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Evolving Symptom Characteristics of Raynaud's Phenomenon in Systemic Sclerosis and Their Association With Physician and Patient‐Reported Assessments of Disease Severity
Author(s) -
Pauling John D.,
Reilly Elizabeth,
Smith Theresa,
Frech Tracy M.
Publication year - 2019
Publication title -
arthritis care and research
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.032
H-Index - 163
eISSN - 2151-4658
pISSN - 2151-464X
DOI - 10.1002/acr.23729
Subject(s) - medicine , scleroderma (fungus) , disease , severity of illness , systemic scleroderma , demographics , clinical phenotype , microangiopathy , physical therapy , pathology , diabetes mellitus , phenotype , biochemistry , chemistry , demography , sociology , inoculation , gene , endocrinology
Objective Assessment of Raynaud's phenomenon ( RP ) in systemic sclerosis ( SS c) is reliant on self‐report. The Raynaud's Condition Score ( RCS ) diary assumes discrete episodic RP attacks, although not all SS c patients identify with this paradigm. We investigated the clinical associations of SS c‐ RP symptom characteristics and the evolution of SS c‐ RP symptoms with disease progression. Methods A cross‐sectional study at UK and US sites captured digital color changes of SS c‐ RP and patients’ ability to identify with diagrammatic representations (and descriptive stems) of 4 distinct theoretical SS c‐ RP patterns (progressing severity A through D) reflecting progressively severe SS c‐ RP experiences. SS c‐ RP self‐management and symptom evolution were explored. Patient demographics, the clinical phenotype, the Scleroderma Health Assessment Questionnaire ( SHAQ ), the 2‐week RCS diary, and patient and physician global assessments were collected. Results We enrolled 107 patients with SS c (with questionnaires returned by 94). A higher number of self‐reported digital color changes of SS c‐ RP were associated with increased SS c‐ RP symptom severity but not with the SS c clinical phenotype. Patients could identify with distinct patterns of SS c‐ 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 with conceptually unrelated outcomes (e.g., SHAQ breathing subscale). SS c‐ RP characteristics and symptom severity evolve during the disease course. Conclusion Patients identify with distinct patterns of SS c‐ RP that may relate to progression of the obliterative microangiopathy of SS c. Difficulty distinguishing discrete SS c‐ RP attacks from persistent digital ischemia in patients with advanced SS c could influence diary‐based approaches to assessing SS c‐ RP , with implications for future clinical trials.