The relationship between skin symptoms and the scleroderma modification of the health assessment questionnaire, the modified Rodnan skin score, and skin pathology in patients with systemic sclerosis
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OBJECTIVE: To determine how well skin symptoms considered specific to SSc are captured by patient reported outcomes currently used for assessing patients with SSc, the SHAQ, or skin disease, the Skindex-29; and how well these symptoms correlate with the extent of skin disease on physical exam and skin pathology. METHODS: SSc patients completed the scleroderma modification of the Health Assessment Questionnaire (SHAQ), Skindex-29 and a Skin Symptom Assessment questionnaire developed for this study. Correlations were assessed between the Skin Symptom Assessment and SHAQ, Skindex-29, modified Rodnan skin score, and skin pathological features including myofibroblast staining completed on the same date. RESULTS: Tight, hard and rigid/stiff skin symptoms correlated moderately highly with the modified Rodnan skin score (r = 0.445, P = 0.0008; r = 0.486, P = 0.0002; and r = 0.488, P = 0.0002, respectively). Tight skin symptoms correlated moderately with myofibroblast infiltration (r = 0.544, P = 0.0023) and hyalinized collagen (r = 0.442, P = 0.0164), while both hard and rigid/stiff skin correlated moderately with inflammation (r = 0.401, P = 0.0310 and r = 0.513, P = 0.0045), myofibroblast infiltration(r = 0.480, P = 0.0084 and r = 0.527, P = 0.0033) and hyalinized collagen (r = 0.453, P = 0.0137 and r = 0.478, P = 0.0087), while the SHAQ was not found to correlate with any of these pathological changes. In contrast, painful skin symptoms correlated moderately with the SHAQ (r = 0.413, P = 0.0073), and with the three domains of Skindex-29: Symptoms, Emotions and Functioning. Skindex-29 indicates that dcSSc patient skin symptoms are nearly as severe as those of patients with psoriasis or atopic dermatitis. CONCLUSION: Patient reported skin symptoms correlate with clinical and pathological measures in the skin. A validated patient reported skin symptom instrument might considerably improve evaluation of SSc skin disease.
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In skin, the presence of myofibroblasts correlates with the amount of hyalinized collagen and skin fibrosis parameters such as tightness, hardness and stiffness, more significantly than inflammation.
“In skin, the presence of myofibroblasts correlates with the amount of (hyalinized) collagen and skin parameters related to fibrosis such as tightness, hardness and stiffness, and does so more significantly than inflammation (21–23), supporting for a role of myofibroblasts in the pathogenesis of these clinical signs.”
From Unraveling SSc Pathophysiology; The Myofibroblast (2018) · cites this paper as [22]
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Unraveling SSc Pathophysiology; The Myofibroblast
van Caam, A., Vonk, M., van den Hoogen, F., van Lent, P., & van der Kraan, P. (2018) · Frontiers in Immunology · doi:10.3389/fimmu.2018.02452 · Cited by 153 · see citing works
- Authors
- Ziemek, J., Man, A., Hinchcliff, M., Varga, J., Simms, R. W., & Lafyatis, R.
- Affiliations
- Boston University (US)
- Northwestern University (US)
- Venue
- Rheumatology
- Publisher
- Oxford University Press (OUP)
- Published
- 2016-02-15
- Volume
- 55
- Issue
- 5
- Pages
- 911-917
- ISSN
- 1462-0324 (ISSN-L)
- 1462-0332
- PMC
- PMC5854039
- OpenAlex
- W2278207122
- Cited by
- 33
- References
- 23
- Citations by year
- 2026: 1 · 2025: 2 · 2024: 3 · 2023: 4 · 2022: 4 · 2021: 3 · 2020: 5 · 2019: 4 · 2018: 5 · 2017: 2
- FWCI
- 3.33
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Lara D. Veeken (publishedVersion)
- PubMed Central (publishedVersion)
- PubMed (publishedVersion)
- HighWire Press Open Archive (submittedVersion)
- Funders
- Scleroderma Research Foundation
- Bristol-Myers Squibb
- Pfizer
- Sanofi
- Biogen
- Celgene
- Regeneron Pharmaceuticals
- National Institutes of Health (UL1TR000157)
- Genentech
- EMD Serono
- National Institute of Arthritis and Musculoskeletal and Skin Diseases (K23AR059763, R01AR051089, P30AR061271, P50AR060780)
- National Center for Advancing Translational Sciences
- Topics
- Health Sciences › Medicine › Pathology and Forensic Medicine › Systemic Sclerosis and Related Diseases
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Connective tissue disorders research
- Health Sciences › Medicine › Rheumatology › Dupuytren's Contracture and Treatments
- Keywords
- systemic sclerosis, cutaneous manifestations, Skindex-29, modified Rodnan skin score, dermatopathology, skin inflammation, patient-reported outcomes, diffuse cutaneous systemic sclerosis, psoriasis
- MeSH terms
- Adult, Aged, Biopsy, Cell Movement, Female, Humans, Male, Middle Aged, Quality of Life, Surveys and Questionnaires, Scleroderma, Localized, Scleroderma, Systemic, Severity of Illness Index, Skin, Scleroderma, Diffuse, Myofibroblasts
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