Classification of Japanese patients with mild/early systemic sclerosis (SSc) by the 2013 ACR/EULAR classification criteria for SSc

Abstract

Objective: To classify Japanese patients with mild/early systemic sclerosis (SSc) by the 2013 American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) Classification Criteria (new 2013 criteria).

Methods: We assessed 120 patients who visited Kanazawa University Hospital suspected of SSc and who did not meet the 1980 ACR preliminary classification criteria for SSc. We clinically diagnosed 16 patients with primary Raynaud’s disease and 104 with mild/early SSc prior to being assessed by the new 2013 criteria.

Results: None of the 16 patients with primary Raynaud’s disease met the new 2013 criteria. On the other hand, 94 out of the 104 patients (90.3%) with mild/early SSc by our clinical diagnosis met the new 2013 criteria. Among the 94 SSc patients, sclerodactyly was detected in 58 (62%), puffy fingers in 62 (66%), abnormal nailfold capillaries in 89 (95%), Raynaud’s phenomenon in 93 (99%), and SSc-related autoantibodies (Abs) in 85 (90%). The median (range) score of these 94 patients was 12 (9–14). Ten mild/early SSc patients who did not meet the new 2013 criteria had the following clinical features: puffy fingers in 1 (10%), abnormal nailfold capillaries in 8 (80%), Raynaud’s phenomenon in 9 (90%), and SSc-related autoAbs in 8 (80%). The median (range) score of these 10 patients was 7 (5–8).

Conclusion: The new 2013 criteria can classify most mild/early Japanese SSc patients, which may contribute to early treatment interventions.

Keywords:

• New classification criteria
• Raynaud’s phenomenon
• Systemic sclerosis

Conflict of interest

The authors report no conflicts of interest. The authors alone are responsible for the content and writing of this article.

This work was supported by a grant for Research on Intractable Diseases from the Ministry of Health, Labor and Welfare of Japan (to Kazuhiko Takehara).