ACR Meeting Abstracts

ACR Meeting Abstracts

Abstract Number: 720

Measures of Disease Status in Systemic Sclerosis: Systematic Review

Tien Tay1, Nava Ferdowsi1, Wendy Stevens1, Marie Hudson2, Murray Baron3, Candice Rabusa1, David Prior4, Susanna Proudman5 and Mandana Nikpour6, 1Rheumatology, The University of Melbourne at St Vincent’s Hospital, Melbourne, Australia, 2Rheumatology, Lady David Institute for Medical Research and Jewish General Hospital, Montreal, QC, Canada, 3Pavillion A, Rm 216, Lady David Institute for Medical Research and Jewish General Hospital, Montreal, QC, Canada, 4Cardiology, The University of Melbourne at St Vincent’s Hospital, Melbourne, Australia, 5Royal Adelaide Hospital, Rheumatology Unit and University of Adelaide, Discipline of Medicine, Adelaide, Australia, 6Medicine, The University of Melbourne at St Vincent’s Hospital, Melbourne, Australia

Meeting: 2014 ACR/ARHP Annual Meeting

Keywords: ,

Session Information

Title: Systemic Sclerosis, Fibrosing Syndromes and Raynaud's - Clinical Aspects and Therapeutics: Systemic Sclerosis Measures and Outcomes

Session Type: Abstract Submissions (ACR)

Background/Purpose : To identify the measures of disease status in systemic sclerosis (SSc) using a systematic review. Methods: A systematic review of Medline (1966-2014), EMBASE (1974-2014), and Cochrane Library (inception-2014) was undertaken to identify indices of disease status in systemic sclerosis. We focussed on objective measures and excluded non-English articles, animal-only studies and those relating to morphea, localized scleroderma or juvenile systemic sclerosis. Results: Of the 5687 articles identified through the search, we identified 45 articles for review. We found a further 22 articles through a search of the bibliography of relevant articles. We identified 10 ‘composite’ (multi-organ) indices: two disease activity indices, six disease severity scales, and two combined response indices (Tables 1 and 2). There was no disease damage index for SSc. Furthermore, we found no objective organ-specific index for the gastrointestinal system. Conclusion: We identified a number of composite and organ-specific indices in SSc, incorporating subjective and/or objective measures, developed to quantify disease activity, severity and response in clinical trials. Most of the existing indices require further evaluation according to the OMERACT filter. None of the indices measures organ damage per se, highlighting this as an area for future research.

 

Table 1: Features of existing composite measures of disease status in systemic sclerosis

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

Features

Disease activity indices

Disease severity indices

 

 

 

 

 

 

 

Outcome measures

 

 

Valentini

Minier

Medsger

Geirrson

Morita

Furst

Hughes

Casas

CRISS^

EPOSS~

Methodology

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

Consensus-based

Y

 

?

 

Y

 

?

 

?

 

?

 

?

 

?

 

Y

 

Y

 

 

Number of experts

3

 

?

 

20

 

?

 

?

 

?

 

?

 

?

 

9

 

12

 

 

Data-driven

Y

 

Y

 

Y

 

?

 

?

 

?

 

?

 

?

 

N

 

Y

 

Variables

10

 

17

 

31

 

5

 

19

 

33

 

7

 

9

 

30

 

14

 

Organ systems

5

 

5

 

9

 

5

 

9

 

7

 

5

 

6

 

11

 

6

 

 

 

Obj

Sub

Obj

Sub

Obj

Sub

Obj

Sub

Obj

Sub

Obj

Sub

Obj

Sub

Obj

Sub

Obj

Sub

Obj

Sub

 

Musculoskeletal

Y

Y

Y

Y

Y

N

N*

N

Y

N

Y#

Y

N

N

N

N

Y

Y

N

N

 

Vascular

Y

Y

Y

Y

Y

N

N*

N

Y

N

Y

N

N

N

Y

Y

Y

Y

N

N

 

Skin

Y

Y

Y

Y

Y

N

Y

N

Y

N

Y

N

Y

N

N

N

N

N

N

N

 

Cardiac

N

Y

N

Y

Y

N

N*

N

Y

Y

Y

Y

Y

N

Y

Y

Y

Y

Y

Y

 

Respiratory

N

Y

N

Y

Y

N

Y

N

Y

N

Y

Y

Y

N

Y

Y

Y

Y

Y

Y

 

Gastrointestinal

N

N

N

N

Y

N

Y

N

Y

N

Y

Y

Y

N

Y

Y

N

Y

N

N

 

Renal

N

N

N

N

Y

N

Y

N

Y

N

Y

N

Y

N

Y

N

Y

N

N

N

 

General lab tests

Y

 

Y

 

Y

 

Y

 

N

 

N

 

N

 

N

 

Y

 

N

 

 

Others

N

N

N

Y

N

N

N*

N

Y

N

N

N

?

?

N

N

Y

Y

Y

Y

Patient-reported outcomes

Y

 

Y

 

N

 

N

 

N

 

Y

 

N

 

N

 

Y

 

Y

 

Weighted score

Y

 

Y

 

N

 

N

 

N

 

Y

 

N

 

N

 

N

 

N

 

Global assessment score

Y

 

Y

 

N

 

N

 

N

 

Y

 

N

 

N

 

N

 

N

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

? Insufficient data to evaluate the item; Obj: objective tests; Sub: subjective tests; * omitted due to lack of recorded data;

 

 

# includes SJC, TJC, bony erosions, lysis, serum creatine phosphokinase, myositis (EMG) and myopathy (EMG)

^ The Combined Response Index for Systemic Sclerosis; ~ Expert Panel on Outcome Measures in Pulmonary Arterial Hypertension related to Systemic Sclerosis

 

 

 

 


Table 2: Summary of composite measures of health status in systemic sclerosis according to the OMERACT filter

 

 

 

 

 

 

 

OMERACT filter

Disease activity indices

Disease severity indices

 

 

 

Outcome measures

 

 

Valentini

Minier

Medsger

Geirrson

Morita

Furst

Hughes

Casas

CRISS~

EPOSS`

Validity

 

 

 

 

 

 

 

 

 

 

 

Face validity

Y

Y

Y

Y

Y

Y

Y

Y

Y

Y

 

Content validity

PV*^

PV*^

Y

N

Y

Y

N

N

Y

Y

 

Criterion validity#

PV

NT

Y

Y

?

?

N

N

NT

NT

 

Construct validity$

Y

NT

NT

N

?

?

N

N

NT

NT

Discrimination capacity

 

 

 

 

 

 

 

 

 

 

 

Reliability

Y

NT

NT

NT

?

?

NT

NT

NT

NT

 

Sensitivity to change

PV*

NT

NT

?

?

NT

NT

NT

NT

NT

Feasibility

Y

NT

Y

Y

Y

N

Y

Y

Y

Y

Validation

 

 

 

 

 

 

 

 

 

 

 

Internal validation

Y

NT

Y

NT

?

?

NT

NT

IPR

IPR

 

External validation

Y

NT

Y

NT

?

?

NT

NT

IPR

IPR

 

 

 

 

 

 

 

 

 

 

 

 

*PV: partially validated; ^lacks gastrointestinal and renal components; #tested against mortality; NT: not tested;

? insufficient data to evaluate item; $ tested against physician global assessment of disease activity or disease severity;

~ Combined Response Index in Systemic Sclerosis; ` Expert Panel on Outcome Measures in Pulmonary Arterial

Hypertension related to Systemic Sclerosis: IPR: in progress

 

 

 

 

 

 


 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

Disclosure:

T. Tay,
None;

N. Ferdowsi,
None;

W. Stevens,
None;

M. Hudson,
None;

M. Baron,
None;

C. Rabusa,
None;

D. Prior,
None;

S. Proudman,
None;

M. Nikpour,
None.

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