Background/Purpose: To investigate  whether patients affected with any of the 3 subsets of  early systemic sclerosis (SSc)  i.e. Raynaud’s Phenomenon (RP) with  SSc marker autoantibody (ACA or anti-Scl70 or anti-RNA polymerase III or anti-fibrillarin or anti-Th/To) and  typical capillaroscopic findings (megacapillaries and/or avascular areas) (subset I); or autoantibody positive only (subset II); or capillaroscopy positive only (subset III) (1) and unsatisfying the 2012 ACR/EULAR classification criteria for SSc (2) at admission  differ in the lag time to satisfy the new SSc classification  criteria.

Methods: Early SSc patients consecutively admitted to a Rheumatology  and an Angiology center and unsatisfying the 2012 ACR/EULAR classification criteria for SSc at admission, were subdivided into the 3 above referred subsets and followed-up for 7-101 months (median 45). They were re-evaluated six-monthly by history, clinical examination, B-mode echoDopplercardiography and Lung functional study including DLCO evaluation and yearly by lung HRCT to assess whether and when each of them satisfied new ACR/EULAR classification criteria i.e. developed a disease score ≥9 (2).               

Results: During the follow-up, 11 out of 21 subset I patients (52.3%) (baseline score  8); 10 out of 15 subset II patients (66.6%) (baseline score 6) and 0 out of 24 subset III patients (baseline score 5-7)  satisfied the criteria; the difference being significant  between each of the 2 autoantibody positive (subsets I and II) and the capillaroscopic positive-autoantibody negative subset  (subset I versus III: X²  by  log rank test=17.45, p=0.0001; subset II versus III: X² =11.04, p=0.0009), no difference being detected between the 2 autoantibody positive subsets (X² =0.55, p=0.454). The 11 subset I patients satisfied the criteria  because of the development of teleangectasias in 5 cases; puffy fingers in 3 cases; lung fibrosis in 2 cases; digital ulcers in 1 case. The 10 subset II patients did it because of the development of at least 2 of the following manifestations: scleroderma capillaroscopic pattern in 5 cases, teleangectasias in 5 cases, puffy fingers in 5 cases, digital ulcers in 3 cases, pulmonary hypertension in 1 case and lung fibrosis in 1 case. Despite the unfulfillment of the criteria, among the 24 subset III patients, 21 of whom already presented puffy fingers at baseline, 2 developed telangectasias, 1 digital ulcers, 4 a DLCO<80%.

Conclusion:

We have recently pointed out that autoantibody positive early SSc patients differ from subset III patients in the pattern of activation markers (increased serum concentration of procollagen I carbossipropeptide versus increased serum concentration of E-selectin) and preclinical internal organ involvement  (higher prevalence of  decreased DLCO) (3). Here we point out that autoantibody positive patients present a faster pace of the disease.                                                             

 

 

 

References

 

1) Koenig M et al. Arthritis Rheum. 2008;58:3902-12

2) Van den Hoogen F et al. Eular Congress 2013, OP0033

3) Valentini G et al. Arthritis Res Ther. 2013; 29;15:R63

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