TOPLINE

Long-term follow-up of patients with early Raynaud's phenomenon (RP) over more than 20 years confirms an association between abnormal nailfold capillaries, antinuclear antibodies (ANA), and increased mortality compared with those with neither marker.

METHODOLOGY

• A retrospective cohort study analyzed 2922 patients (median age, 46 years; 77.6% women) with incipient RP and no known connective tissue disease (CTD) who presented to a tertiary care vascular outpatient clinic in Vienna, Austria, between January 1994 and April 2008.
• Participants underwent nailfold capillaroscopy to assess microvascular abnormalities including capillary dilations, hemorrhages, reduced capillary density, tortuous capillaries, giant capillaries, ramifications, edema, and avascular fields. Serological testing for ANA (titer ≥ 1:160 considered positive) and ANA subsets (anti-Scl-70, anti-CENP-B, anti-SSA [Ro], anti-SSB [La], anti-U1-RNP, anti-SM, anti-Jo-1, and anti-ds-DNA antibodies) was also performed.
• Mortality data and causes of death were obtained from the standard population, and survival was compared with an age- and sex-matched reference population.

TAKEAWAY

• Over a median follow-up period of 23 years, 832 patients died; both females (P < .0001) and males (P = .01) with RP had significantly lower survival compared with the matched reference population.
• Mortality was highest among patients who displayed both abnormal nailfold capillaries and autoantibodies (log-rank test < .001).
• In females with RP, pathognomonic capillary aberrations were associated with increased mortality (hazard ratio [HR], 1.59; P < .001), as was ANA positivity (HR, 1.44; P < .01) and anti-Scl-70 antibodies (HR, 2.2; P < .001).
• In male patients, no significant associations were observed between abnormal nailfold capillaries or ANA positivity and mortality.

IN PRACTICE

"The coexistence of ANA positivity and abnormal nailfold capillaries may identify a subgroup with an underlying systemic biological vulnerability that adversely influences outcomes across a broad range of intercurrent illnesses. Whether this reflects generalized microvascular dysfunction, immune dysregulation, or another systemic process remains to be determined," the authors of the study wrote.

SOURCE

The study was led by Markus Müller, Division of Angiology, Department of Medicine II, Medical University of Vienna, Vienna, Austria. It was published online on August 4 in Rheumatology International.

LIMITATIONS

The study lacked structured follow-up to capture the development of autoimmune rheumatic disease and key time-varying variables such as prescribed treatments. Different laboratory methods for antibody detection were used over the 15-year inclusion period, potentially introducing heterogeneity in ANA-positivity definitions. ANA subsets were not tested in all participants.

DISCLOSURES

Open access funding was provided by the Medical University of Vienna. The authors reported no relevant conflicts of interest.

This article was created using several editorial tools, including AI, as part of the process. Human editors reviewed this content before publication.