Publications by authors named "Amber Vanhaecke"

20 Publications

  • Page 1 of 1

Laser speckle contrast analysis is a reliable measure of digital blood perfusion in Black Africans with systemic sclerosis.

Clin Exp Rheumatol 2021 Jul 7. Epub 2021 Jul 7.

Department of Internal Medicine, Ghent University; Department of Rheumatology, Ghent University Hospital; Unit for Molecular Immunology and Inflammation, VIB Inflammation Research Centre (IRC), Ghent, Belgium.

Objectives: Laser speckle contrast analysis (LASCA) is evolving as a promising non-invasive tool to assess cutaneous microvascular function in systemic sclerosis (SSc). Reliability studies have mainly focused on Caucasian populations. To determine for the first time the inter-rater reliability of fingertip blood perfusion (BP) using LASCA in Black South African patients with SSc.

Methods: Consecutive Black adult patients with SSc were evaluated for peripheral BP using LASCA. Mean BP in defined regions of interest for dorsal fingertips and volar fingertips were measured in two subgroups of 20 SSc patients, each by three independent operators. Two operators were experienced in the use of the LASCA instrument and one was newly trained. Standardised protocols for conditions were followed for all measurements. Inter-rater reliability was tested using the intraclass correlation coefficient (ICC).

Results: The majority (87.5%) of the 40 patients included were females and 67.5% had diffuse cutaneous SSc. The mean age (standard deviation) was 48.5 (9.9) years and the median disease duration (interquartile range) was 8.5 (4, 13) years. There was good to excellent agreement, inter-rater ICC (dorsal fingertip range: 0.86-0.97 and volar fingertip range: 0.85-0.96), in both subgroups irrespective of operator skill.

Conclusions: LASCA is a credible instrument in patients of Black ethnicity with SSc, and across operator experience.
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July 2021

Reliable detection of subtypes of nailfold capillary haemorrhages in childhood-onset systemic lupus erythematosus.

Clin Exp Rheumatol 2021 Jun 8. Epub 2021 Jun 8.

Department of Internal Medicine, Ghent University, and Department of Rheumatology, Ghent University Hospital, Ghent, Belgium.

Objectives: In systemic lupus erythematosus (SLE), it is necessary to obtain biomarkers that predict cardiovascular complications due to premature atherosclerosis, which is related to endothelial dysfunction. Nailfold capillary abnormalities might be a biomarker for endothelial dysfunction. In adults and children with SLE, nailfold capillary haemorrhages have shown to be significantly correlated with disease activity. Recently, different subtypes of capillary haemorrhages have been described in childhood-onset SLE (cSLE). The aim of the current study was to assess the inter- and intra-rater reliability of observations of different subtypes of haemorrhages in cSLE patients.

Methods: Five raters blindly evaluated 140 capillaroscopy images from 35 cSLE-patients (diagnosed according to the 2012 SLICC criteria). The images were assessed qualitatively (present or absent) and quantitatively (total number) on four different subtypes of haemorrhages: 1) punctate extravasations, 2) perivascular haemorrhage, 3) large confluent haemorrhage and 4) non-definable. As subgroups 1) and 2) were interpreted as a continuous spectrum, a post-hoc analysis with "merged" (mean) kappa/ICC was additionally calculated as one sub-group.

Results: Qualitative assessment showed a kappa 0.65 (95% CI: 0.60-0.70) for "punctate extravasations and perivascular haemorrhages merged" and a kappa 0.78 (95% CI: 0.72-0.83) for large confluent haemorrhages. For the quantitative assessment, ICC was 0.82 (95% CI: 0.76-0.87) for the "merged groups" and ICC 0.93 (95% CI: 0.91-0.95) for large confluent haemorrhages.

Conclusions: Our study shows that different subtypes of capillary haemorrhages in cSLE-patients could be reliably reproduced by different raters. This confirms our recent observation of perivascular extravasations as a subgroup of capillary haemorrhage in cSLE that might reflect endothelial dysregulation.
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June 2021

HIGH FREQUENCY ULTRASONOGRAPHY: RELIABLE TOOL TO MEASURE SKIN FIBROSIS IN SSC? A systematic literature review and additional pilot study.

Rheumatology (Oxford) 2021 May 25. Epub 2021 May 25.

Department of Internal Medicine, Ghent University, Ghent, Belgium.

Objectives: To investigate the reliability of high frequency ultrasound (HFUS) in measuring skin fibrosis in systemic sclerosis (SSc).

Methods: First, a systematic review (according to PRISMA) was conducted to identify studies that documented HFUS' reliability in SSc as a primary outcome. Then, in an additional pilot study, the inter- and intra-rater reliability of two investigators performing HFUS for dermal thickness (DT) measurements in a standardised manner across all 17 areas of the modified Rodnan Skin Score was evaluated in a group of 59 SSc patients and descriptively in 44 healthy controls (HC). As an external validation, DT measurements by HFUS were performed in a separate group of 30 SSc patients by the same first and another third investigator.

Results: The systematic review retained few (4/1719 unique records) small-scale studies, with mixed study populations (combining SSc and HC). The reported data herein are suggestive of the inter-/intra-rater reliability of HFUS (ICCs ranging 0.65-0.94/0.55-0.96, respectively). Additionally, in our pilot study, inter-/intra-rater reliability was good-to-excellent in both SSc groups and HC (ICCs ranging 0.70-0.97/0.70-0.98 and 0.65-0.95/0.63-0.96, respectively).

Conclusion: The identified small-scale studies were not only combining data from SSc and HC, they were also heterogeneous in terms of technical aspects (probes and frequency), image acquisition methods ([number of] areas assessed) and definitions used for skin thickness, which prevents drawing unequivocal conclusions. Despite these limitations, our standardized pilot study corroborated the findings in literature, paving the way for the applicability of HFUS as a reliable (complementary) tool to quantify skin fibrosis in SSc.
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http://dx.doi.org/10.1093/rheumatology/keab462DOI Listing
May 2021

Laser speckle contrast analysis in rheumatoid arthritis: a pilot study.

Clin Exp Rheumatol 2021 Mar 9. Epub 2021 Mar 9.

Department of Internal Medicine, Ghent University, Department of Rheumatology, Ghent University Hospital, and Unit for Molecular Immunology and Inflammation, VIB Inflammation Research Center (IRC), Ghent, Belgium.

Objectives: Early diagnosis and treatment is paramount in rheumatoid arthritis (RA). Nowadays, there is a need for quick and non-invasive imaging modalities, for which laser speckle contrast analysis (LASCA), a technique that assesses the peripheral blood perfusion (PBP) on a microvascular level, seems to be a promising candidate. The goal of this pilot study was to examine whether the expected increased PBP in active synovitis in RA patients can be detected by LASCA.

Methods: Thirty RA patients with active synovitis in a finger joint and 44 healthy controls (HC) underwent LASCA examination. The PBP measured over the finger joints was expressed in perfusion units (PU). For the final analysis, all 30 RA patients were matched by age and gender to 30 HC. For the primary analysis the mean difference in PU between joints with active synovitis compared to matched HC, adjusted for type of joint (MCP/PIP), finger, surface and side of hand and for the matching variables (age and gender), was calculated using a multilevel linear model. For the secondary analysis this mean difference in PU was calculated on a monoarticular level.

Results: The primary analysis showed an estimated mean difference of 8.79 PU (95%CI -7.79-25.37 PU; p=0.299). For the secondary analysis on a monoarticular level, none of the estimated mean differences differed significantly.

Conclusions: In this pilot study examining the use of LASCA in RA, no significant difference in estimated mean PBP between joints with active synovitis in RA and joints without active synovitis in HC could be detected.
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March 2021

Durometry in SSc: The hard facts. A systematic literature review and additional pilot study.

Rheumatology (Oxford) 2021 05;60(5):2099-2108

Department of Internal Medicine, Ghent University.

Objectives: To investigate the reliability of durometry in systemic sclerosis (SSc), by means of a systematic review and additional pilot study.

Methods: Literature was systematically reviewed according to the PRISMA guidelines to identify all original studies assessing the reliability of durometry in SSc. Additionally, in the pilot study, intra-rater reliability was evaluated in a first cohort of 74 SSc patients (61 female, 13 LSSc/53 LcSSc/8 DcSSc). In a second separate set of 30 SSc patients (21 female, 4 LSSc/20 LcSSc/6 DcSSc), intra- and inter-rater reliability were evaluated.

Results: Only two unique records identified through the systematic review were qualified to generate conclusions. Regarding intra-rater reliability, Kissin reported excellent intra-class correlation coefficient values (ICC, 0.86-0.94) for measurements at nine skin sites in two DcSSc patients. Merkel and Kissin described, both in five DcSSc patients, good to excellent inter-rater reliability (ICC, 0.82-0.96 and 0.61-0.85) for measurements at respectively, six and nine skin sites. In our pilot study, ICC for intra-rater reliability at 17 standardized skin sites were excellent in both cohorts, ranging 0.93-0.99 and 0.78-0.98, respectively. ICC for inter-rater reliability at 17 standardized skin sites were good to excellent 0.63-0.93, except for the feet (0.48 and 0.52).

Conclusion: The preliminary findings in the literature are supported by our pilot study in which we have attested the reliability of durometry in SSc patients. However, prior to including durometry as an (additional) outcome measure in SSc clinical trials, its validation status in the assessment of skin fibrosis needs to be completely attested.
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http://dx.doi.org/10.1093/rheumatology/keab028DOI Listing
May 2021

Nailfold capillaroscopy in Sjögren's syndrome: a systematic literature review and standardised interpretation.

Clin Exp Rheumatol 2020 Jul-Aug;38 Suppl 126(4):150-157. Epub 2020 Sep 21.

Department of Rheumatology, Ghent University Hospital; Department of Internal Medicine, Ghent University, and Unit for Molecular Immunology and Inflammation, VIB Inflammation Research Centre (IRC), Ghent, Belgium.

Objectives: To identify the role of nailfold capillaroscopy (NC) in Sjögren's syndrome (SS).

Methods: The literature was systematically reviewed in three databases. All published original studies which assess patients with SS by NC were revised. A quality assessment was applied to all studies based on population description, presence of a control group, presence of instrumental specifications and/or standardly applied NC methodology, presence of clear descriptions of capillaroscopic characteristics and based on the used statistical analysis. The capillaroscopic findings per study were described in a EULAR consented standardised way. Significant associations of capillaroscopic characteristics in SS patients with clinical and laboratory variables were summarised.

Results: The search resulted in 869 hits. Based on title and abstract screening 29 original studies were identified and of these, 14 full texts described an assessment by NC in SS. Seven studies were retained after performing a critical quality assessment. One study compared NC in SS with healthy controls and attested a lower capillary density in SS. Concerning clinical associations, capillary density was associated with Raynaud's phenomenon in two studies and with interstitial lung disease or systemic manifestations in one study each. No association between serologic features (anti-nuclear antibodies, anti-SSA, anti-SSB and anti-RF) and NC characteristics were found.

Conclusions: A small number of studies have investigated the role of NC in SS. More studies, including prospective follow up studies with standard NC evaluation in SS are needed.
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October 2020

May capillaroscopy be a candidate tool in future algorithms for SSC-ILD: Are we looking for the holy grail? A systematic review.

Autoimmun Rev 2020 Sep 12;19(9):102619. Epub 2020 Jul 12.

Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, IRCCS San Martino Polyclinic Hospital, Genoa, Italy. Electronic address:

Objective: To investigate whether nailfold videocapillaroscopy (NVC), an increasingly worldwide used non-invasive tool to reliably evaluate the peripheral microcirculation, may be an outcome measure in future screening algorithms for systemic sclerosis related interstitial lung disease (SSc-ILD).

Methods: A systematic review to identify original research papers documenting an association between NVC and SSc-ILD was performed in 3 electronic databases according to the PRISMA guidelines. Subsequently, NVC parameters were subdivided according to the consented standardised capillaroscopic definitions of the EULAR Study Group on Microcirculation in Rheumatic Diseases / Scleroderma Clinical Trials Consortium on Capillaroscopy, into quantitative (capillary density, capillary dimension, capillary morphology and haemorrhages) and qualitative assessment (NVC pattern).

Results: The systematic search identified 310 unique search results, of which 2 cross-sectional and 1 longitudinal study were retained. In both cross-sectional studies, the presence of SSc-ILD was found to be inversely associated with capillary density (p = .008 and p = .005). The presence of a severe (active/late) NVC pattern was evaluated and associated with the presence of SSc-ILD in one of the cross-sectional studies. In the longitudinal study, incident SSc-ILD was associated with progressive capillary loss (p = .03) and the conversion to a worse (active/late) NVC pattern (p = .001/p = .003).

Conclusions: This first systematic literature review investigating the role of NVC in SSc-ILD using standardised capillaroscopic definitions uncovered associations between NVC and (incident) SSc-ILD. If large prospective studies further corroborate and elucidate these findings, NVC might possibly be a candidate outcome measure to be integrated in screening algorithms for incident/progressive SSc-ILD.
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http://dx.doi.org/10.1016/j.autrev.2020.102619DOI Listing
September 2020

Coexistence of systemic and localized scleroderma: a systematic literature review and observational cohort study.

Rheumatology (Oxford) 2020 Oct;59(10):2725-2733

Department of Internal MedicineGhent University.

Objective: SSc and localized sclerosis (LoS) are considered clinically distinct entities. We describe herein the coexistence of SSc and LoS by both a systematic literature review and an observational cohort study of unselected SSc patients.

Methods: Original studies documenting the coexistence of SSc and LoS were identified in three electronic databases by means of a systematic literature search according to the Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) guidelines. Additionally, the coexistence of SSc and LoS was studied in a prospective cohort of SSc patients visiting the Ghent University Scleroderma Unit for their yearly follow-up visit between January 2018 and January 2019.

Results: Five studies were finally included for quality appraisal and data extraction. The coexistence of SSc and LoS ranged between 2.4 and 7.4%. RP, scleroderma pattern on nailfold videocapillaroscopy (NVC) and the presence of SSc-specific antibodies were commonly observed in coexistent cases. Additionally, coexistence of SSc and LoS was found in 8/296 (2.7%) consecutive SSc patients of the Ghent University Scleroderma Unit. RP was present in 6/8 coexistent cases; a scleroderma pattern on NVC was observed in all coexistent cases, and SSc-specific antibodies (i.e. cenp-B) were found in 4/8 coexistent cases.

Conclusion: This is the first systematic literature review with additional cohort evaluation investigating the coexistence of SSc and LoS. A relatively high overlap of SSc and LoS was revealed, which is peculiar because both are rare diseases.
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http://dx.doi.org/10.1093/rheumatology/keaa297DOI Listing
October 2020

Development of eosinophilic fasciitis during treatment with certolizumab pegol for ankylosing spondylitis.

Clin Exp Rheumatol 2020 Jul-Aug;38(4):799. Epub 2020 Apr 3.

Department of Rheumatology, Ghent University Hospital, Ghent, Belgium.

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September 2020

Standardisation of nailfold capillaroscopy for the assessment of patients with Raynaud's phenomenon and systemic sclerosis.

Autoimmun Rev 2020 Mar 10;19(3):102458. Epub 2020 Jan 10.

Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, IRCCS San Martino Polyclinic Hospital, Genoa, Italy. Electronic address:

Capillaroscopy is a non-invasive and safe tool which allows the evaluation of the morphology of the microcirculation. Since its recent incorporation in the 2013 American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) classification criteria for systemic sclerosis together with its assessed role to monitor disease progression, capillaroscopy became a 'mainstream' investigation for rheumatologists. Given its increasing use by a variety of physicians internationally both in daily practice to differentiate primary from secondary Raynaud's phenomenon, as well as in research context to predict disease progression and monitor treatment effects, standardisation in capillaroscopic image acquisition and analysis seems paramount. To step forward to this need, experts in the field of capillaroscopy/microcirculation provide in this very consensus paper their view on image acquisition and analysis, different capillaroscopic techniques, normal and abnormal capillaroscopic characteristics and their meaning, scoring systems and reliability of image acquisition and interpretation.
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http://dx.doi.org/10.1016/j.autrev.2020.102458DOI Listing
March 2020

Evaluation of the primary biliary cholangitis-related serologic profile in a large cohort of Belgian systemic sclerosis patients.

Clin Chem Lab Med 2020 02;58(3):416-423

Department of Diagnostic Sciences, Ghent University, Ghent, Belgium.

Background Systemic sclerosis (SSc) and primary biliary cholangitis (PBC) are autoimmune diseases that may occur concomitantly and are both strongly associated with disease-specific autoantibodies. This study investigated the prevalence and fine specificity of PBC-specific serology (PBC-Ab) and associations with the SSc-subtypes and SSc-specific antibodies as well as the association with cholestatic liver enzymes. Furthermore, three different techniques for the detection of PBC-Ab were compared. Methods Serum of 184 Belgian SSc patients with a known SSc-antibody profile, was analyzed for PBC-Ab (antimitochondrial antibodies [AMA], anti-Gp210, anti-Sp100 and anti-PML) using indirect immunofluorescence (IIF) analysis on human epithelioma-2000 (HEp-2000) cells (ANA-IIF, Immunoconcepts) and liver-kidney-stomach tissue sections (IIF-LKS) (Menarini), and a line immunoblot (LB) (EuroImmun). Alkaline phosphatase/γ-glutamyl transferase (ALP/GGT) were evaluated at time of first sampling (t0) and after 3 years of follow-up (t3). Results PBC-Ab were present in 13% of patients and significantly correlated with centromere antibodies (anti-CENP-B), but not correlated with the limited cutaneous SSc subgroup (lcSSc). The most frequent reactivities were AMA (11%, with 9% AMA-M2) and Sp-100 antibodies (5%), showing a major overlap. There was no relevant association between the presence of PBC-Ab and ALP or GGT elevation at t0 nor at t3. Detection of AMA with IIF-LKS is comparable to LB. ANA-IIF screening was less sensitive compared to LB. Conclusions A wide range of PBC-Ab is detectable in SSc in the absence of cholestatic liver enzyme elevations, even after 3 years of follow-up. However, as these antibodies may precede PBC-disease up to 10 years further prospective follow-up of our cohort will be necessary.
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http://dx.doi.org/10.1515/cclm-2019-0655DOI Listing
February 2020

The preliminary validation of laser Doppler flowmetry in systemic sclerosis in accordance with the OMERACT filter: A systematic review.

Semin Arthritis Rheum 2020 04 6;50(2):321-328. Epub 2019 Sep 6.

Department of Internal Medicine, Ghent University, Corneel Heymanslaan 10, 9000 Ghent, Belgium; Department of Rheumatology, Ghent University Hospital, Corneel Heymanslaan 10, 9000 Ghent, Belgium; Unit for Molecular Immunology and Inflammation, VIB Inflammation Research Center (IRC), Corneel Heymanslaan 10, 9000 Ghent, Belgium. Electronic address:

Objectives: To investigate the validation status of laser Doppler flowmetry (LDF) in systemic sclerosis (SSc) according to the 'Outcome Measures in Rheumatologic Clinical Trials' (OMERACT) filter.

Methods: The literature was systematically reviewed to identify all reports assessing the microcirculatory flow in SSc patients. The OMERACT filter -including the domains of truth, discrimination and feasibility- was applied and a quality assessment was done by the 'Good Methods Checklist'. To ease the comparison between studies the results were grouped per dynamic test situation: basal, cold/heat challenge and occlusion.

Results: The literature search resulted in 4332 hits. Based on title and abstract screening 243 hits were retained and of these, 52 full texts described an assessment by LDF in SSc patients. Finally, 18 studies passed the quality assessment and form the object of this review. The review reveals that expert consensus is lacking on the face and content validity of LDF in SSc. The construct validity of LDF, on the other hand is partially validated. Conflicting results exist on the discriminant capacity of LDF in distinguishing healthy from diseased patients, primary from secondary Raynaud's phenomenon and in differentiating between disease subsets. Yet, complementing an LDF-measurement with a heat challenge, as well as the evaluation of the post-occlusive hyperaemic response, has the potential to elicit a difference between healthy and diseased patients. Lastly, data on the feasibility of LDF in SSc is lacking in the identified literature.

Conclusion: This systematic review emphasizes the very preliminary validation status of LDF in the assessment of the microcirculatory flow in SSc.
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http://dx.doi.org/10.1016/j.semarthrit.2019.08.007DOI Listing
April 2020

Fast track algorithm: How to differentiate a "scleroderma pattern" from a "non-scleroderma pattern".

Autoimmun Rev 2019 Nov 11;18(11):102394. Epub 2019 Sep 11.

Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, IRCCS San Martino Polyclinic Hospital, Genoa, Italy. Electronic address:

Objectives: This study was designed to propose a simple "Fast Track algorithm" for capillaroscopists of any level of experience to differentiate "scleroderma patterns" from "non-scleroderma patterns" on capillaroscopy and to assess its inter-rater reliability.

Methods: Based on existing definitions to categorise capillaroscopic images as "scleroderma patterns" and taking into account the real life variability of capillaroscopic images described standardly according to the European League Against Rheumatism (EULAR) Study Group on Microcirculation in Rheumatic Diseases, a fast track decision tree, the "Fast Track algorithm" was created by the principal expert (VS) to facilitate swift categorisation of an image as "non-scleroderma pattern (category 1)" or "scleroderma pattern (category 2)". Mean inter-rater reliability between all raters (experts/attendees) of the 8th EULAR course on capillaroscopy in Rheumatic Diseases (Genoa, 2018) and, as external validation, of the 8th European Scleroderma Trials and Research group (EUSTAR) course on systemic sclerosis (SSc) (Nijmegen, 2019) versus the principal expert, as well as reliability between the rater pairs themselves was assessed by mean Cohen's and Light's kappa coefficients.

Results: Mean Cohen's kappa was 1/0.96 (95% CI 0.95-0.98) for the 6 experts/135 attendees of the 8th EULAR capillaroscopy course and 1/0.94 (95% CI 0.92-0.96) for the 3 experts/85 attendees of the 8th EUSTAR SSc course. Light's kappa was 1/0.92 at the 8th EULAR capillaroscopy course, and 1/0.87 at the 8th EUSTAR SSc course.

Conclusion: For the first time, a clinical expert based fast track decision algorithm has been developed to differentiate a "non-scleroderma" from a "scleroderma pattern" on capillaroscopic images, demonstrating excellent reliability when applied by capillaroscopists with varying levels of expertise versus the principal expert and corroborated with external validation.
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http://dx.doi.org/10.1016/j.autrev.2019.102394DOI Listing
November 2019

Nailfold Videocapillaroscopy in Systemic Sclerosis-related Pulmonary Arterial Hypertension: A Systematic Literature Review.

J Rheumatol 2020 06 15;47(6):888-895. Epub 2019 Aug 15.

From the Department of Internal Medicine, Ghent University; the Department of Rheumatology, and the Department of Cardiology, Ghent University Hospital; the Unit for Molecular Immunology and Inflammation, VIB Inflammation Research Center (IRC), Ghent, Belgium; Rheumatology Department, Centro Hospitalar Vila Nova de Gaia/Espinho, Vila Nova de Gaia, Portugal; Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, Institute for Research and Health Care (IRCCS) San Martino Polyclinic Hospital, Genoa, Italy.

Objective: Pulmonary arterial hypertension (PAH) is one of the leading causes of death in systemic sclerosis (SSc). Current screening algorithms are hampered by low positive predictive values. Outcome measures that could add to performance characteristics would be welcome. We aim to evaluate the role of nailfold videocapillaroscopy (NVC) using standardized definitions, in SSc-related PAH (SSc-PAH).

Methods: A systematic review to identify original research papers documenting an association between NVC and right heart catheterization-defined SSc-PAH was performed according to the Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) guidelines. Subsequently, NVC characteristics were subdivided into quantitative (capillary density, dimension, morphology, and hemorrhages), semiquantitative, and qualitative assessment (NVC pattern), according to the definitions of the European League Against Rheumatism Study Group on Microcirculation in Rheumatic Diseases.

Results: The systematic search identified 316 unique search results, of which 5 were included in the final qualitative analysis. The occurrence of incident SSc-PAH unequivocally associated in 2 longitudinal studies with progressive capillary loss (p = 0.04 and p = 0.033) and the progression to a severe (active/late) NVC pattern (p = 0.05/0.01 and HR = 5.12, 95% CI 1.23-21.27). In 3 cross-sectional studies, SSc-PAH was found to be unequivocally inversely associated with capillary density (p = 0.001 and p < 0.05) and associated with the presence of a severe NVC pattern (p = 0.03 and p < 0.05).

Conclusion: This is the first systematic literature review investigating the role of NVC in SSc-PAH using standardized description, to our knowledge. Unequivocal associations were found between (incident) SSc-PAH and capillary density and NVC pattern. Integration of NVC into current screening algorithms to boost their performance may be a future step.
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http://dx.doi.org/10.3899/jrheum.190296DOI Listing
June 2020

Ocular involvement in systemic sclerosis: A systematic literature review, it's not all scleroderma that meets the eye.

Semin Arthritis Rheum 2019 08 28;49(1):119-125. Epub 2018 Dec 28.

Department of Internal Medicine, Ghent University, Corneel Heymanslaan 10, 9000 Ghent, Belgium; Department of Rheumatology, Ghent University Hospital, Corneel Heymanslaan 10, Ghent, Belgium; Unit for Molecular Immunology and Inflammation, VIB Inflammation Research Center (IRC), Corneel Heymanslaan 10, 9000 Ghent, Belgium. Electronic address:

Objectives: Systemic sclerosis (SSc) is a rare and complex autoimmune disorder characterized by microvascular damage and progressive fibrosis which affects the skin and multiple other organs. Much of the published data concerning SSc and the eye consists of single case reports or small case studies. This systematic review aims to provide an overview of the current level of evidence for SSc-related ocular changes.

Materials And Methods: A systematic literature review was conducted using 3 electronic databases, according to the Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) guidelines. A combination of following keywords was used: "Systemic Sclerosis" and ophthalmology-related search terms, including the keywords "Eye", "Ocular" and "Ophthalmic". All articles were screened by 2 independent reviewers at title, abstract and full text level. We solely included case-control studies that investigated specific ocular findings in SSc patients compared to healthy controls.

Results: Nine of 270 articles were retained. Dry eye symptoms are associated with SSc, whereas objective signs (Schirmer I testing) show conflicting results. There is insufficient evidence of SSc-related changes to the central corneal thickness. In terms of posterior segment involvement, choroidal vasculature appears to be affected to greater extent than the retinal microcirculation. However, the limited number of patients included in the studies renders it hazardous to draw overall conclusions.

Conclusions: There is a paucity of well-designed case-control studies investigating possible ocular involvement in SSc. Our systematic review demonstrates limited proven associations between SSc and ocular abnormalities, mainly in terms of dry eye symptoms and choroidal thickness. Future standardized prospective studies are needed to clarify the impact of the disease on the eye.
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http://dx.doi.org/10.1016/j.semarthrit.2018.12.007DOI Listing
August 2019

Systemic sclerosis: state of the art on clinical practice guidelines.

RMD Open 2018 18;4(Suppl 1):e000782. Epub 2018 Oct 18.

Department of Rheumatology, Hospital de Santa Maria, Centro Hospitalar Lisboa Norte, Lisbon, Portugal.

Systemic sclerosis (SSc) is an orphan disease characterised by autoimmunity, fibrosis of the skin and internal organs, and vasculopathy. SSc may be associated with high morbidity and mortality. In this narrative review we summarise the results of a systematic literature research, which was performed as part of the European Reference Network on Rare and Complex Connective Tissue and Musculoskeletal Diseases project, aimed at evaluating existing clinical practice guidelines or recommendations. Only in the domains 'Vascular & Ulcers' (ie, non-pharmacological approach to digital ulcer), 'PAH' (ie, screening and treatment), 'Treatment' and 'Juveniles' (ie, evaluation of juveniles with Raynaud's phenomenon) evidence-based and consensus-based guidelines could be included. Hence there is a preponderance of unmet needs in SSc referring to the diagnosis and (non-)pharmacological treatment of several SSc-specific complications. Patients with SSc experience significant uncertainty concerning SSc-related taxonomy, management (both pharmacological and non-pharmacological) and education. Day-to-day impact of the disease (loss of self-esteem, fatigue, sexual dysfunction, and occupational, nutritional and relational problems) is underestimated and needs evaluation.
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http://dx.doi.org/10.1136/rmdopen-2018-000782DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6203100PMC
October 2018

Is laser speckle contrast analysis (LASCA) the new kid on the block in systemic sclerosis? A systematic literature review and pilot study to evaluate reliability of LASCA to measure peripheral blood perfusion in scleroderma patients.

Autoimmun Rev 2018 Aug 6;17(8):775-780. Epub 2018 Jun 6.

Department of Rheumatology, Ghent University Hospital, Department of Internal Medicine, Ghent University, Corneel Heymanslaan 10, Ghent, Belgium. Electronic address:

Objectives: A reliable tool to evaluate flow is paramount in systemic sclerosis (SSc). We describe herein on the one hand a systematic literature review on the reliability of laser speckle contrast analysis (LASCA) to measure the peripheral blood perfusion (PBP) in SSc and perform an additional pilot study, investigating the intra- and inter-rater reliability of LASCA.

Methods: A systematic search was performed in 3 electronic databases, according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. In the pilot study, 30 SSc patients and 30 healthy subjects (HS) underwent LASCA assessment. Intra-rater reliability was assessed by having a first anchor rater performing the measurements at 2 time-points and inter-rater reliability by having the anchor rater and a team of second raters performing the measurements in 15 SSc and 30 HS. The measurements were repeated with a second anchor rater in the other 15 SSc patients, as external validation.

Results: Only 1 of the 14 records of interest identified through the systematic search was included in the final analysis. In the additional pilot study: intra-class correlation coefficient (ICC) for intra-rater reliability of the first anchor rater was 0.95 in SSc and 0.93 in HS, the ICC for inter-rater reliability was 0.97 in SSc and 0.93 in HS. Intra- and inter-rater reliability of the second anchor rater was 0.78 and 0.87.

Conclusions: The identified literature regarding the reliability of LASCA measurements reports good to excellent inter-rater agreement. This very pilot study could confirm the reliability of LASCA measurements with good to excellent inter-rater agreement and found additionally good to excellent intra-rater reliability. Furthermore, similar results were found in the external validation.
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http://dx.doi.org/10.1016/j.autrev.2018.01.023DOI Listing
August 2018

Nailfold capillaroscopy in systemic lupus erythematosus: A systematic review and critical appraisal.

Autoimmun Rev 2018 Apr 8;17(4):344-352. Epub 2018 Feb 8.

Department of Rheumatology, Ghent University Hospital, C. Heymanslaan 10, Ghent, Belgium; Department of Internal Medicine, Ghent University, C. Heymanslaan 10, Ghent, Belgium. Electronic address:

Nailfold capillaroscopy is an easy, non-invasive technique to assess microvascular involvement in rheumatic diseases. Multiple studies describe capillaroscopic changes in systemic lupus erythematosus (SLE), including a wide range of non-specific findings. On behalf of the European League Against Rheumatism (EULAR) study group on microcirculation in rheumatic diseases, a systematic review was done to obtain all original research studies (in English) in which SLE patients had capillaroscopy. Forty such studies are identified. This article firstly provides a résumé of the results of these studies according to capillaroscopic parameters (density, dimensions, morphology, haemorrhages), semi-quantitative assessment and qualitative assessment of capillaroscopy in SLE patients. Secondly, the correlations between capillaroscopic parameters in SLE patients and clinical and laboratory parameters (including auto-immune parameters) are outlined. The following capillaroscopic parameters are found to be significantly more prevalent in SLE patients compared to healthy controls: tortuous capillaries, abnormal morphology and haemorrhages. Hairpin-shaped capillaries are significantly less prevalent than in healthy persons. The semi-quantitatively determined nailfold capillaroscopic score (NFC score) in SLE patients is also higher than in healthy controls. Several correlations between clinical and laboratory parameters and capillaroscopic parameters are identified in the review. Disease activity is correlated with NFC score in seven studies, with abnormal morphology (i.e. "meandering") in one study and with haemorrhages in one study. Frequent attacks of Raynaud's phenomenon (RP) and gangrene are significantly correlated with dilated capillaries. In two studies a possible correlation between anti-SSA antibodies and lower density of capillaries is withheld. About other immune parameters conflicting results are found. In one study a significant negative correlation is found between 24-hour proteinuria and abnormal morphology (i.e. "meandering"). For the first time, an overview of the nailfold capillaroscopic changes that have been described in SLE and their correlations with clinical and laboratory findings is given. Further large-scale research on the identification of capillaroscopic changes in SLE and their correlations with standardised clinical and laboratory parameters, is ongoing at the EULAR study group on microcirculation in rheumatic diseases.
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http://dx.doi.org/10.1016/j.autrev.2017.11.025DOI Listing
April 2018

Vitamin D deficiency and clinical correlations in systemic sclerosis patients: A retrospective analysis for possible future developments.

PLoS One 2017 9;12(6):e0179062. Epub 2017 Jun 9.

Research Laboratory And Academic Division Of Clinical Rheumatology, Department Of Internal Medicine, Irccs San Martino Aou, University Of Genoa, Genoa, Italy.

Objective: Assessment of serum 25-hydroxyvitamin D (25(OH)D) correlations with clinical parameters and evaluation of the efficacy of standard oral supplementation in systemic sclerosis (SSc) patients.

Methods: 154 SSc patients were recruited, in all seasons. Serum 25(OH)D concentrations were evaluated using LIAISON 25-OH (Diasorin, Italy). Medsger disease severity scale (DSS), nailfold videocapillaroscopy (NVC) and all instrumental exam contemplated by international guidelines were performed. Drug assumption, including oral colecalciferol, was evaluated. Non-parametric tests were used for statistical analysis.

Results: Average 25(OH)D serum concentration was 18.7 ±9 ng/ml (<20 classified as deficiency). A significant correlation was found with presence/absence of lung bi-basal fibrotic changes (16.1 ±8 ng/ml and 20 ±10 ng/ml, respectively; p = 0.04). Peripheral vascular (p = 0.03), kidney (p = 0.02), gastrointestinal (p = 0.05) Medsger's DSS parameters were found to correlate with 25(OH)D serum concentrations. No significant correlations were observed with digital ulcers incidence, strictly correlated to patterns of microangiopathy, defined at NVC analysis (p<0.0001). Interestingly, no effects of treatment with oral colecalciferol (Dibase 1,000 IU daily for at least 6 months) were found on 25(OH)D serum concentrations in treated (18.8 ±10 ng/ml) or untreated (18.7 ±9 ng/ml) SSc patients (p = 0.81). A significant difference was observed among seasonal 25(OH)D serum concentrations (winter: 14.6 ±7.8 ng/ml, spring: 17.2 ±7.9 ng/ml, summer: 21.43 ±10 ng/ml, autumn: 20.2 ±10 ng/ml; p = 0.032) in all patients.

Conclusion: Serum 25(OH)D deficiency was found to correlate with lung involvement, peripheral vascular, kidney and gastrointestinal Medsger's DSS parameters and with seasonality In SSc patients. Supplementation with oral colecalciferol was found not effective in increasing 25(OH)D serum concentrations. Therefore, for successful replacement, supra-physiological vitamin D3 doses or programmed UVB light exposure should be tested.
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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0179062PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5466326PMC
September 2017