Background/Purpose:

The diagnosis of systemic sclerosis (SSc) in the early stages of the disease is frequently a challenge. In 2001, LeRoy and Medsger proposed criteria for the classification of early SSc, defined by the presence of Raynaud”s phenomenon (RP) associated with scleroderma specific antibodies and/or typical scleroderma changes on nailfold capillaroscopy (NFC). After the development of the 2013 ACR/EULAR classification criteria for SSc, which is more sensitive than previous, there are still a group of patients affected by early SSc who do not meet the 2013 ACR/EULAR criteria. Factors associated with disease evolution in these patients are not well recognized. This study aimed to evaluate the disease evolution and to identify predictors of evolution to definite SSc (ACR/EULAR classification criteria for SSc) in patients with early SSc.

Methods:

In this prospective study 46 consecutive patients with early SSc (2001 LeRoy and Medsger criteria), were evaluated at baseline and at 3 years of follow-up. Clinical evaluation and widefield nailfold capillaroscopy using a stereomicroscope (10–40x magnification) were performed in all subjects at baseline and at the end of the study. At the end of follow-up, the fulfillment of the 2013 ACR/EULAR criteria was also assessed in each patient. The following parameters were determined by NFC: number of enlarged and giant capillaries, the number of microhaemorrhages, and the avascular score. The scleroderma pattern was classified as early, active and late.

Results:

After 3 years, 34 patients with early SSc were reevaluated (mean age 53 ± 12.7 years) and 12 patients were lost to follow-up. Eight (23.5%) patients with early SSc developed definite SSc. A higher frequency of puffy fingers at baseline was observed in the group of patients who developed definite SSc compared to those who did not (37.5% vs 0%, respectively; p=0.01). A higher proportion of patients with early SSc who progressed to definite SSc presented a significant increase in the number of giant capillaries (50% vs 7.7%, respectively; p=0.02) and a increase in the avascular score compared with those who remained with a diagnosis of early SSc (50% vs 0%, respectively; p=0.01). A higher frequency of active pattern in NFC at baseline was also observed in patients who progressed to definite SSc compared with those who did not (57.1% vs 6.3%, respectively; p=0.02). By multivariate analysis, increase in the number of giant capillaries (OR=12.0, 95% CI 1.6-88.7, p=0.02), and an active pattern in NFC (OR=30.0, 95% CI 2.1-421.1, p=0.01) were independent risk factors for developing definite SSc in patients with early SSc.

Conclusion:

In this prospective study, patients with early SSc who developed definite disease showed a worsening in capillaroscopic parameters including an increase in the number of giant capillaries and in the avascular score during a follow-up of 3 years. The presence of an active pattern and an increase in the number of giant capillaries in NFC were independent predictive risk factors for developing definite SSc in patients with early SSc.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/risk-of-development-of-definite-disease-in-patients-with-early-systemic-sclerosis/