FRI0345 CAPILLAROSCOPY IN SYSTEMIC SCLEROSIS RELATED PULMONARY ARTERIAL HYPERTENSION

Abstract

Background Despite its importance in diagnosing SSc, the role of nailfold videocapillaroscopy (NVC) in evaluating SSc related PAH (SSc-PAH) using standardized NVC definitions has not yet been the subject of a systematic literature review. Objectives To identify and critically appraise all currently available literature evaluating NVC in SSc-PAH according to the standardized definitions of the EULAR Study Group on Microcirculation in Rheumatic Diseases (EULAR SG MC/RD). Methods Original longitudinal or cross-sectional studies, documenting an association between NVC parameters and SSc-PAH (confirmed on right heart catheterization) were identified, by means of a systematic literature search in PubMed, EMBASE and Web of Science. All retrieved records were screened by two raters based on title, abstract and full-text level to finally include manuscripts eligible for quality appraisal and data extraction. NVC parameters were evaluated as proposed by the EULAR SG MC/RD as follows: quantitative (capillary density, dimension, morphology and haemorrhages), semiquantitative and qualitative assessment (NVC pattern). Results The systematic search retrieved 316 records, of which a total of 234 unique titles were screened. Of those, 28 records eligible for full-text review. Finally, only 5 fulfilled the predetermined eligibility criteria. Two high-quality longitudinal studies were identified [1,2]. Both baseline and progression of capillary loss were found to be associated with incident SSc-PAH [1,2]. Both studies reported an association between baseline abnormal capillary morphology and incident SSc-PAH [1,2]. No association with incident SSc-PAH was found between capillary dimension nor presence of haemorrhages [1]. Concerning qualitative assessment, Avouac et al. reported worsening to a late NVC pattern over time to be more commonly associated with incident SSc-PAH [1]. Finally, Sulli et al. found severe NVC patterns (i.e. active or late) to be more commonly associated with incident SSc-PAH [2]. Three fair-quality cross-sectional studies were identified [3-5]. Capillary density has been unequivocally inversely associated with SSc-PAH [3,4]. Capillary dimension has been evaluated in 2 studies, with no unequivocal results [3,4]. Corrado et al. found abnormal morphology to be more commonly associated with SSc-PAH [4]. No association was found between presence of haemorrhages and SSc-PAH [4]. Riccieri et al. found that NVC score and avascular area grade were more commonly associated with SSc-PAH [5]. Concerning qualitative assessment, severe NVC patterns have been unequivocally found to be more commonly associated with SSc-PAH [4,5]. Conclusion This is the first systematic literature review to investigate the role of NVC in SSc-PAH using standardized definitions as proposed by the EULAR SG MC/RD. Unequivocal associations with incident SSc-PAH were found in longitudinal studies between capillary density, abnormal morphology and NVC pattern. Unequivocal associations with SSc-PAH were found in cross-sectional studies between capillary density and severe NVC pattern.