Parameters That Predict Worsening of Skin Thickness in Patients with Early Diffuse Cutaneous Systemic Sclerosis

Masataka Kuwana¹, Minoru Hasegawa², Yucihiro Shirai¹, Osamu Ishikawa³, Hirahito Endo⁴, Fumihide Ogawa⁵, Daisuke Goto⁶, Shinichi Sato⁷, Hironobu Ihn⁸, Yasushi Kawaguchi⁹ and Kazuhiko Takehara¹⁰, ¹Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan, ²Dermatology, Faculty of Medical Sciences, University of Fukui, Fukui, Japan, ³Department of Dermatology, Gunma University Graduate School of Medicine, Gunma, Japan, ⁴Department of Rheumatology, Jusendo General Hospital, Koriyama, Japan, ⁵Department of Dermatology, Nagasaki University Graduate School of Biomedical Science, Nagasaki, Japan, ⁶Division of Clinical Immunology, Doctoral Program in Clinical Sciences, University of Tsukuba, Graduate School of Comprehensive Human Sciences, Tsukuba, Japan, ⁷Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan, ⁸Department of Dermatology and Plastic Surgery, Faculty of Life Sciences, Kumamoto University, Kumamoto, Japan, ⁹Institute of Rheumatology, Tokyo Women's Medical University, Tokyo, Japan, ¹⁰Dermatology, Kanazawa University Graduate School of Medical Science, Kanazawa City, Japan

Meeting: 2015 ACR/ARHP Annual Meeting

Date of first publication: September 29, 2015

Keywords: Outcome measures, scleroderma and skin fibrosis

Session Information

Date: Monday, November 9, 2015

Title: Systemic Sclerosis, Fibrosing Syndromes and Raynaud's - Clinical Aspects and Therapeutics Poster II

Session Type: ACR Poster Session B

Session Time: 9:00AM-11:00AM

Background/Purpose: Skin thickness is a hallmark of systemic sclerosis (SSc), and its progression is associated with poor prognosis. The modified Rodnan total skin thickness score (MRSS) is widely used for evaluating extent and severity of skin thickness. Serial changes of MRSS during the disease course are highly heterogeneous among patients with diffuse cutaneous SSc (dcSSc): some show worsening of the score, but others experience regression. This study was aimed to identify parameters that predict worsening of MRSS in patients with early dcSSc using a multicenter prospective cohort conducted by the Scleroderma Study Conference of Japan.

Methods: A total of 171 patients with early dcSSc were selected from the prospective cohort database based on fulfillment of 1980 American College of Rheumatology preliminary criteria, dcSSc, MRSS ≥7 at entry, disease duration <60 months at entry, and valid data for MRSS at one year. Worsening of skin thickness was defined as increase in MRSS ≥3 points and ≥25% from baseline to one year. In univariate analysis, patients who experienced progression of skin thickness were compared with non-progressors, and selected predictive markers were included in multivariate logistic regression analysis.

Results: Only 23 patients (13.5%) experienced worsening of MRSS at one year. In progressors, MRSS increased at one year from 14.4 ± 6.5 to 21.4 ± 7.2 (P < 0.0001), and still maintained a high score over 5 years (20.6 ± 9.0 and 16.8 ± 8.7 at 3 and 5 years, respectively). In contrast, non-progressors showed continuous decrease of MRSS in the following 5 years (21.5 ± 8.9 at baseline, 13.0 ± 7.0 at one year, 11.2 ± 7.6 at 3 years, and 9.0 ± 6.4 at 5 years). Univariate analyses identified short disease duration (P = 0.002), negative anti-RNA polymerase III (P = 0.03), low baseline MRSS (P = 0.0003), tendon friction rubs (P = 0.02), absence of nailfold bleeding (P = 0.001), joint synovitis (P = 0.01), high KL-6 (P = 0.005), and high erythrocyte sedimentation rate (ESR) (P= 0.0001) as predictors for worsening of MRSS at one year. There was no difference in use of immunosuppressant or corticosteroids between progressors and non-progressors. In the multivariate analysis, disease duration (odds ratio 0.93 [0.87-0.99]), baseline MRSS (0.85 [0.77-0.95]), nailfold bleeding (0.25 [0.07-0.85]), and ESR (1.04 [1.01-1.08]) were selected as independent parameters associated with subsequent progression of the disease. Assessment of the best predictive model revealed that patients with disease duration ≤20 months, baseline MRSS ≤20, and ESR ≥21 mm/hour had the high risk of worsening of MRSS within one year, at sensitivity of 57% and specificity of 91% (odds ratio 13.5 [5.0-36.8]).

Conclusion: This study successfully identified dcSSc patients at the high risk of subsequent worsening of skin thickness. This information is useful in selecting patients who require intensive treatment with potential disease-modifying agents and in improving clinical trial design by enrichment for eligible progressors.

Disclosure: M. Kuwana, None; M. Hasegawa, None; Y. Shirai, None; O. Ishikawa, None; H. Endo, None; F. Ogawa, None; D. Goto, None; S. Sato, None; H. Ihn, None; Y. Kawaguchi, None; K. Takehara, Actelion Pharmaceuticals Japan, 5.

To cite this abstract in AMA style:

Kuwana M, Hasegawa M, Shirai Y, Ishikawa O, Endo H, Ogawa F, Goto D, Sato S, Ihn H, Kawaguchi Y, Takehara K. Parameters That Predict Worsening of Skin Thickness in Patients with Early Diffuse Cutaneous Systemic Sclerosis [abstract]. Arthritis Rheumatol. 2015; 67 (suppl 10). https://acrabstracts.org/abstract/parameters-that-predict-worsening-of-skin-thickness-in-patients-with-early-diffuse-cutaneous-systemic-sclerosis/. Accessed .

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