299
[ 84 ]; however, predictive validity has not been examined in SSc. Both the TDI and
BDI are able to detect change [ 89 , 90 ].
There is disagreement about which scales should be used in SSc trails regarding
dyspnea [ 28 ].
Pulmonary Arterial Hypertension
The Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) is the
fi rst pulmonary hypertension-specifi c instrument for assessing HRQoL using 3
scales with 65 items [ 91 ]. Though this instrument is specifi c to pulmonary arterial
hypertension ( PAH ) , it has not been validated specifi cally in SSc-associated
PAH. The CAMPHOR assesses 25 items for impairment, 15 for function, and 25 for
QOL, as well as the symptoms of energy, breathlessness, and mood through sub-
scales [ 91 ]. The CAMPHOR has very good internal consistency, convergent and
divergent validity, and good reproducibility when tested in idiopathic PAH [ 28 , 91 ].
This instrument is also responsive to change and has been correlated with change in
New York functional class [ 92 , 93 ]. The CAMPHOR Utility Index and subscales
are as responsive to change in class as the 6-min walking test (6MWT) [ 92 ]. Though
the CAMPHOR has been tested for idiopathic PAH, it may not refl ect all of th e
QOL aspects specifi c to SSc [ 28 ].
Raynaud’s Condition Score and Digital Ulcers
Raynaud’s phenomenon (RP) is a common and painful condition associated with
SSc. The Raynaud’s Condition Score (RCS) is a self-reported global assessment of
RP activity in SSc patients [ 30 ]. The RCS uses a 0– 10 ordinal scale to measure
frequency, duration, severity, and impact of RP attacks. This information is used to
calculate a compos ite score from daily measures over a defi ned period of time [ 30 ].
Research has indicated that the RCS is reliable [ 30 , 94 ], with construct, content,
criterion, and discriminant validity and is sensitive to change [ 30 , 95 ]. Though this
score is used in RP trials, its interpretation is sometimes uncertain and scoring may
be confused as a visual analog scale [ 28 ].
Digital ul cers are a potential outcome in scleroderma and are associated with
RP. The impact of digital ulcers can be measured from the SHAQ [ 32 ].
The Future of PROS in SSC: PROMIS and FACIT
With the emergence of better technologies, PROs have shifted toward more sophis-
ticated computerized techniques. In the past, measuring PROs has relied on organ—
or symptom-specifi c scales, which are not fl exible or adaptive to specifi c patients.
Some believe that continuing in this fashion is resource consumptive and ineffi cient
11 PROMs for Systemic Sclerosis (Scleroderma)