Background Microangiopathy, which features may be detected by nailfold videocapillaroscopy (NVC), is an early aspect of systemic sclerosis (SSc). It is not a static event, but dynamic and may progress through different patterns of microvascular damage (named “early”, “active” and “late”) during follow-up (1,2). The time of transition is variable, and it was little investigated (2). Furthermore, only a few studies demonstrated correlations between microvascular damage extent and organ involvement over long time (2–4).
Objectives Longitudinal study to investigate the transition of nailfold microangiopathy through different NVC patterns of microangiopathy in SSc, looking for associations with organ involvement and progression over long time.
Methods Thirty-four SSc patients according to LeRoy criteria (mean age 57±11 years, mean disease duration from Raynaud's phenomenon onset 9±11 years) with the “early” pattern of nailfold microangiopathy at first capillaroscopic visit were enrolled and followed by both NVC and clinical assessment for a mean time of 12±3 years. Only SSc patients with fully complete documentation were included into the study. The proper pattern of microangiopathy was recorded by NVC at each visit as previously reported (1,2), as well as organ involvement and antinuclear antibody (ANA) profile.
Results At the end of the twelve-year follow-up, 12 (35%) of patients were still showing the “early” scleroderma-pattern, while the NVC pattern of microangiopathy was changed in 65% of the patients. The NVC pattern was found “active” in 10 patients (30%) and “late” in 12 patients (35%). The median time of progression from the “early” to the “active” pattern was 31 months, from “active” to “late” 38 months, and from “early” to “late” 66 months. Interestingly, in the subgroup of patients whose microangiopathy progressed from the “early” to the “late” NVC pattern through the “active” pattern, the median time of progression from the “early” to the “active” pattern was only 14 months, while in the subgroup of patients whose microangiopathy progressed only from the “early” to the “active” NVC pattern it was 42 months. The median time of progression from “early” to “late” pattern was shorter in SSc patients with either nucleolar IIF ANA pattern or Scl70 autoantibodies. Organ involvement was found progressively larger in SSc patients with “early”, “active” and “late” NVC pattern of microangiopathy, respectively.
Conclusions The results confirm the progression of the SSc microvascular damage, quantified through the transition of different NVC patterns, in 65% of SSc patients. Patients showing either a fast progression from the “early” to the “active” NVC pattern of microangiopathy or positive ANA with a nucleolar IIF pattern should be strictly monitored as at risk of rapid progression to the “late” NVC pattern of microangiopathy which is associated to a larger risk of organ involvement.
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Disclosure of Interest None declared