Disease Heterogeneity Observed in Patients With Very Early Systemic Sclerosis

Raynaud’s Syndrome Phenomena Adult Hand
Researchers analyzed the extent of heterogeneity in clinical, epidemiologic, and immunologic characteristics of patients with very early systemic sclerosis.

Patients with very early systemic sclerosis (SSc) are a heterogeneous mixture of patients with mild and early disease, according to study results published in The Journal of Rheumatology.

Currently available classification criteria cannot differentiate between patients with very early, potentially progressive SSc and those who have very mild, nonprogressive SSc. Using clinical, epidemiologic, and immunologic data from patients enrolled in the Zurich cohort, investigators aimed to explore the heterogeneity of patients with very early SSc.

A total of 102 patients (90.2% women) were included in the study who did not meet either the 1980 American College of Rheumatology or the 2013 American College of Rheumatology/European League Against Rheumatism classification criteria for SSc but had a clinical diagnosis of SSc based on Raynaud phenomenon and additional clinical features of SSc, including puffy fingers, SSc-specific antibodies, SSc pattern on nailfold-capillaroscopy, or any organ involvement.

Antinuclear antibodies (96.0%) and nailfold capillaroscopy changes (77.2%) were highly prevalent in the cohort. Higher levels of heterogeneity were observed in other clinical parameters, including puffy fingers (19.0%), telangiectasia (15.3%), gastrointestinal esophageal involvement (28.3%), joint synovitis (12.7%), and interstitial lung disease (5.2%).

Median disease duration was 4.1 years (interquartile range, 1.6-11.5 years). Approximately 54% of patients had a disease duration <5 years, and 29.2% of patients had a disease duration >10 years. Patients with an extended disease duration were classified as having a mild, nonprogressive form of SSc.

No significant differences in disease manifestations were observed between patients with <5 years and ≥5 years of disease duration. Puffy fingers (22.9% vs 14.0%), joint synovitis (15.4% vs 9.1%), telangiectasia (18.6% vs 13.5%), and diffusing capacity for carbon monoxide <70% (10.6% vs 5.0%) were more common in patients with a shorter disease duration (<5 years) than those with a longer disease duration. No association was found between clinical parameters and disease duration.

Investigators noted that small sample size represents a limitation of the study.

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“[P]atients fulfilling the criteria for very early SSc are a heterogeneous mixture of patients with early disease potentially at risk [for] progression and patients with long standing, very mild SSc,” the researchers concluded. “The current classification of these patients should be reconsidered and needs to be divided into very mild/long-standing and very early potentially progressive patients.”

Disclosure: Several study authors declared affiliations with the pharmaceutical industry. Please see the original reference for a full list of disclosures.


Blaja E, Jordan S, Mihai CM, et al. The challenge of very early systemic sclerosis: a combination of mild and early disease? [published online March 15, 2020]. J Rheumatol. doi:10.3899/jrheum.190976