
 Central nervous system involvement 3
Ears Max 2
 Hearing loss Moderate/severe3 1/2
Ocular Max 3
 Ocular involvement Mild/moderate/severe4 1/2/3
Musculoskeletal Max 4
 Joint restriction 2
 Bone deformity 2
 Osteoporosis 1
 Musculoskeletal pain 1

The total ADDI score is the sum of the eight categories (maximum 27 points).

*

Grading: scoring depends on the severity of damage:

1

Amyloidosis: limited, affecting one organ extensive, affecting more than one organ.

2

Renal insufficiency: moderate, glomerular filtration rate (GFR) between 15 and 60 mL/min/1.73 m2; severe, GFR <15 mL/min/1.73 m2, dialysis or transplantation.

3

Hearing loss: moderate, hearing impairment without requirement of hearing aids or a cochlear implant; severe, hearing impairment requiring hearing aids or a cochlear implant.

4

Ocular involvement: mild, ocular damage without visual impairment; moderate, with visual impairment; severe, legal blindness.

†

Points are given when the item is present. For items with grading in severity, the lowest score is given for mild involvement and the highest for severe involvement. For each category, the score is limited to a specific maximum.

ADDI, Autoinflammatory Disease Damage Index; Max, maximum.

Glossary of terms

Amenorrhea: Primary amenorrhea: absence of menarche at the age of 16 years or absence of menarche 5 years after the larche in a woman. Secondary amenorrhea: absence of the menses for six consecutive months or more in a woman who previously had menstrual cycles.

Amyloidosis: Symptomatic amyloidosis confirmed by examination of tissue sections by Congo red dye or serum amyloid P component (SAP) scintigraphy.

Bone deformity: Bone deformation or overgrowth on clinical examination and/or imaging studies.

Central nervous system involvement: Focal deficits (gross and/or fine sensorimotor), diffuse deficits (eg, memory, behaviour), seizures and spinal cord symptoms. Neuropsychiatric disorders unrelated to the disease should not be scored.

Cognitive impairment: Requirement of special education because of cognitive impairment or IQ below 70 as defined by neuropsychological assessment (eg, Wechsler Intelligence Scale for Children, WISC) or other age-appropriate equivalents.

Developmental delay$: Failure to reach age-appropriate developmental milestones, including language/speech, motor, social/emotional, and cognitive milestones.

Elevated intracranial pressure: Signs and/or symptoms of elevated intracranial pressure supported by appropriate techniques#.

Growth failure: Defined as the presence of at least two of the three features:
  • Lower than the third percentile or −2 SD height for age.
  • Growth velocity over 6 months lower than the third percentile or −2 SD for age.
  • Crossing at least two centiles (5%, 10%, 25%, 50%, 75%, 90%, 95%) on growth chart.

For patients older than 18 years: Pathological short stature (eg, below third percentile or −2 SD for normal ethnic population).

Hearing loss: Sensorineural hearing impairment of better ear, confirmed by audiometry or another age-appropriate technique, or requirement of hearing aids or a cochlear implant.

Infertility: A disease of the reproductive system defined by the failure to achieve a clinical pregnancy after 12 months or more of regular unprotected sexual intercourse, not due to known disorders in the unaffected partner.

Joint restriction: Fixed limitation in the normal range of motion of joints affecting function, with or without destructive arthropathy or avascular necrosis.

Musculoskeletal pain: Non-inflammatory musculoskeletal pain impairing activities of daily living.

Ocular involvement: Ocular damage (eg, optic nerve atrophy, elevated intraocular pressure or cataract) of better eye, documented by an ophthalmologist, with or without visual impairment.

Osteoporosis: Reduced bone mineral density with vertebral collapse and/or pathological fractures confirmed with imaging, which may include bone densitometry. Requires both evidence of decreased bone density and fracture, ‘low bone density’ by itself is insufficient.

Proteinuria: Persistent urinary protein-to-creatinine ratio of >20 mg/mmol in the first morning void; and/or a daily protein excretion of >0.3 g/24 hours, or urine albumin-to-creatinine ratio of >15 mg/mmol.

Puberty delay: A Tanner stage below −2 SDs for age or below the third percentile for age or any Tanner stage after pharmacological induction of puberty.

Renal insufficiency: Glomerular filtration rate (GFR) of <60 mL/min/1.73 m2, dialysis or transplantation.

Serosal scarring: Symptomatic adhesions or fibrosis affecting pericardium, pleura, peritoneum and/or retroperitoneum, supported by imaging techniques, endoscopy or surgery.

$Only for paediatric patients; #such as funduscopy, neuroimaging or lumbar cerebrospinal fluid (CSF) pressure measurement.