Table 1

Demographic and clinical data, including ADDI, are summarized in the table.

HS patients group (n=47)Percentage (%)
Age ( μ±σ )39,64±15,16x
Sex (male/female )25/2253,19/46,81
Body Mass index(BMI) ( μ±σ kg/m2 )28,68±3,21x
Disease duration ( μ±σ years )7,27±4,98x
Hurley stage III 47100
HS-Sonografic scoring (SOS) stage III 47100
HS- Physician Global
Assessment (PGA)
  • Clear:

  • Minimal:

  • Mild:

  • Moderate:

  • Severe:

  • Very severe:

  • 0

  • 0

  • 0

  • 5

  • 24

  • 18

  • 0

  • 0

  • 0

  • 10,64

  • 51,06

  • 38,3

Dermatological Life Quality Index (DLQI) (μ±σ )22,83±3,13x
Autoinflammatory disease damage index (ADDI) itemsHS patientsPercentage (%)
Reproductive 2246,80
Sub/infertility 1736,17
Amenorrhoea 510,64
Renal/amyloidosis 1225,53
Amyloidosis
  •  Limited

  •  Extended

  • 0

  • 2

  • 0

  • 4,26

Proteinuria 714,89
Renal insufficiency
  •  Moderate renal insufficiency

  •  Severe renal insufficiency

  • 2

  • 1

  • 4,26

  • 2,13

Developmental 1838,3
 Growth failure 36,38
 Puberty delay 1531,91
Serosal 36,38
Serosal scarring 36,38
Neurological 919,15
Developmental delay 00
Cognitive impairment* 919,15
Elevated intracranial pressure 00
Central nervous system involvement 00
Ears 12,13
Hearing loss
  • Moderate hear loss of better ear

  • Severe hear loss of better ear

  • 1

  • 0

  • 2,13

  • 0

Ocular 1123,40
Ocular involvement
  • Mild ocular involvement of better eye

  • Moderate ocular involvement of better eye

  • Severe ocular involvement of better eye

  • 3

  • 5

  • 3

  • 6,38

  • 10,64

  • 6,38

Musculoskeletal 4697,87
Joint restriction 1225,53
Bone deformity 612,77
Osteoporosis 2042,55
Musculoskeletal pain 4493,62
ADDI Points ( μ±σ )7,27±4,98x
Autoimmune/Autoinflammatory comorbidities 12
Chron's disease: 6
Pyoderma gangrenosum: 2
Spondyloarthropathy :2
Psoriasis: 1
Hashimoto's thyroiditis: 1
25,53
  • * Cognitive impairment was calculated as positive if IQ<80 as defined by neuropsychological assessment, namely Wechsler Intelligence Scale for Adults (WAIS) IV, or if Mini-Mental State Examination (MMSE) results in a score<23.