Nailfold Microvascular Imaging by Dynamic Optical Coherence Tomography in Systemic Sclerosis: A Case-Controlled Pilot Study.

In systemic sclerosis, outcome measures of skin microvasculopathy are needed for both clinical trials and practice. The aim of this study was to determine whether dynamic-optical coherence tomography (D-OCT) is able to provide information on microvasculopathy compared with the current gold standard, nailfold videocapillaroscopy (NVC), in patients with systemic sclerosis. This case-controlled study included (i) 40 patients with systemic sclerosis, classified by NVC pattern in four age- and sex-matched groups (normal/nonspecific, early, active, late); (ii) a fifth group of 10 age- and sex-matched healthy controls. All participants underwent NVC and D-OCT. D-OCT images were compared with the corresponding NVC images. Reliability was assessed. D-OCT images visualized the corresponding NVC patterns. D-OCT microvascular flow density was different across the five NVC pattern groups (P = 0.0114) with a significant trend test (P = 0.0006). Microvascular flow density correlated with the NVC semiquantitative score (r = -0.7, P < 0.0001), number of abnormal shapes/mm (r = ‒0.3, P = 0.0264), and number of capillaries/mm (r = 0.6, P < 0.0001). Reliability was excellent (intraclass correlation coefficient > 0.9). In conclusion, in patients with systemic sclerosis, D-OCT provided qualitative and quantitative information on nailfold microvasculopathy, showing a correlation between microvascular flow density and NVC scores. The development of D-OCT as a standardized imaging technique could provide a quantitative outcome measure in clinical trials and practice.

European multicentre study validates enhanced liver fibrosis test as biomarker of fibrosis in systemic sclerosis.

Objectives: To validate enhanced liver fibrosis (ELF) test and its components-amino-terminal propeptide of procollagen type III (PIIINP), tissue inhibitor of matrix metalloproteinase-1 (TIMP-1) and HA-as biomarkers of fibrosis in SSc in an independent, international, multicentre cohort. Methods: Two hundred and fifty-four SSc patients from six Rheumatology Centres were included. Sera were collected and stored according to EUSTAR biobanking recommendations and analysed through automated high throughput diagnostics. Statistical analysis was performed with SPSS software. Results: Two hundred and forty-seven SSc patients (mean age 55.7 ± 13.9 years, 202 F) were analysed. ELF score, TIMP-1 and PIIINP levels were higher in males (P = 0.0197, P = 0.0107, P = 0.0108 respectively) and in dcSSc (P = 0.001, P = 0.0008, P < 0.0001 respectively). ELF score and the single markers significantly correlated with modified Rodnan skin score (r = 0.37, P < 0.0001), disease activity and severity (P < 0.0001 for all markers, except for HA P = 0.0001) and inversely with forced vital capacity, (FVC) % (TIMP-1, r = -0.21, P = 0.0012; PIIINP, r = -0.26, P = 0.0001), TLC% (ELF score, r = -0.20, P = 0.0036; TIMP-1, r = -0.32, P < 0.0001; PIIINP, r = -0.28, P < 0.0001), diffusion capacity of the lung for carbon monoxide (DLCO) % (P < 0.0001 for all markers, except for HA P = 0.0115). Multivariate analysis indicated that age (P < 0.001), modified Rodnan skin score (P < 0.001) and DLCO% (P = 0.005) were independently associated with ELF score. Conclusion: Between the first and this validation studies, the value of the ELF score as independent marker of skin and lung involvement in SSc is confirmed in 457 patients. A longitudinal study is on-going to identify an SSc specific algorithm with predictive value for skin and lung progression.