295major domains in SSc. They include skin involvement, calcinosis, diffi culties
sleeping, hand mobility, dyspnea, eating, bowel involvement, and pain. Some of
these are specifi c to SSc, compared to the HAQ. The patient assesses each question
in the SBI on a Likert scale ranging from 0 to 10, with 10 indicating worse severity,
frequency, or higher symptoms according to fi ve questions per each domain, for a
total of 40 questions [ 52 ]. Each scale is scored and an average burden score for each
of the eight domains is calculated. The data from the SBI also can be used to indi-
cate the proportion experiencing each SSc-related problem and the number of prob-
lems each patient experiences.
The SBI has good reliability between items, with moderate to high interitem and
item-total score correlations per domain and high internal consistency reliability
estimates. The SBI is correlated with the HAQ and the SF-36 for both physical and
mental health [ 52 ]. The SBI has construct validity, but has not been investigated
with regard to sensitivity to change [ 52 ]. Despite the existence of research demon-
strating its strengths, the SBI has not been widely used in SSc trials.
The UK Scleroderma Functional Score
Like the SBI and the SHAQ, the UK Scleroderma Functional Score ( UKFS ) is a
general measure made to examine elements of disease specifi c to SSc. The UKFS is
an 11-item, 4-grade questionnaire used for functional assessment [ 53 ]. Eleven items
for upper and lower extremity function and weakness are given a score of 0 (normal
ability to perform a task) to 3 (impossible to perform) to produce an overall score
between 0 and 33. In SSc, the UKFS is reliable and valid [ 6 , 53 , 54 ], with acceptable
test–retest reliability and can differentiate between lcSSc and dcSSc. The UKFS
correlates well with the HAQ-DI (r = 0.90) and can demonstrate change in a longi-
tudinal study [ 6 , 55 ]. Like the SBI, the UKFS is meant to be an SSc-specifi c mea-
sure. However, like the SBI, the UKFS is not widely used.
Symptom and Organ-Specifi c Measures
Gastrointestinal Disease
In SSc, gastrointestinal (GI) organ involvement occurs in approximately 90 % of
patients and is burdensome [ 56 , 57 ]. The entire GI tract can be affected, leading to
the possibility for a wide range of presentations and complications. The negative
HRQoL can be diffi cult to capture due to the unique complexity and overlap of GI
symptoms seen in SSc [ 58 , 59 ].
In 2007, Khanna et al. noted the lack of GI instruments pertinent to SSc
HRQoL. As a result, they developed the SSc Gastrointestinal Tract 1.0 (SSc-GIT
1.0) [ 60 ]. The SSc-GIT 1.0 was a comprehensive, reliable, and valid 52-item, self-
reported measure of SSc-related GI disease [ 60 ]. Although thorough, it was found
to take a lot of time to complete [ 61 ].
11 PROMs for Systemic Sclerosis (Scleroderma)