Table 2.

Core set items selected for 11 domains for the CRISS. Modified from Khanna, et al. Ann Rheum Dis 2008; 67:703–9, with permission.

Soluble biomarkers
  Acute phase reactants ESR and/or CRP
Cardiac
  Cardiac echocardiogram with Doppler
  Right heart catheterization
  6-Minute Walk Test*
  Borg Dyspnea Instrument*
Digital ulcers
  Active digital tip ulcer count on the volar surface
  VAS digital ulcer (part of Scleroderma HAQ-DI)
Gastrointestinal
  Body mass index
  Validated GI tract VAS or other SSc-validated GI questionnaire
Global health
  VAS/Likert scale for patient global severity
  VAS/Likert scale for physician global severity
  Scleroderma-related health transition by patient
  Scleroderma-related health transition by physician**
Health-related quality of life and function
  HAQ-DI
  VAS pain scale from the HAQ-DI
  Short Form-36 score, version 2
Musculoskeletal
  Tender Joint Count
  Tendon friction rubs assessed by the physician**
  Serum creatinine phosphokinase, aldolase
Pulmonary
  Pulmonary function testing
Validated measure of dyspnea
  Breathing VAS from the Scleroderma HAQ
  HRCT of the lungs: quantifiable scale*
Raynaud’s phenomenon
  Raynaud’s condition score
  VAS (part of S-HAQ)
Renal
  Calculated creatinine clearance based on serum creatinine (Cockroft-Gault or MDRD formula)
  Pre-defined renal crisis (presence or absence)
Skin
  Modified Rodnan Skin Score (range 0–51)
  VAS/Likert of patient global assessment for skin activity**
  VAS/Likert of physician global assessment for skin activity**
  Durometer
  • Standardized central reading mechanism strongly encouraged;

  • * if relevant to the study;

  • ** Items were based on Steering Committee and SCTC consensus despite lack of full validation. CRISS: Combined Response Index for SSc; ESR: erythrocyte sedimentation rate; HAQ-DI: Health Assessment Questionnaire Disability Index; VAS: visual analog scale; HRCT: high resolution computed tomography; MDRD: modification of diet in renal disease.