Classification, categorization and essential items for digital ulcer evaluation in systemic sclerosis: a DeSScipher/European Scleroderma Trials and Research group (EUSTAR) survey

Jelena Blagojević ,Laura Cometi ,Ulrich A Walker ,Marc Frerix ,Gabriela Riemekasten ,Britta Maurer ,Serena Vettori ,Francesco Del Galdo ,Svetlana I Nihtyanova ,Ingo H Tarner ,Ulf Müller‐Ladner ,Silvia Bellando-Randone ,László Czirják ,Elise Siegert ,Jérôme Avouac ,Serena Guiducci ,Veronika Lóránd ,Veronika K Jaeger ,Christopher P Denton ,Yannick Allanore ,Giuseppina Abignano ,Dörte Huscher ,Oliver Distler ,Marco Matucci‐Cerinic

doi:10.1186/s13075-019-1822-1

Abstract

BackgroundA consensus on digital ulcer (DU) definition in systemic sclerosis (SSc) has been recently reached (Suliman et al., J Scleroderma Relat Disord 2:115-20, 2017), while for their evaluation, classification and categorisation, it is still missing. The aims of this study were to identify a set of essential items for digital ulcer (DU) evaluation, to assess if the existing DU classification was useful and feasible in clinical practice and to investigate if the new categorisation was preferred to the simple distinction of DU in recurrent and not recurrent, in patients with systemic sclerosis (SSc).MethodsDeSScipher is the largest European multicentre study on SSc. It consists of five observational trials (OTs), and one of them, OT1, is focused on DU management. The DeSScipher OT1 items on DU that reached ≥ 60% of completion rate were administered to EUSTAR (European Scleroderma Trials and Research group) centres via online survey. Questions about feasibility and usefulness of the existing DU classification (DU due to digital pitting scars, to loss of tissue, derived from calcinosis and gangrene) and newly proposed categorisation (episodic, recurrent and chronic) were also asked.ResultsA total of 84/148 (56.8%) EUSTAR centres completed the questionnaire. DeSScipher items scored by ≥ 70% of the participants as essential and feasible for DU evaluation were the number of DU defined as a loss of tissue (level of agreement 92%), recurrent DU (84%) and number of new DU (74%). For 65% of the centres, the proposed classification of DU was considered useful and feasible in clinical practice. Moreover, 80% of the centres preferred the categorisation of DU in episodic, recurrent and chronic to simple distinction in recurrent/not recurrent DU.ConclusionsFor clinical practice, EUSTAR centres identified only three essential items for DU evaluation and considered the proposed classification and categorisation as useful and feasible. The set of items needs to be validated while further implementation of DU classification and categorisation is warranted.Trial registrationObservational trial on DU (OT1) is one of the five trials of the DeSScipher project (ClinicalTrials.gov; OT1 Identifier: NCT01836263, posted on April 19, 2013).

Highlights

A consensus on digital ulcer (DU) definition in systemic sclerosis (SSc) has been recently reached (Suliman et al, J Scleroderma Relat Disord 2:115-20, 2017), while for their evaluation, classification and categorisation, it is still missing
Since different types of DU may occur in SSc, a DU classification according to their main features into DU associated digital pitting scars, DU associated with calcinosis, DU due to loss of tissue not associated with DPS or calcinosis (Pure DU) (Fig. 1) and DU associated with gangrene has been proposed [9]
The aims of this study were to identify in SSc a set of essential items for DU evaluation in clinical practice, starting from a large core of items contained in the OT1, to assess if the existing DU classification was useful and feasible in clinical practice and to investigate whether the DU categorisation was preferred to the simple classification of DU

Summary

Introduction

A consensus on digital ulcer (DU) definition in systemic sclerosis (SSc) has been recently reached (Suliman et al, J Scleroderma Relat Disord 2:115-20, 2017), while for their evaluation, classification and categorisation, it is still missing. The aims of this study were to identify a set of essential items for digital ulcer (DU) evaluation, to assess if the existing DU classification was useful and feasible in clinical practice and to investigate if the new categorisation was preferred to the simple distinction of DU in recurrent and not recurrent, in patients with systemic sclerosis (SSc). In systemic sclerosis (SSc), the pathophysiology is characterised by immune, endothelial and fibroblast dysfunction [1] and microvascular involvement is one of the most important features of the disease [2]. It consists of five observational trials (OTs) focusing on DU (OT1), hand arthritis, interstitial lung disease, pulmonary hypertension and heart disease

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