Background Three different and progressive patterns of nailfold microvascular damage are evident in systemic sclerosis (SSc) (1). The dynamic transition from “Early” to “Active” or to “Late” pattern was described in about half of SSc patients during a 7 year follow-up, but the precise factors or events influencing a slower or faster progression of the SSc microangiopathy are still not yet identified (2).

Objectives To detect serum autoantibodies in SSc patients showing the progression of nailfold microangiopathy, to look at possible correlations between autoantibody patterns and microangiopathy stages.

Methods Forty-two SSc patients (median age 47 years; median disease duration 1 year) with the “Early” pattern of nailfold microangiopathy at baseline were prospectively followed-up by nailfold videocapillaroscopy (NVC) for an average time of 91 months. SSc patients were classified according to their pattern of microangiopathy, as previously described (1,3). Anti-nuclear (ANA), anti-topoisomerase I (Scl70), and anti-centromere (ACA) antibodies were evaluated in all patients.

Results At the end of follow-up, the NVC pattern of microangiopathy changed in 55% of the SSc patients: 14 patients (33%) were showing the “Active” NVC pattern, 9 patients (21%) the “Late” pattern, while 19 patients (45%) were still showing the “Early” scleroderma-pattern. Concerning serological markers in nine patients whose microangiopathy showed a progression to “Late” NVC pattern through the “Active” pattern, four patients were found positive for Scl70 (44%), three patients were ACA positive (33%), and two patients were ANA positive (with nucleolar (11%) and homogeneous (11%) fluorescence pattern, respectively). Prevalence of ACA (positive in 40% of all patients) was found significantly higher in SSc patients showing either the “Early” (41%) or the “Active” (41%) NVCpatterns of microangiopathy at the end of follow-up, confirming previous observations that ACA are likely to be associated with a slower disease progression. Opposite results were obtained for the Scl70 autoantibodies (positive in 17% of patients), which were found more frequent in patients with “Late” (44%)NVC patterns of microangiopathy (p=0.05). Interestingly, 37% of patients showing the “Early” pattern of microangiopathy at the end of the follow-up were found ANA negative.

Conclusions The results of the study confirm the progressive transition of the SSc microvascular damage through different NVC patterns. Both ANA-negative and ACA positive SSc patients might display a slower progression rate of the nailfold microangiopathy. The progression to the “Late” NVC pattern (more advanced stage of microvascular damage) is associated with different autoantibody presence, and needs investigation through larger longitudinal studies.

1. Cutolo M, et al. Rheumatology 2004; 43: 719-26.

2. Sulli A, et al. Arthritis Rheum 2012; 62: 000-000. In press.

3. Cutolo M, et al. Nat Rev Rheumatol 2010; 6: 578-87.

Disclosure of Interest None Declared