Evolving symptoms of Raynaud's phenomenon in systemic sclerosis are associated with physician and patient-reported assessments of disease severity

John D Pauling, Elizabeth Reilly, Theresa Smith, Tracy M Frech

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Abstract

OBJECTIVES: Assessment of Raynaud's phenomenon (RP) in systemic sclerosis (SSc) is reliant on self-report. The Raynaud's Condition Score (RCS) diary assumes discrete episodic RP attacks, although not all SSc patients identify with this paradigm. We investigated the clinical associations of SSc-RP symptom characteristics and evolution of SSc-RP symptoms with disease progression.

METHODS: A cross-sectional study at UK and US sites captured digital colour changes of SSc-RP and patient's ability to identify with diagrammatic representations (and descriptive stems) of four distinct theoretical SSc-RP patterns (progressing severity through A-D) reflecting progressively severe SSc-RP experiences. SSc-RP self-management and symptom evolution were explored. Patient demographics, clinical phenotype, scleroderma Health Assessment Questionnaire (SHAQ), the 2-week RCS diary and patient and physician global assessments were collected.

RESULTS: We enrolled 107 SSc patients (questionnaires returned by 94). A higher number of self-reported digital colour changes of SSc-RP were associated with increased SSc-RP symptom severity but not SSc clinical phenotype. Patients could identify with distinct patterns of SSc-RP. These patterns were associated with disease duration, global disease severity and conceptually-linked physician and patient assessments of peripheral vascular severity (e.g. SHAQ RP subscale and RCS diary parameters), but not conceptually unrelated outcomes (e.g. SHAQ Breathing subscale). SSc-RP characteristics and symptom severity evolve during the disease course.

CONCLUSIONS: Patients identify with distinct patterns of SSc-RP that may relate to progression of the obliterative microangiopathy of SSc. Difficulty distinguishing discrete SSc-RP attacks from persistent digital ischaemia in advanced SSc could influence diary-based approaches to assessing SSc-RP with implications for clinical trials. This article is protected by copyright. All rights reserved.

Original languageEnglish
Pages (from-to)1119-1126
JournalArthritis Care & Research
Volume71
Issue number8
Early online date21 Aug 2018
DOIs
Publication statusPublished - 31 Aug 2019

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Raynaud Disease
Systemic Scleroderma
Physicians
Health
Color
Phenotype

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Evolving symptoms of Raynaud's phenomenon in systemic sclerosis are associated with physician and patient-reported assessments of disease severity. / Pauling, John D; Reilly, Elizabeth; Smith, Theresa; Frech, Tracy M.

In: Arthritis Care & Research, Vol. 71, No. 8, 31.08.2019, p. 1119-1126.

Research output: Contribution to journalArticle

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title = "Evolving symptoms of Raynaud's phenomenon in systemic sclerosis are associated with physician and patient-reported assessments of disease severity",
abstract = "OBJECTIVES: Assessment of Raynaud's phenomenon (RP) in systemic sclerosis (SSc) is reliant on self-report. The Raynaud's Condition Score (RCS) diary assumes discrete episodic RP attacks, although not all SSc patients identify with this paradigm. We investigated the clinical associations of SSc-RP symptom characteristics and evolution of SSc-RP symptoms with disease progression.METHODS: A cross-sectional study at UK and US sites captured digital colour changes of SSc-RP and patient's ability to identify with diagrammatic representations (and descriptive stems) of four distinct theoretical SSc-RP patterns (progressing severity through A-D) reflecting progressively severe SSc-RP experiences. SSc-RP self-management and symptom evolution were explored. Patient demographics, clinical phenotype, scleroderma Health Assessment Questionnaire (SHAQ), the 2-week RCS diary and patient and physician global assessments were collected.RESULTS: We enrolled 107 SSc patients (questionnaires returned by 94). A higher number of self-reported digital colour changes of SSc-RP were associated with increased SSc-RP symptom severity but not SSc clinical phenotype. Patients could identify with distinct patterns of SSc-RP. These patterns were associated with disease duration, global disease severity and conceptually-linked physician and patient assessments of peripheral vascular severity (e.g. SHAQ RP subscale and RCS diary parameters), but not conceptually unrelated outcomes (e.g. SHAQ Breathing subscale). SSc-RP characteristics and symptom severity evolve during the disease course.CONCLUSIONS: Patients identify with distinct patterns of SSc-RP that may relate to progression of the obliterative microangiopathy of SSc. Difficulty distinguishing discrete SSc-RP attacks from persistent digital ischaemia in advanced SSc could influence diary-based approaches to assessing SSc-RP with implications for clinical trials. This article is protected by copyright. All rights reserved.",
author = "Pauling, {John D} and Elizabeth Reilly and Theresa Smith and Frech, {Tracy M}",
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T1 - Evolving symptoms of Raynaud's phenomenon in systemic sclerosis are associated with physician and patient-reported assessments of disease severity

AU - Pauling, John D

AU - Reilly, Elizabeth

AU - Smith, Theresa

AU - Frech, Tracy M

N1 - This article is protected by copyright. All rights reserved.

PY - 2019/8/31

Y1 - 2019/8/31

N2 - OBJECTIVES: Assessment of Raynaud's phenomenon (RP) in systemic sclerosis (SSc) is reliant on self-report. The Raynaud's Condition Score (RCS) diary assumes discrete episodic RP attacks, although not all SSc patients identify with this paradigm. We investigated the clinical associations of SSc-RP symptom characteristics and evolution of SSc-RP symptoms with disease progression.METHODS: A cross-sectional study at UK and US sites captured digital colour changes of SSc-RP and patient's ability to identify with diagrammatic representations (and descriptive stems) of four distinct theoretical SSc-RP patterns (progressing severity through A-D) reflecting progressively severe SSc-RP experiences. SSc-RP self-management and symptom evolution were explored. Patient demographics, clinical phenotype, scleroderma Health Assessment Questionnaire (SHAQ), the 2-week RCS diary and patient and physician global assessments were collected.RESULTS: We enrolled 107 SSc patients (questionnaires returned by 94). A higher number of self-reported digital colour changes of SSc-RP were associated with increased SSc-RP symptom severity but not SSc clinical phenotype. Patients could identify with distinct patterns of SSc-RP. These patterns were associated with disease duration, global disease severity and conceptually-linked physician and patient assessments of peripheral vascular severity (e.g. SHAQ RP subscale and RCS diary parameters), but not conceptually unrelated outcomes (e.g. SHAQ Breathing subscale). SSc-RP characteristics and symptom severity evolve during the disease course.CONCLUSIONS: Patients identify with distinct patterns of SSc-RP that may relate to progression of the obliterative microangiopathy of SSc. Difficulty distinguishing discrete SSc-RP attacks from persistent digital ischaemia in advanced SSc could influence diary-based approaches to assessing SSc-RP with implications for clinical trials. This article is protected by copyright. All rights reserved.

AB - OBJECTIVES: Assessment of Raynaud's phenomenon (RP) in systemic sclerosis (SSc) is reliant on self-report. The Raynaud's Condition Score (RCS) diary assumes discrete episodic RP attacks, although not all SSc patients identify with this paradigm. We investigated the clinical associations of SSc-RP symptom characteristics and evolution of SSc-RP symptoms with disease progression.METHODS: A cross-sectional study at UK and US sites captured digital colour changes of SSc-RP and patient's ability to identify with diagrammatic representations (and descriptive stems) of four distinct theoretical SSc-RP patterns (progressing severity through A-D) reflecting progressively severe SSc-RP experiences. SSc-RP self-management and symptom evolution were explored. Patient demographics, clinical phenotype, scleroderma Health Assessment Questionnaire (SHAQ), the 2-week RCS diary and patient and physician global assessments were collected.RESULTS: We enrolled 107 SSc patients (questionnaires returned by 94). A higher number of self-reported digital colour changes of SSc-RP were associated with increased SSc-RP symptom severity but not SSc clinical phenotype. Patients could identify with distinct patterns of SSc-RP. These patterns were associated with disease duration, global disease severity and conceptually-linked physician and patient assessments of peripheral vascular severity (e.g. SHAQ RP subscale and RCS diary parameters), but not conceptually unrelated outcomes (e.g. SHAQ Breathing subscale). SSc-RP characteristics and symptom severity evolve during the disease course.CONCLUSIONS: Patients identify with distinct patterns of SSc-RP that may relate to progression of the obliterative microangiopathy of SSc. Difficulty distinguishing discrete SSc-RP attacks from persistent digital ischaemia in advanced SSc could influence diary-based approaches to assessing SSc-RP with implications for clinical trials. This article is protected by copyright. All rights reserved.

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DO - 10.1002/acr.23729

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EP - 1126

JO - Arthritis Care and Research

JF - Arthritis Care and Research

SN - 2151-464X

IS - 8

ER -