Lack of small blood vessel damage patterns may predict milder SSc
Study: Follow-up shows fewer vascular complications and less skin thickening
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People with scleroderma (SSc) who don’t show the so-called scleroderma pattern, a common diagnostic finding of small blood vessel damage associated with the condition, may experience a milder course of the disease, an Italian study suggests.
About 5% of study participants didn’t show this characteristic pattern at the time of diagnosis. These individuals were less likely to exhibit severe symptoms and tended to have fewer complications over the follow-up period.
As such, lack of the scleroderma pattern “may identify a [patient] subset characterized by milder disease, with a lower burden of vascular and organ complications,” researchers wrote.
The study, “The clinical profile of systemic sclerosis without a ‘scleroderma pattern’ at nailfold capillaroscopy: results from the multicenter SPRING registry of the Italian Society of Rheumatology,” was published in the Journal of Translational Autoimmunity.
Test examines small blood vessels under the fingernails
SSc is a chronic disease that affects the skin and connective tissues throughout the body. Patches of thick or hardened skin and calcium deposits under the skin and in soft tissues (called calcinosis) are often among the symptoms of scleroderma.
During diagnosis, doctors may perform nailfold videocapillaroscopy (NVC), a test that examines small blood vessels under the fingernails. Many people with scleroderma, though not all, have a distinctive pattern of damage called the NVC scleroderma pattern (NVC-SP).
Previous studies have suggested that different subtypes of NVC-SP are associated with different degrees of SSc severity.
“However, it is less clear whether the risk of developing distinct complications differs when comparing patients with or without NVC-SP,” the team wrote.
To address this, they examined medical records for 1,689 participants in an Italian SSc registry with NVC test results. Of these, 5.3% did not show the alterations to tiny blood vessels, or capillaries, that define the NVC-SP.
“Our data show that in some SSc patients NVC capillary modifications may be not yet detectable, suggesting a very slow progression of the microvascular disease,” the researchers noted.
Patients without pattern older at disease onset
Demographically, the participants with and without NVC-SP were generally similar. However, on average, people without NVC-SP were significantly older at disease onset (median age at scleroderma onset of 53 vs. 49 in the NVC-SP group).
Disease features, including SSc subtype, also differed between the groups. People without NVC-SP were significantly more likely to have sine sclerosis, a form of scleroderma without the characteristic skin thickening seen in other types. They were also significantly less likely to have diffuse systemic scleroderma, a subtype with extensive skin symptoms.
The researchers examined up to five years of follow-up data for participants with and without NVC-SP.
“Compared to patients with the NVC-SP, those without NVC-SP exhibited a significantly lower burden of skin and vascular involvement throughout the follow-up,” the team wrote.
Specifically, they tested for changes in the modified Rodnan Skin Score (mRSS), which measures skin thickness in 17 body areas, and the Unified Vascular Phenotype (UVP) score, which measures changes in blood vessels by looking at signs such as digital ulcers. Both mRSS and UVP remained significantly lower in the non-NVC-SP group after one, two, and five years, indicating a lower disease burden. People without NVC-SP were also significantly less likely to develop calcinosis.
The absence of NVC-SP might have a prognostic implication, as these patients have a lower probability of developing new vascular complications, calcinosis, and worsening mRSS.
A subset of 268 participants met the very early diagnosis of systemic sclerosis, or VEDOSS, diagnostic criteria. VEDOSS criteria can identify people at high risk of developing SSc based on common early symptoms of the condition, such as puffy fingers.
In the VEDOSS group, about one-third didn’t have NVC-SP. These individuals tended to have lower UVP scores as well. However, the relatively small number of participants limits conclusions, according to the researchers.
Taken together, the study’s results suggest that people with SSc who don’t have NVC-SP may have distinctive, milder disease features.
“The absence of NVC-SP might have a prognostic implication, as these patients have a lower probability of developing new vascular complications, calcinosis, and worsening mRSS,” the team wrote.
Future studies could more explicitly test the prognostic value of NVC-SP. They could also assess if changes in NVC-SP over time are relevant for the disease course, the investigators said.
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