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1.
Autoimmun Rev ; : 102458, 2020 Jan 10.
Artigo em Inglês | MEDLINE | ID: mdl-31927087

RESUMO

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.

2.
Clin Chem Lab Med ; 2019 Nov 12.
Artigo em Inglês | MEDLINE | ID: mdl-31714880

RESUMO

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.

3.
Autoimmun Rev ; 18(11): 102394, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31520797

RESUMO

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.


Assuntos
Algoritmos , Esclerodermia Localizada/diagnóstico , Escleroderma Sistêmico/diagnóstico , Humanos , Angioscopia Microscópica/métodos , Reprodutibilidade dos Testes
4.
Artigo em Inglês | MEDLINE | ID: mdl-31526595

RESUMO

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.

5.
J Rheumatol ; 2019 Aug 15.
Artigo em Inglês | MEDLINE | ID: mdl-31416927

RESUMO

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 futurely add to performance characteristics would be very welcome. Against this background, 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 catheterisation defined SSc-PAH was performed according to the PRISMA guidelines. Subsequently, NVC parameters were subdivided into quantitative (capillary density, dimension, morphology, and haemorrhages), semi-quantitative and qualitative assessment (NVC pattern), according to the definitions of the EULAR 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. 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.

6.
Semin Arthritis Rheum ; 49(1): 119-125, 2019 08.
Artigo em Inglês | MEDLINE | ID: mdl-30660382

RESUMO

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.

7.
RMD Open ; 4(Suppl 1): e000782, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30402270

RESUMO

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.

8.
Autoimmun Rev ; 17(8): 775-780, 2018 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-29885540

RESUMO

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.


Assuntos
Fluxometria por Laser-Doppler/métodos , Imagem de Perfusão/métodos , Escleroderma Sistêmico/diagnóstico , Adulto , Estudos de Casos e Controles , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Projetos Piloto , Reprodutibilidade dos Testes , Escleroderma Sistêmico/sangue , Escleroderma Sistêmico/diagnóstico por imagem
9.
Autoimmun Rev ; 17(4): 344-352, 2018 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-29427827

RESUMO

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.


Assuntos
Lúpus Eritematoso Sistêmico/imunologia , Angioscopia Microscópica/métodos , Unhas/irrigação sanguínea , Capilares , Feminino , Humanos , Masculino
11.
PLoS One ; 12(6): e0179062, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-28598975

RESUMO

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.


Assuntos
Escleroderma Sistêmico/sangue , Deficiência de Vitamina D/sangue , Adulto , Idoso , Bélgica , Biomarcadores , Suplementos Nutricionais , Feminino , Humanos , Itália , Masculino , Pessoa de Meia-Idade , Escleroderma Sistêmico/diagnóstico , Escleroderma Sistêmico/tratamento farmacológico , Escleroderma Sistêmico/epidemiologia , Estações do Ano , Índice de Gravidade de Doença , Vitamina D/análogos & derivados , Vitamina D/sangue , Deficiência de Vitamina D/epidemiologia
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