Nailfold capillaroscopy in systemic sclerosis: data from the eular scleroderma trials and research (EUSTAR) registry

Abstract

Background: Microvascular findings in Systemic Sclerosis (SSc) document a significant loss of the peripheral vascular network, with loss of capillaries, deficient vascular repair and the absence of new vessel growth, and subsequent tissue ischemia and fibrosis. The sequence of these alterations in the microcirculation can be observed using a reliable, rapid, non-invasive examination such as nailfold capillaroscopy, which can be regarded as the most valuable technique for assisting the early diagnosis of SSc and monitoring the evolution of microangiopathy in overt SSc. Objectives: To obtain cross-sectional data on theuse of capillaroscopy in an international multi-centre cohort of SSc patients and to investigate the frequency of the capillaroscopic patterns and their disease-phenotype associations. Methods: This study examined data collected betweenJune 2004 and October 2011in the EULAR Scleroderma Trials And Research (EUSTAR) registry. SSc adult patients who had at least one capillaroscopy were analyzed. Patients' profiles based on clinical and laboratory data were obtained by cluster analysis and the association between profiles and capillaroscopy was investigated by multinomial logistic regression. Results: 67.6% of EUSTAR centers that responded to the survey perform capillaroscopy regularly using mainly the videocapillaroscope (70.3%), otherwise microscope (24.3%) and dermatoscope (5.4%) were used. 62 of 110 (56.4%) EUSTAR centers entered data on capillaroscopy in the EUSTAR database. 376 of 2754 patients (13.65%) were classified as scleroderma pattern absent, but non-specific capillary abnormalities were noted in 55.48% of cases. Four major patients' profiles were identified. They are characterised by a progressive severity for skin involvement, as well as an increased number of systemic manifestations. The \u201cearly\u201d and \u201cactive\u201d scleroderma patterns were generally observed in patients with mild/moderate skin involvement and a low number of disease manifestations, while the \u201clate\u201d scleroderma pattern was found more frequently in the more severe forms of the disease. Conclusions: This is the largest multicenter international study on SSc and capillaroscopy. These data indicate that capillaroscopic patterns are directly related to the extent of organ involvement and to disease progression. Widespread use of capillaroscopy may be of a great value in monitoring the disease course