Nailfold videocapillaroscopy micro-haemorrhage and giant capillary counting as an accurate approach for a steady state definition of disease activity in systemic sclerosis.

Domenico Sambataro,Eleonora Zaccara,Wanda Maglione,Claudio Vitali,Gianluca Sambataro,Riccardo Polosa,Antonella MV Afeltra,Nicoletta Del Papa

doi:10.1186/s13075-014-0462-8

Abstract

IntroductionNailfold videocapillaroscopy (NVC) in systemic sclerosis (SSc) is a procedure commonly used for patient classification and subsetting, but not to define disease activity (DA). This study aimed to evaluate whether the number of micro-haemorrhages (MHE), micro-thrombosis (MT), giant capillaries (GC), and normal/dilated capillaries (Cs) in NVC could predict DA in SSc.MethodsEight-finger NVC was performed in 107 patients with SSc, and the total number of MHE/MT, GC, and the mean number of Cs were counted and defined as number of micro-haemorrhages (NEMO), GC and Cs scores, respectively. The European Scleroderma Study Group (ESSG) index constituted the gold standard for DA assessment, and scores ≥3.5 and =3 were considered indicative of high and moderate activity, respectively.ResultsNEMO and GC scores were positively correlated with ESSG index (R = 0.65, P <0.0001, and R = 0.47, P <0.0001, respectively), whilst Cs score showed a negative correlation with that DA index (R = −0.30, P <0.001). The area under the curve (AUC) of receiver operating characteristic plots, obtained by NEMO score sensitivity and specificity values in classifying patients with ESSG index ≥3.5, was significantly higher than the corresponding AUC derived from either GC or Cs scores (P <0.03 and P <0.0006, respectively). A modified score, defined by the presence of a given number of MHE/MT and GC, had a good performance in classifying active patients (ESSG index ≥3, sensitivity 95.1%, specificity 84.8%, accuracy 88.7%).ConclusionsMHE/MT and GC appear to be good indicators of DA in SSc, and enhances the role of NVC as an easy technique to identify active patients.

Highlights

Nailfold videocapillaroscopy (NVC) in systemic sclerosis (SSc) is a procedure commonly used for patient classification and subsetting, but not to define disease activity (DA)
This allows distinguishing patients with early, active, and late NVC pattern, where the active pattern is defined by the presence of numerous ectasic and giant capillaries (GC), ramified capillaries, micro-haemorrhages (MHE) and micro-thrombosis (MT), and initial loss of capillaries with avascular areas [15]
Active patients became 41 when an European Scleroderma Study Group (ESSG) index ≥3.0 was used as cut-off value to included patients with moderate DA (26 with lcSSc and 15 with dcSSc)

Summary

Introduction

Nailfold videocapillaroscopy (NVC) in systemic sclerosis (SSc) is a procedure commonly used for patient classification and subsetting, but not to define disease activity (DA). This study aimed to evaluate whether the number of micro-haemorrhages (MHE), micro-thrombosis (MT), giant capillaries (GC), and normal/dilated capillaries (Cs) in NVC could predict DA in SSc. Systemic sclerosis (SSc) is a connective tissue disease clinically characterized by Raynaud’s phenomenon, and progressive fibrotic changes in the skin and internal organs, such as heart, lung, and kidney. NVC has been used to define the different phases of the disease This allows distinguishing patients with early, active, and late NVC pattern, where the active pattern is defined by the presence of numerous ectasic and giant capillaries (GC), ramified capillaries, micro-haemorrhages (MHE) and micro-thrombosis (MT), and initial loss of capillaries with avascular areas [15]

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