Ab0680 naifold Videocapillaroscopy Pattern Correlates With Organ Involvement and Disease Outcome in Systemic Sclerosis: A Single-Center Observational Study

Abstract

BackgroundSystemic sclerosis (SSc) is characterized by systemic autoimmunity, vasculopathy and fibrosis. Nailfold Videocapillaroscopy (NVC) is a safe method to detect and to analyze morphological microvascular abnormalities in SSc patients. There is circumstantial evidence to suggest an association between NVC abnormalities and disease severity, particularly vascular manifestations.1 ObjectivesTo evaluate the association of NVC patterns with demographic, clinical, laboratory features and disease progression in the SSc cohort of University Hospital of Heraklion.MethodsRetrospective study (2011-2018) of SSc patients who underwent NVC. NVC findings were classified into three distinct patterns namely early, active and late according to Cutolo et al2 . NVC patterns were correlated with the clinical characteristics of the patients. We also correlated baseline NVC patterns with the progression of the disease at 3 years.Results62 patients [(88.8% women, mean age of diagnosis 49.8 years, range 18-82)] were included. Compared to early/active pattern, patients with late pattern were more likely to have diffuse SSc (dSSc) (p=0.006), interstitial Lung Disease (ILD) (80.0% vs 38.1% p=0.002) and esophageal involvement (80.0% vs 47.6% p=0.0034). There was a trend for increased frequency of Pulmonary arterial Hypertension (PAH), digital ulcers (DU) and calcinosis in patients with late pattern (p=0.079, p=1.103 and p=0.079 respectively). Patients with active NVC pattern were more likely to have arthritis (early/active/late: 36.4%/75.9%/55.0%, p=0.054). A total 42 patients had NVC data at the time of SSc diagnosis. At 3 years there were no significant differences regarding disease progression, although patients with late NVC pattern had more hospitalizations due to infections (33.3% vs 9.1%, p=0.191), higher death rate (16.7% vs. 8.3%, p=0.543), need to use cyclophosphamide (33.3% vs 17.4%, p=0.391) and/or bosentan (40.0% vs 22.7%, p=0.426).ConclusionIn this single-center SSc cohort, NVC pattern was found to correlate with organ involvement especially ILD, esophageal involvement and dSSc. Serious infections and treatment intensification were also more frequent among patients with late NVC pattern. NVC may be a useful prognostic marker for SSc patients.References[1] Paxton Det al, Semin arthritis Rheum. 2018Dec;48(3):482-494[2] Cutolo M, et al. Assessing microvascular changes in systemic sclerosis diagnosis and management, Nat Rev Rheumatol. 2010AcknowledgementThis study was funded by Hellenic Society of Rheumatology & Professionals Union of Rheumatologists of GreeceDisclosure of interestsNone declared