RT Journal Article
SR Electronic
T1 Systemic sclerosis - continuing progress in developing clinical measures of response.
JF The Journal of Rheumatology
JO J Rheumatol
FD The Journal of Rheumatology
SP 1194
OP 1200
VO 34
IS 5
A1 Daniel Furst
A1 Dinesh Khanna
A1 Marco Matucci-Cerinic
A1 Philip Clements
A1 Virginia Steen
A1 Janet Pope
A1 Peter Merkel
A1 Ivan Foeldvari
A1 James Seibold
A1 David Pittrow
A1 Richard Polisson
A1 Vibeke Strand
YR 2007
UL http://www.jrheum.org/content/34/5/1194.abstract
AB Few randomized controlled trials (RCT) have shown a demonstrable treatment effect in systemic sclerosis (SSc), making it difficult to evaluate outcome measures in this disease indication. Results from recent RCT, including those evaluating cyclophosphamide for SSc interstitial lung disease and endothelin receptor antagonists for pulmonary hypertension, have allowed analysis of certain organ-specific endpoints using the OMERACT filter. An earlier metaanalysis established that skin score, measures of Raynaud's, pulmonary function tests, blood pressure, pain, Health Assessment Questionnaire, and Medical Outcomes Survey Short-Form 36 are validated outcome measures in SSc. At OMERACT 8, data regarding validation of high-resolution computed tomography of the lungs, 6-minute walk test, and patient reported outcomes in SSc were presented. A Delphi exercise to develop consensus regarding a combined set of noninvasive measures for pulmonary arterial hypertension (PAH) is under way. Given the protean nature of this illness and its multiorgan system involvement, a composite responder index may be preferable. Another Delphi exercise is designed to develop consensus regarding a combined SSc response index to be validated in future RCT.