Phenotype of diffuse cutaneous systemic sclerosis patients with positive anticentromere antibodies: A systematic literature review and meta-analysis.

Objectives: Anticentromere antibodies (ACA) are typically found in limited cutaneous systemic sclerosis (lcSSc), whereas patients with anti-topoisomerase I antibodies (ATA) usually exhibit diffuse cutaneous involvement (dcSSc). We aimed to investigate the clinical phenotype and outcome of ACA-dcSSc.
Methods: A systematic literature review was conducted (January 1970 to April 2023) across MEDLINE, Scopus and OVID, to define whether SSc patients (population) within the ACA-dcSSc subset (exposure) had higher/lower risk for major organ involvement (interstitial lung disease-ILD, pulmonary hypertension-PH, primary myocardial involvement-PMI, scleroderma renal crisis-SRC) and mortality (outcomes) compared to ACA-lcSSc and ATA-dcSSc. Inclusion criteria were: 1) adult SSc patients with identifiable demographic and clinical features by subtype; 2) observational studies. The quality of the studies was evaluated by the Newcastle-Ottawa Scale. Random-effects meta-analysis was performed to compare odds ratios (OR) for major organ involvement, and the 5- and 10-year mortality of ACA-dcSSc with the other subsets.
Results: Out of 1570 hits, six articles were included, identifying 177 ACA-dcSSc patients. In ACA-dcSSc, ILD was more frequent than in ACA-lcSSc (OR 2.60; 95 %CI 1.39-4.87) but less frequent compared to ATA-dcSSc (OR 0.17; 95 %CI 0.10-0.29). ACA-dcSSc patients had a higher prevalence of PH vs. both conventional subsets; PMI and SRC were more frequent in ACA-dcSSc compared to ACA-lcSSc, and similar to ATA-dcSSc. While 5-year survival rates were comparable among the subsets, ACA-dcSSc patients exhibited a lower 10-year mortality than ATA-dcSSc (OR 0.42; 95 %CI 0.2-0.85).
Conclusion: Although uncommon, the ACA-dcSSc subset appears to have a distinct clinical phenotype, with a better prognosis than ATA-dcSSc.
Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.
(Copyright © 2024. Published by Elsevier Inc.)