Background/Purpose: Nailfold capillaroscopy is the validated technique to assess scleroderma microangiopathy, an early and dynamic event that may progress through different patterns of microvascular damage (“early”, “active” and “late”) during follow-up (1,2). The time of transition is variable among patients, and it was little investigated (2). Several studies demonstrated correlations between microvascular damage extent and organ involvement degree (2-4). The aim of this longitudinal study was to investigate the time of transition of nailfold microangiopathy throughout different patterns of microvascular damage in SSc, assessing correlations with organ involvement.

Methods: Thirty-four SSc patients according to LeRoy criteria (mean age 57±11 years) with the “early” pattern of nailfold microangiopathy at first capillaroscopic visit were enrolled and followed for a mean time of 12±3 years. Only SSc patients with fully complete documentation were included into the study. The pattern of microangiopathy was recorded and the microangiopathy evolution score (MES) calculated by nailfold videocapillaroscopy (NVC) at each visit, as previously reported (1,2); organ involvement and antinuclear antibody (ANA) profile were also assessed.

Results: After a mean twelve years follow-up, 12 (35%) 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. 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. A correlation was confirmed between microvascular damage extent and organ involvement degree, as MES progressively increased and organ involvement was progressively greater in SSc patients with “early”, “active” and “late” NVC pattern of microangiopathy, respectively. The median time of progression from “early” to “late” pattern was shorter in SSc patients with either nucleolar IIF ANA pattern or Scl70 autoantibodies.

Conclusion: This study confirms the progression of nailfold microangiopathy through different patterns of microvascular damage in almost 65% of SSc patients. Patients showing a fast progression from the “early” to the “active” NVC pattern of microangiopathy (one year), as well as positive ANA with either a nucleolar IIF pattern or Scl70 positivity should be strictly monitored since at risk of rapid progression to the “late” NVC pattern of microangiopathy which is linked to a larger risk of organ involvement. References.
1. Cutolo M, et al. Rheumatology 2004;43:719-26. 2. Sulli A, et al. Arthritis Rheum. 2012;64:821-5. 3. Ingegnoli F, et al. Microvasc Res 2013;89:122-8. 4. Smith V, et al. J Rheumatol. 2013;40:2023-8.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/transition-of-nailfold-microangiopathy-throughout-different-patterns-of-microvascular-damage-and-correlations-with-organ-involvement-in-systemic-sclerosis-a-twelve-year-follow-up/