Capillary loss reflects disease activity and prognosis in patients with systemic sclerosis

Abstract

Capillary density on nailfold capillaroscopy (NFC) is considered a promising instrument for assessing disease characteristics in patients with systemic sclerosis (SSc), however, there is no agreement yet over how to analyze and interpret the results. The objective of this study was to investigate the possible associations of the mean number of capillaries with disease characteristics, disease activity [measured by the European Scleroderma Study Group (EScSG) disease activity score] and survival in a single-center cohort of patients with SSc. Sixty-eight patients were included; 54 had follow-up at 6 months. Thirty-two images per patient were assessed independently by two raters, scoring the mean number of capillaries in all fingers (N), in the 3rd finger of the dominant hand (dN 3) and in the 4th finger of the non-dominant hand (ndN 4) for each patient. NFC 'early', 'active' and 'late' patterns were also assessed. Two thousand and seventy-six images were scored at baseline, 1,728 at follow-up. Baseline N was median (IQR) 5.1 (2.7) for rater 1, and 4.9 (1.7) for rater 2, respectively. N was significantly lower in patients with a history of digital ulcers (DUs), vs. those who never had DUs 4.8 (1.4) vs. 6.4 (3.1), P=0.016. A lower N was associated with higher disease activity at baseline and follow-up (linear regression adjusted for age, sex and history of DUs). A lower ndN 4 was associated with increased mortality (logistic regression adjusted for age and sex). In conclusion, in patients with SSc, a lower mean number of capillaries assessed by NFC was associated with higher disease activity after 6 months of follow-up and with shorter survival.

[Retracted] The Function of Retinal Thickness and Microvascular Alterations in the Diagnosis of Systemic Sclerosis

“…[ 2 , 24 ] Maricq and Cutolo capillaroscopic classification system may be useful in recognizing patients at different phases of illness. [ 25 , 26 ] Over time, the capillaroscopic pattern in SSc patients evolved from destruction of giant capillaries to that of capillary beds [ 27 ]. Furthermore, capillaroscopy may be used to detect a window of opportunity for treatment in patients who progress from giant capillary to capillary bed destruction.…”

ANGPT1 and ANGPT2 polymorphisms in systemic sclerosis: ANGPT2 rs2442598 and rs3739390 are associated with disease susceptibility and diffuse subtype. Preliminary observations

“…Digital ulcers are thought to constitute an important clinical manifestation of SSc-related microangiopathy related to capillary loss and impaired angiogenesis that corresponds in vitro with sustained increase in Ang2 expression [5,7,11,15,[33][34][35][36][37]. However, no data has been reported yet concerning specifically DUs and angiopoietins polymorphisms in SSc.…”

Microcirculation disorders of the skin

“…With sodium fluorescein, increased diffusion into the pericapillary tissue is seen as a sign of increased permeability (Figure 15a, b). 39,40 Rheological examinations revealed increased blood viscosity. Elevated fibrinogen levels were measured, which were associated with increased aggregation of erythrocytes and platelets.…”