Background/Purpose: Anti-centromere (aCENP) antibodies are considered highly specific of systemic sclerosis (SSc), typically defining a limited cutaneous phenotype, usually associated to Raynaud´s phenomenon (RP). It is less understood the significance of these antibodies in the abscence of RP. Our aim in this study was to describe manifestations and outcomes in patients testing positive for aCENP over a five years period.

Methods: We carried out a review of charts of all patients with positive tests for aCENP antibodies detected at our Institution between 2015 and 2019. Data are shown in mean ± SEM, and frequencies. Two-sample t test and Chi2 were used for comparisons.

Results: The population comprised 298 cases, of whom 204 were available for follow-up. Eighty seven % were women. Mean age at study entry was 65,4 ± 0,9 y.o. First manifestation was RP in 84 patients (36% of the cohort). Time from aCENP detection to study entry was 1,6 ± 0,2 yrs., and 63 patients (21%) fulfilled SSc classification criteria at that moment.

At first evaluation, 192 patients (85%) did not have skin involvement, although 46 (24%) of them fulfilled SSc criteria. Only 19 patients (9%) had sclerodactily, out of whom 14 cases fulfilled SSc criteria. Ten patients had limited SSc with proximal to metacarpophalangeal joints involvement and 1 additional patient had diffuse SSc.

Interestingly, 97 patients (44%) did not report RP at study entry (as compared to 119 patients with this symptom).

The presence of RP was associated with a higher frequency of fulfillment of SSc criteria at study entry (59 patients, 50% of cases vs 4 patients, 4%, in the subgroup of cases without RP (p 0.001).

Out of 153 nailfold videocapillaroscopies (NVC) performed during workup only 15% (23 cases) were normal. An abnormal NVC pattern was found in 96 of the patients with RP (91%) as compared with 33 of the patients without RP (62%), (p < 0.001). No significant differences were found between RP and non-RP patients as regards to non-SSc specific abnormalities and early SSc patterns, while the presence of an active or a late pattern were strongly associated to the RP group (p 0.001, p 0.016 respectively).

After 3.6 ± 0.2 yrs of follow-up (732.2 patient-year) 90 patients fulfilled SSc classification criteria (corresponding to 22 new cases).

RP was associated to the development of SSc during follow-up (68% of the patients vs. 12% of those without RP, p < 0.001). A normal pattern in the NVC was associated to a lack of fulfillment of SSc criteria at the end of follow-up (NPV of 89,5% , p < 0.001).

Conclusion: Our results suggest that all patients with aCENP antibodies should undergo an NVC, even in the absence of RP, since many of them have an underlying vasculopathy.

« Back to ACR Convergence 2024

ACR Meeting Abstracts - https://acrabstracts.org/abstract/are-anti-centromere-antibodies-acenp-predictive-of-systemic-sclerosis-development-in-patients-without-raynauds-phenomenon/