Scleroderma Health Assessment Questionnaire (SHAQ)

Systemic Sclerosis: (Genrinho et al., 2023; n = 102, mean age = 57 (12.5) years, female = 84 (82.4%), mean disease duration since diagnosis = 5.6 (6.0) years, Portuguese translation of the SHAQ, calculated SEM)

• HAQ-DI: 0.119
• S-HAQ Pain VAS: 10.423
• S-HAQ Intestinal VAS: 8.591
• S-HAQ Breathing VAS: 12.471
• S-HAQ Raynaud’s VAS: 18.032
• S-HAQ Finger ulcer VAS: 11.86
• S-HAQ Overall disease severity VAS: 9.228

Systemic Sclerosis: (Genrinho et al., 2023; calculated MDC at 95% CI)

• HAQ-DI: 0.33
• S-HAQ Pain VAS: 28.89
• S-HAQ Intestinal VAS: 23.81
• S-HAQ Breathing VAS: 34.57
• S-HAQ Raynaud’s VAS: 49.98
• S-HAQ Finger ulcer VAS: 32.87
• S-HAQ Overall disease severity VAS: 25.58

Systemic Sclerosis: (Genrinho et al., 2023; n = 102)

• Most common subtype of Scleroderma: Limited Scleroderma n = 64
• Most common symptoms:
  • Raynaud’s phenomenon n = 96 (94.1%)
  • Abnormal nail fold capillary  n = 81 (79.4%)
  • Sclerodactyly  n = 65 (63.7)
  • Telangiectasia  n = 64 (62.7)

Summary data for the Scleroderma Health Assessment Questionnaire Disability Index (SHAQ) (n = 102)

SHAQ Scale

Min

Max

Mean

Std. Dev.

HAQ-DI

0.00

2.13

0.58

0.51

Pain VAS

0.00

85.0

31.7

22.8

Intestinal VAS

0.00

95.0

16.0

24.8

Breathing VAS

0.00

84.0

17.4

24.6

Raynaud’s VAS

0.00

100.0

26.2

28.8

Finger ulcer VAS

0.00

100.0

18.3

27.8

Overall disease severity VAS

0.00

90.0

35.2

25.4

HAQ-DI: Health Assessment Questionnaire-Disability Index; VAS: Visual Analog Scale

Systemic Sclerosis: (Merkel et al., 2002; n = 281, mean age = 50.4 (11.6), female = 248 (88.3%), mean disease duration since diagnosis = 8.5 (7.3) years)

Scores on SHAQ scales at baseline (mean range = 0.0-3)

SHAQ Scale

Mean

Std. Dev.

HAQ-DI

0.86

0.63

Pain VAS

1.19

0.75

Intestinal VAS

0.53

0.71

Breathing VAS

0.43

0.65

Raynaud’s VAS

1.15

0.87

Finger ulcer VAS

0.75

0.93

Overall disease severity VAS

1.17

0.77

HAQ-DI: Health Assessment Questionnaire-Disability Index; VAS: Visual Analog Scale

Systemic Sclerosis: (Steen & Medsger, 1997; n = 1,250, diffuse cutaneous involvement = 55%, mean disease duration from first symptom to first HAQ = 5.6 years)

Baseline mean disability index (HAQ-DI) scores in selected systemic sclerosis (SSc) patient subsets

Patient subsets

Mean DI*

Diffuse SSc (n = 647)

1.09

Limited SSc (n = 520)

0.72

Early diffuse SSc (<5 years duration) (n = 222)

1.22

Late diffuse SSc (>5 years duration) (n = 232)

0.96

Early limited SSc (<5 years duration) (n = 120)

0.37

Late limited SSc (>5 years duration) (n = 400)

0.76

Joint pain present (n = 505)

1.29

Joint pain absent (n = 603)

0.68

High total skin score (>20) (n  = 210)

1.26

Low total skin score (<20) (n  = 404)

0.62

Diffuse SSc, heart or kidney involved (n = 74)

1.36

Diffuse SSc, heart or kidney uninvolved (n = 573)

0.68

HAQ-DI: Health Assessment Questionnaire-Disability Index

*p < 0.001 for all patient subset pairs

Systemic Sclerosis: (Genrinho et al., 2023)

• Excellent test-retest reliability for HAQ-DI (ICC = 0.946)
• Acceptable test-retest reliability for SHAQ VAS scales (ICC):
  • Pain (0.791)
  • Intestinal (0.880)
  • Breathing (0.743)
  • Finger ulcer (0.818)
  • Overall disease severity (0.868)
• Poor test-retest reliability for Reynaud’s VAS (ICC = 0.608)

Systemic Sclerosis: (Genrinho et al., 2023, n = 102)

• Excellent: Cronbach’s alpha for the HAQ-DI = 0.866

Concurrent validity:

Systemic Sclerosis: (Genrinho et al., 2023)

• Excellent concurrent validity of the HAQ-DI (all p < 0.001) with:
  • EQ-5D-5L (r = -0.723)
  • EQ-VAS (-0.645)
  • SF-36v2 Physical Summary Measure (-0.688)
• Adequate concurrent validity of the HAQ-DI with the SF-36v2 Mental Summary Measure (r = -0.337, p = 0.001) and UCLA Scleroderma Clinical Trial Consortium Gastrointestinal Tract Instrument total score (r = 0.392, p < 0.001)

Systemic Sclerosis: (Smyth et al., 2003; n = 115 (75 w/limited cutaneous scleroderma, mean age = 57 (12.6), mean duration of scleroderma = 10.38 (12.8) years; 40 w/diffuse cutaneous scleroderma, mean age = 49.3 (12.8), mean duration of scleroderma = 6.5 (5.2) years)

• Excellent correlation between the HAQ-DI and the UK scleroderma Functional Score (UKFS) (r = 0.9)
• Excellent correlation between the visual analogue scales (VAS) for overall disease severity (r = 0.68*) and pain (r = 0.66*) with the HAQ-DI
• Excellent correlation between the VAS for overall disease severity (r = 0.72*) and pain (r = 0.66*) with the UKFS

*p < 0.001

Predictive validity:

Systemic Sclerosis: (Steen & Medsger, 1997; n = 285 patients with diffuse cutaneous involvement and a symptom duration of <3 years)

• Initial erythrocyte sedimentation rate and initial HAQ-DI were the most accurate tests in predicting survival

Multiple regression of first-visit features predicting death in patients with early diffuse systemic sclerosis

First-visit features

Correlation coefficient

P

Initial total skin score

0.325

0.328

Palpable tendon friction rubs

1.089

0.0005

Severe heart or kidney involvement

0.977

0.001

FVC % predicted

0.981

0.002

DLco % predicted

0.689

0.022

Serum anti-topoisomerase I antibody

0.072

0.763

Erythrocyte sedimentation rate

0.965

0.0001

HAQ Disability Index

1.118

<0.0001

FVC = forced vital capacity; DLco = diffusing capacity for carbon monoxide; HAQ = Health Assessment Questionnaire

Discriminant validity:

Systemic Sclerosis: (Genrinho et al., 2023)

• Significantly worse HAQ-DI scores for known groups: Males (t = 2.204, p = 0.030), Patients ≥ 65 years of age (F = 3.245, p  = 0.043), and Patients with the diffuse form of systemic sclerosis (t = 2.653, p = 0.011)

“Content validity was tested through a clinical review involving two rheumatologists and a cognitive debriefing conducted with two panels of five patients each. Both procedures did not result in any changes to the Portuguese version (Genrinho et al., p. 5).”