Predictors of clinical features in early‐onset severe systemic sclerosis: An analysis from a multicenter prospective observational Japanese cohort

Author:

Uesugi‐Uchida Saori1,Fujimoto Manabu2,Asano Yoshihide3,Endo Hirahito4,Goto Daisuke5,Jinnin Masatoshi6,Kawaguchi Yasushi7,Tanaka Sumiaki8,Tokunaga Takahiro910,Makino Katsunari11,Matsushita Takashi12ORCID,Motegi Sei‐Ichiro13ORCID,Yoshizaki Ayumi1415,Sato Shinichi14,Hasegawa Minoru1ORCID

Affiliation:

1. Department of Dermatology University of Fukui Fukui Japan

2. Department of Dermatology, Graduate School of Medicine Osaka University Osaka Japan

3. Department of Dermatology Tohoku University Graduate School of Medicine Sendai Japan

4. Department of Rheumatology Southern Tohoku General Hospital Fukushima Japan

5. Department of Internal Medicine, Faculty of Medicine University of Tsukuba Ibaraki Japan

6. Department of Dermatology Wakayama Medical University Graduate School of Medicine Wakayama Japan

7. Institute of Rheumatology, Tokyo Women's Medical University Tokyo Japan

8. Kitasato University Hospital Sagamihara Japan

9. Medical Research Support Center University of Fukui Hospital Fukui Japan

10. Research Promotion Office Shinseikai Toyama Hospital Toyama Japan

11. Department of Dermatology and Plastic Surgery, Faculty of Life Sciences Kumamoto University Kumamoto Japan

12. Department of Dermatology, Faculty of Medicine, Institute of Medical, Pharmaceutical and Health Sciences Kanazawa University Kanazawa Japan

13. Department of Dermatology Gunma University Graduate School of Medicine Maebash Gunma Japan

14. Department of Dermatology University of Tokyo Graduate School of Medicine Tokyo Japan

15. Department of Clinical Cannabinoid Research University of Tokyo Graduate School of Medicine Tokyo Japan

Abstract

AbstractAs the clinical course of systemic sclerosis (SSc) varies widely, prognostic indicators have been sought to predict the outcomes of individual patients. Racial differences in SSc render it necessary to validate prognostic indicators in different patient cohorts. In this study, we aimed to assess clinical and laboratory parameters in Japanese patients with early‐stage SSc with diffuse cutaneous involvement and/or interstitial lung disease, and identify predictive factors for disease progression. We performed multivariate analyses of baseline clinical information to estimate symptoms 4 years later in Japanese patients with diffuse cutaneous SSc and/or SSc with interstitial lung disease. Patients were enrolled in the study within 5 years of disease onset at 10 Japanese SSc centers. Over 12 years, 115 patients followed up for 4 years were included in this study. The modified Rodnan skin score (mRSS) at 4 years correlated with the baseline mRSS and finger‐to‐palm distance, defined as the average length from the distal tip of the fourth finger to the distal palmar crease. The percentage predicted vital capacity (%VC) in year 4 positively and negatively correlated with initial %VC and the presence of anti‐topoisomerase I antibodies, respectively. The Health Assessment Questionnaire Disability Index (HAQ‐DI) at 4 years was positively and negatively associated with baseline HAQ‐DI and %VC, respectively. The occurrence of digital ulcers within 4 years was associated with the initial presence of digital ulcers, finger‐to‐palm distance, and the presence of digital pitting scars and anti‐topoisomerase I antibodies. This study identified several factors that may predict the progression of early‐stage SSc in Japanese patients. Finger‐to‐palm distance may be a useful tool for predicting the progression of skin thickening and the development of digital ulcers in the early stages of severe SSc, but larger, long‐term prospective studies are needed to confirm our findings.

Publisher

Wiley

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