Gastric dysrhythmias in patients with early systemic sclerosis: a cross-sectional study.

Objectives: Gastric involvement in patients with early systemic sclerosis (SSc) has not been previously investigated. We aim to evaluate the association of gastric dysrhythmias with gastrointestinal (GI) symptoms and nailfold video capillaroscopy (NVC). Methods: Cross-sectional study. Patients with early SSc, completed the UCLA GIT 2.0 questionnaire, performed an NVC, and a surface Electrogastrography (EGG). Descriptive statistics was used for demographic and clinical characteristics and Fisher and Kendall Tau tests were used for association analysis. Results: 75 patients were screened, 30 patients were consecutively enrolled, 29 performed the EGG and 1 patient had a non-interpretable NVC. 29/30 were female with a mean age of 48.7 years (25-72). The mean disease duration from the first non-RP symptom was 22.6 +/-10.8 months and most of the patients had limited disease (76.6%). Total GIT 2.0 score symptoms were moderate-severe in 63% of the participants and 28/29 had an abnormal EGG. Bradygastria was the most common pattern present in 70% of the participants. NVC patterns: 17% early, 34% active, 28% scleroderma-like, 14% non-specific, and 2 patients had a normal NVC. There was no association between severe GI symptoms or NVC patterns and severely abnormal EGG, but the presence of bradygastria was associated with severe impairment in the social functioning area (p 0.018). Conclusions: Gastric dysmotility is common in early SSc and there is a lack of correlation between GI symptoms and NVC scleroderma patterns. EGG is a sensitive, cheap, and non-invasive exam, that may be an alternative to early diagnosis of GI involvement.

Detección de cambios vasculares en el fenómeno de Raynaud cuando se utilizan la capilaroscopia y la termografía. Revisión sistemática de la literatura / Detection of vascular changes in Raynaud's phenomenon when capillaroscopy and thermography are used. Systematic literature review

RESUMEN Introducción: El fenómeno de Raynaud (FR) es una respuesta vascular exagerada al frío o al estrés que se manifiesta por cambios de coloración de la piel; puede ser primario (RP) o secundario (RS). Objetivos: El objetivo de esta revisión sistemática de la literatura (RSL) es describir y ana lizar las principales diferencias para la detección de cambios vasculares en FR entre la capilaroscopia (NC) y la termografía infrarroja (TRI). Métodos: RSL siguiendo guías Prisma en las siguientes fuentes de información: Medline, Cochrane, Pubmed, ClinicalKey y ScienceDirect. Criterios de inclusión: artículos publicados hasta septiembre del 2020, observacionales o analíticos, que incluyeran población con FR (primario o secundario), con evaluación diagnóstica concomitante utilizando NC (micros cópica o videocapilaroscopia) y TRI. En la construcción de las ecuaciones de búsqueda se utilizaron términos MeSH («Thermography¼, «Microscopic angioscopy¼) y diferentes «Keywords¼ cruzadas a través de diferentes operadores booleanos. Se utilizó la herramienta «Joanna Briggs Institute Critical Appraisal Tools for Use in Systematic Reviews Checklist¼ para evaluar el riesgo de sesgo. Se siguió la guía SWiM para sintetizar y presentar los resultados. Resultados: Se identificaron 1.397 artículos, de los que luego de cribado y elegibilidad, se incluyeron cinco. Contemplaron poblaciones diferentes, evaluadas con equipos diferentes, dos artículos en niños y tres artículos en adultos. La población fue predominantemente caucásica y de género femenino, en total 403 individuos (79 menores de edad). Los dos estudios realizados en población pediátrica presentan resultados no concordantes y los estudios en población adulta presentan similitudes en sus resultados (NC técnica diagnóstica que mejor discrimina RP de RS), pero con patologías del tejido conectivo asociadas a RS diferentes. Conclusiones: A través de RSL se presentan hallazgos microvasculares de dos herramientas diagnósticas (NC y TRI) cuando se utilizan de manera concomitante en el FR. No se puede concluir a partir de los cinco artículos que existan diferencias claras o ventajas/desventajas en el uso preferente de una de las dos técnicas diagnósticas en el FR; el valor de la NC destaca para la diferencia entre RP y RS. Se requieren nuevos estudios para analizar diferencias entre las dos técnicas.

ABSTRACT Introduction: Raynaud's phenomenon (RP) is an exaggerated vascular response to cold or stress that is manifested by changes in skin colour. It can be primary (PR) or secondary (SR). Objectives: The objective of this systematic review of the literature (SLR) is to describe and analyse the main differences for the detection of vascular changes in RP between capillaroscopy (NC) and infrared thermography (IRT). Methods: An SLR following PRISMA guidelines in the following information sources: Medline, Cochrane, Pubmed, ClinicalKey and ScienceDirect. Inclusion criteria: observational or analytical articles published until September 2020, that included a population with RP (primary or secondary), with concomitant diagnostic evaluation using NC (microscopic or videocapillaroscopy) and IRT. In the construction of the search equations, MeSH terms («Thermography¼, «Microscopic Angioscopy¼) and different keywords crossed with different Boolean operators were used. The Joanna Briggs Institute Critical Appraisal tools for use in Systematic Reviews Checklist was used to assess the risk of bias. The SWiM guideline was followed to synthesize and present the results. Results: 1,397 articles were identified, of which, after screening and eligibility, five were included. They included different populations, evaluated with different equipment; two articles in children and three in adults. Predominantly Caucasian and female population, total of 403 individuals (79 minors). The two studies carried out in the paediatric population showed non-concordant results and the studies in the adult population showed similarities in their results (NC better discriminates PR from SR), but with different connective tissue pathologies associated with SR. Conclusiones: Microvascular findings from two diagnostic tools (NC and IRT) are presented through the SLR when used concomitantly in RP. Through the 5 articles, it is not possible to conclude that there are clear differences or advantages / disadvantages in the preferential use of one of the two diagnostic techniques in RP, highlighting the value of NC for differen tiating between PR and SR. Further studies are required to analyse differences between the two techniques.

Microparticles: potential new contributors to the pathogenesis of systemic sclerosis?

BACKGROUND: Microparticles (MPs) are membrane-derived vesicles released from cells undergoing activation or apoptosis with diverse proinflammatory and prothrombotic activities, that have been implicated in the pathogenesis of systemic sclerosis (SSc). We aimed to evaluate the plasma levels of platelet-derived microparticles (PMPs), endothelial cell-derived microparticles (EMPs), and monocyte-derived microparticles (MMPs) in SSc patients, and the association between MPs and the clinical features of SSc. METHODS: In this cross-sectional study, 70 patients with SSc and 35 age- and sex-matched healthy controls were evaluated. Clinical and nailfold capillaroscopy (NFC) data were obtained from all patients. Plasma levels of PMPs (CD42+/31+), EMPs (CD105+), and MMPs (CD14+) were quantified by flow cytometry. RESULTS: Patients were mainly females (90%), with a mean age of 48.9 years old. PMP, EMP, and MMP levels were significantly increased in SSc patients compared to controls (79.2% ± 17.3% vs. 71.0% ± 19.8%, p = 0.033; 43.5% ± 8.7% vs. 37.8% ± 10.4%, p = 0.004; and 3.5% ± 1.3% vs. 1.1% ± 0.5%, p < 0.0001, respectively). PMP levels were significantly higher in patients with positive anti-topoisomerase-I antibodies (p = 0.030) and in patients with a disease duration > 3 years (p = 0.038). EMP levels were lower in patients with a higher modified Rodnan skin score (p = 0.015), and in those with an avascular score > 1.5 in NFC (p = 0.042). CONCLUSION: The increased levels of PMPs, EMPs and MMPs in scleroderma patients might indicate a possible role for these agents in the pathogenesis of this challenging disease.

Training the Next Generation of Rheumatologists: What Is the Best Way to Teach Fellows About Scleroderma?

Rheumatology is rich in educational opportunities, learning about a variety of diseases. Rheumatology subspecialty training is a time of unparalleled learning, and within the curriculum of a training program, the connective tissue diseases (CTDs) represent a unique challenge to the fellows. The challenge therein lies in the multisystem presentations they are faced with mastering. Scleroderma, as a rare and life-threatening CTD, remains one of the most difficult conditions to manage and treat. In this article, the authors focus on an approach to training the next generation of rheumatologists to take care of patients with scleroderma.

Peripheral Microvascular Abnormalities Associated with Open-Angle Glaucoma.

PURPOSE: To investigate peripheral microvascular abnormalities associated with patients with open-angle glaucoma (OAG). DESIGN: This was a cross-sectional study. PARTICIPANTS: Patients with OAG and controls. METHODS: All subjects underwent detailed ophthalmic evaluation, including Humphrey visual field (HVF) tests and swept source OCT. To evaluate peripheral microvascular abnormalities, nailfold capillaroscopy (NFC) and laser Doppler imaging (LDI) were performed. The presence of microhemorrhages, tortuous capillaries, dilated capillaries, avascular areas, and the capillary density, among other characteristics, were recorded using NFC; fingertip blood flow (FBF) was measured using LDI at different time points, before and 1, 10, and 20 minutes after exposure to a cold stimulus. In addition, venous blood samples were collected to measure serum endothelin-1 (ET-1) concentrations as well as serum autoantibodies. MAIN OUTCOME MEASURES: Presence of microhemorrhages, tortuous capillaries, and dilated capillaries; FBF; ET-1; and autoantibodies. RESULTS: Sixty-eight subjects (43 patients with OAG and 25 controls) were enrolled in the study. Microhemorrhages were found in the nail bed of 65.1% of the patients with OAG compared with 25.0% of the controls (P = 0.003). There was a significant difference in the mean FBF at the baseline in patients with OAG versus controls (293.6 ± 100.2 vs 388.8 ± 52.0 perfusion units, respectively, P < 0.001), together with a significant decrease in the mean FBF 10 and 20 minutes after cold stimulus in patients with OAG in comparison to controls (P < 0.001 for all comparisons). There was a positive correlation between mean baseline FBF and HVF mean deviation (r = 0.27, P = 0.03) and between mean baseline FBF and average retinal nerve fiber layer thickness (r = 0.44, P = 0.001). Neither the analysis of ET-1 concentrations (P= 0.71) nor the autoantibodies measurements (P > 0.05, for all) showed any difference between the 2 groups. CONCLUSIONS: Significant peripheral microvascular abnormalities were found in patients with OAG compared to controls, suggesting that microvascular changes might play a role in the pathogenesis of the disease. In addition, part of these peripheral microvascular abnormalities seems to be correlated with both functional and structural glaucomatous damage. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Microparticles: potential new contributors to the pathogenesis of systemic sclerosis?

Abstract Background Microparticles (MPs) are membrane-derived vesicles released from cells undergoing activation or apoptosis with diverse proinflammatory and prothrombotic activities, that have been implicated in the pathogenesis of systemic sclerosis (SSc). We aimed to evaluate the plasma levels of platelet-derived microparticles (PMPs), endothelial cell-derived microparticles (EMPs), and monocyte-derived microparticles (MMPs) in SSc patients, and the association between MPs and the clinical features of SSc. Methods In this cross-sectional study, 70 patients with SSc and 35 age- and sex-matched healthy controls were evaluated. Clinical and nailfold capillaroscopy (NFC) data were obtained from all patients. Plasma levels of PMPs (CD42+/31+), EMPs (CD105+), and MMPs (CD14+) were quantified by flow cytometry. Results Patients were mainly females (90%), with a mean age of 48.9 years old. PMP, EMP, and MMP levels were significantly increased in SSc patients compared to controls (79.2% ± 17.3% vs. 71.0% ± 19.8%, p = 0.033; 43.5% ± 8.7% vs. 37.8% ± 10.4%, p = 0.004; and 3.5% ± 1.3% vs. 1.1% ± 0.5%, p < 0.0001, respectively). PMP levels were significantly higher in patients with positive anti-topoisomerase-I antibodies (p = 0.030) and in patients with a disease duration > 3 years (p = 0.038). EMP levels were lower in patients with a higher modified Rodnan skin score (p = 0.015), and in those with an avascular score > 1.5 in NFC (p = 0.042). Conclusion The increased levels of PMPs, EMPs and MMPs in scleroderma patients might indicate a possible role for these agents in the pathogenesis of this challenging disease.

Animal Models of Systemic Sclerosis: Using Nailfold Capillaroscopy as a Potential Tool to Evaluate Microcirculation and Microangiopathy: A Narrative Review.

Systemic sclerosis (SSc) is an autoimmune disease with three pathogenic hallmarks, i.e., inflammation, vasculopathy, and fibrosis. A wide plethora of animal models have been developed to address the complex pathophysiology and for the development of possible anti-fibrotic treatments. However, no current model comprises all three pathological mechanisms of the disease. To highlight the lack of a complete model, a review of some of the most widely used animal models for SSc was performed. In addition, to date, no model has accomplished the recreation of primary or secondary Raynaud's phenomenon, a key feature in SSc. In humans, nailfold capillaroscopy (NFC) has been used to evaluate secondary Raynaud's phenomenon and microvasculature changes in SSc. Being a non-invasive technique, it is widely used both in clinical studies and as a tool for clinical evaluation. Because of this, its potential use in animal models has been neglected. We evaluated NFC in guinea pigs to investigate the possibility of applying this technique to study microcirculation in the nailfold of animal models and in the future, development of an animal model for Raynaud's phenomenon. The applications are not only to elucidate the pathophysiological mechanisms of vasculopathy but can also be used in the development of novel treatment options.

Absence of association between nailfold capillary findings and mild cognitive dysfunction in systemic lupus erythematosus.

OBJECTIVE: The role of vascular damage in cognitive dysfunction (CD) in SLE is not entirely understood. Nailfold capillaroscopy (NFC) is a noninvasive method that may aid the description of further vascular contributions to CD in SLE. Therefore, the aim of our study was to examine and compare finger nailfold capillary morphology in subjects with SLE with and without CD. METHODS: We conducted a cross-sectional study in patients with SLE. Demographic, clinical, and laboratory characteristics were collected. We evaluated nailfold capillary findings including avascular zones, hemorrhage, dilated and tortuous capillaries, disarrangement, crossing, subpapillary venular plexus, branched loops, and shortened loops by NFC. The Montreal Cognitive Assessment (MoCA) scale was used to screen cognitive function. CD was defined as a score < 26/30. RESULTS: Sixty-five females (97.0%) and 2 males (3%) with SLE were analyzed. Means of age and disease duration were 44.3 ± 12.0 years and 15.5 ± 7.6 years, respectively. Thirty-five (54.7%) patients had CD. The rate of patients with ≥ 1 NFC abnormality was 50% in both patients with and without CD (P = 0.14). Eight (22.8%) patients with CD compared to 1 without (3.5%) displayed dilated capillaries (P = 0.036). Other NFC abnormalities differed between patients with and without CD, but the possible relationships between dilated capillaries and CD disappeared after adjusting by age, diabetes, and hypertension. CONCLUSIONS: NFC findings were not associated with mild CD in patients with SLE. Our exploratory data do not support systemic microvasculopathy measured by NFC related to CD in patients with SLE.

Predictors of progression to systemic sclerosis: analysis of very early diagnosis of systemic sclerosis in a large single-centre cohort.

OBJECTIVE: This study analysed the very early disease of SSc (VEDOSS) characteristics in a group of 217 patients with RP and at least one manifestation of SSc in search of predictors for the progression to SSc. METHODS: This was a cross-sectional single-centre analysis of patients presenting with RP with a specific SSc clinical manifestation or SSc autoantibody or SD pattern at nailfold capillaroscopy (SD-NFC), without skin involvement, who attended a scleroderma outpatient clinic between 2010 and 2019. The performance of VEDOSS and the importance of the combination of VEDOSS characteristics to predict the progression to SSc were evaluated. RESULTS: Among 217 patients, 153 (70.5%) were classified as SSc, including 65 (30%) in the first investigation; 69.3% of the SSc patients met VEDOSS criteria compared with 6.3% of patients who did not progress to SSc. The combinations most associated with progression to SSc were RP + puffy fingers (PF) + positive ANA + SD-NFC and/or SSc-specific antibody (VEDOSS level 2), with an odds ratio (OR) of 19.52 (95% CI 4.48, 85.06; P < 0.001) and RP + PF + positive ANA (VEDOSS level 1; 'red flags') (OR 15.45; P < 0.001), while combinations without non-RP clinical symptoms, as RP + SD-NFC (OR 0.03; P < 0.001) and RP + anticentromere + SD-NFC (OR 0.06; P = 0.006) were associated with non-progression to SSc. CONCLUSION: Among patients with RP with at least one manifestation of SSc, without skin involvement, combinations of VEDOSS characteristics were the strongest predictors of progression to SSc at a median follow-up of 4 years.

Systemic Sclerosis: Elevated Levels of Leukotrienes in Saliva and Plasma Are Associated with Vascular Manifestations and Nailfold Capillaroscopic Abnormalities.

The role of leukotrienes (LTs) in the pathogenesis of systemic sclerosis (SSc) needs clarification. We analyzed the association of salivary (sa) and plasma (p) levels (pg/mL) of cysteinyl-leukotrienes (CysLT) and LTB4 with SSc vascular manifestations and nailfold capillaroscopy (NFC) in a cross-sectional study. Patients and healthy controls were evaluated for vascular manifestations and NFC. LTs were compared between groups as follows: SSc with or SSc without vascular features and controls, and by NFC parameters. Twenty SSc patients and 16 volunteers were recruited; Raynaud's phenomenon (RP) history (SSc: saCysLT 99.4 ± 21.8 vs. controls: 23.05 ± 23.7, p = 0.01), RP at examination (SSc: saCysLT 129.3 ± 24.6 vs. controls: 23.05 ± 22.46, p = 0.01; pCysLT SSc: 87.5 ± 11.2 vs. controls: 32.37 ± 10.75, p = 0.002), capillary loss (saCysLT 138.6 ± 26.7 vs. 23.05 ± 21.6, p = 0.0007; saLTB4 3380.9 ± 426.6 vs. 1216.33 ± 346.1, p = 0.0005), "late" scleroderma pattern vs. controls (saCysLT 205.6 ± 32 vs. 23 ± 19.6, p = 0.0002; saLTB4 4564.9 ± 503.6 vs. 1216.3 ± 308.3; p < 0.0001) were all significant. Late patterns had higher levels (saCysLT, p = 0.002; LTB4 p = 0.0006) compared to active and early patterns (LTB4, p = 0.0006), and giant capillaries (p = 0.01) showed higher levels of LTs. Levels of pCysLT were higher in patients with RP at examination vs. patients without RP; saCysLT and LTB4 were higher in SSc group with vs. without capillary loss. LTs could be involved in the pathophysiology of vascular abnormalities. Further research is required to determine if blocking LTs could be a therapeutic target for SSc vascular manifestations.

Prognostic value of capillaroscopy in organ involvement and identification of subtypes in systemic sclerosis (SS): A systematic literature review / Valor pronóstico de la capilaroscopia en el compromiso de órganos e identificación de subtipos en la esclerosis sistémica (ES): una revisión sistemática de la literatura

ABSTRACT Background: Capillaroscopy is an essential tool for the diagnosis of systemic sclerosis. Using this exam as a prognostic factor will allow earlier intervention and probably, delay on disease progression. We aimed to evaluate the prognostic value of capillaroscopy for the prediction of systemic compromise and subtype differentiation in systemic sclerosis. Methods: A systematic literature search was applied in the following electronic databases: Medline, PubMed, Embase, Cochrane, and Lilacs. The research question was designed based on the PICOT model, and the search strategy was built using the MeSH terms "Microscopic Angioscopy," "Scleroderma systemic," "Scleroderma diffuse," Scleroderma Limited," "Early Diagnosis" and Boolean operators. The language was restricted to papers published in Spanish or English, from 1990 to 2019. The search terms were explored for each database, and new terms were added, as appropriate. The searches were made again before the final analyses and further studies were retrieved for inclusion at that time. Reference lists of included studies and recent aligned systematic reviews were also screened. Gray literature was not considered in this review. Results: A total of 183 articles were found in the selected databases: Medline (n: 115), Embase (n: 66), Cochrane (n: 2), Lilacs (n: 0). After excluding articles due to duplication, a total of 66 studies were selected. Within these articles, a screening process was applied based on the title and abstract, taking into account the eligibility criteria, finally obtaining 21 references. Two researchers assessed the selected articles, and all disagreements were solved by consensus. Finally, a total of 14 articles were included. Conclusions: The different abnormalities found in capillaroscopy, especially loss of capillaries, have been consistently associated not only with organ involvement but also with severity of the disease, especially with vascular manifestations (digital ulcers and pulmonary hypertension). The importance of capillaroscopy is not only its diagnostic value but also its predictive value with its consequent implications in the follow-up and management of systemic sclerosis.

RESUMEN Introducción: La capilaroscopia es una herramienta esencial para el diagnóstico de la esclerosis sistémica. Usar este examen como factor pronóstico permitirá realizar una intervención temprana y probablemente retardará la progresión de la enfermedad. Se realizó una revisión de la literatura evaluando el valor pronóstico de la capilaroscopia para predecir el compromiso sistémico de la esclerosis sistémica y su diferenciación por subtipos. Métodos: Se realizó una revisión sistemática de la literatura en las siguientes bases de datos: Medline, PubMed, Embase, Cochrane y Lilacs. La búsqueda se hizo basada en el modelo PICOT y la estrategia de búsqueda fue construida mediante los términos MeSH «Microscopic angioscopy¼, «Scleroderma systemic¼, «Scleroderma diffuse¼, «Scleroderma limited¼, «Early diagnosis¼ y operadores booleanos. El lenguaje fue restringido a artículos publicados en español e inglés desde 1990 hasta 2019. Se realizó la búsqueda en cada base de datos y se adicionaron nuevos términos según fuera apropiado. La búsqueda se realizó de nuevo al final del análisis y se incluyeron los estudios más recientes. La lista de referencias de los estudios incluidos y las revisiones sistemáticas recientemente adicionadas también fueron registradas. No se consideró literatura gris en esta revisión. Resultados: Un total de 183 artículos fueron encontrados en las siguientes bases de datos: Medline (n = 115), Embase (n = 66), Cochrane (n = 2), Lilacs (n = 0). Después de excluir los que estaban duplicados, un total de 66 estudios fueron seleccionados. Dentro de estos artículos, se realizó un proceso de selección basado en título y resumen tomando en cuenta los criterios de elegibilidad, obteniendo finalmente 21 referencias. Dos investigadores revisaron los artículos seleccionados y todas las discrepancias fueron resueltas en consenso. Finalmente, un total de 14 artículos fueron incluidos. Conclusiones: Las diferentes anormalidades encontradas en la capilaroscopia, especialmente la pérdida de capilares, han sido constantemente asociadas no solo con compromiso de órganos sino también a la severidad de la enfermedad, especialmente con manifestaciones vasculares (úlceras digitales e hipertensión pulmonar). La importancia de la capilaroscopia no solo es por su valor diagnóstico sino también por su valor predictivo en relación al seguimiento y manejo de la esclerosis sistémica.

Pan-American League of Associations for Rheumatology (PANLAR) capillaroscopy study group consensus for the format and content of the report in capillaroscopy in rheumatology.

OBJECTIVE: The aim of this work was to produce a consensus-based report for capillaroscopy in rheumatology to be used in daily clinical practice. METHODS: A written Delphi questionnaire regarding capillaroscopy report was developed from a literature review and expert consensus. The Delphi questionnaire was sent to an international panel including 25 rheumatologists experts in capillaroscopy, asking them to rate their level of agreement or disagreement with each statement. The exercise consisted of three online rounds and a face-to-face (live meeting) that took place in the PANLAR 2018 congress held in Buenos Aires, Argentina. RESULTS: The participants to the first, second, third, and face-to-face round were 22, 21, 21, and 16 rheumatologists, respectively. Fifty-five items were discussed in the first round, 58 in the second, 22 in the third, and 9 in the face-to-face meeting. At the end of the exercise, 46 recommendations for the capillaroscopy report in rheumatology reached a consensus. CONCLUSION: This is the first consensus-based report in capillaroscopy. It will be useful in daily clinical practice and to address the effort of the standardization in the technique. KEY POINTS: • The current lack of consensus for the capillaroscopy report makes difficult the interpretation of findings as well as follow-up of rheumatic diseases. • This study produced the first international consensus for the format and content of the naifold capillaroscopy report in rheumatology. • The report is an integral part of the capillaroscopy examination and its use in a homogeneous form can help in the correct interpretation of findings in daily practice.

Capillaroscopy changes are associated with disease progression in patients with early systemic sclerosis: A prospective study.

AIM: After the development of the 2013 American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) classification criteria for systemic sclerosis (SSc), there are still a group of patients affected by early SSc who do not meet the new criteria. This study aimed to evaluate capillaroscopy changes and to identify predictors of progression to definite SSc in patients with early SSc over a 3-year follow-up. METHODS: In this prospective study, 44 patients with early SSc (LeRoy and Medsger 2001 criteria) were included. Clinical evaluation and widefield nailfold capillaroscopy were performed at baseline and after 3 years of follow-up. At the end of follow-up, the fulfilment of the 2013 ACR/EULAR criteria was also assessed. RESULTS: After 3 years, 34 patients with early SSc were re-evaluated. Of these, eight patients (23.5%) developed definite SSc. Worsening of capillaroscopy parameters was observed in 55.9% of patients. An increase in the number of giant capillaries and worsening of the avascular score were more frequent in patients who developed SSc than in those who did not (P = 0.02; P = 0.002, respectively). By multivariate analysis, an active or a late pattern at baseline on capillaroscopy was an independent predictor for the development of definite SSc (odds ratio = 30.0, 95% CI 2.1-421.1). CONCLUSIONS: In this prospective study, worsening in capillaroscopy parameters was observed in early SSc patients. An active or a late pattern on capillaroscopy was an independent predictive risk factor for the development of SSc, suggesting that capillaroscopy might be a useful tool to identify patients with early SSc at risk of disease progression.

Nailfold capillary changes in adult new-onset dermatomyositis: a prospective cross-sectional study.

OBJECTIVES: It is to prospectively analyze nailfold capillaroscopy (NC) findings in new-onset dermatomyositis (DM) and to correlate NC findings with serum angiogenic cytokines and DM clinical and laboratory features. MATERIALS AND METHODS: Twenty-three patients with DM who experienced < 12 months of symptoms were included in the study. To assess serum cytokine levels, 23 age-, sex-, and ethnicity-matched healthy volunteers were used. NC characteristics and DM activity parameters were analyzed. RESULTS: Significantly higher serum angiogenin (ANG) and vascular endothelial growth factor-1 (VEGF1) levels were observed in DM patients than in controls. Capillary density and avascular areas correlated positively and negatively, respectively, with serum levels of ANG. Moreover, the capillary density correlated inversely with the number of enlarged and giant capillaries and avascular areas. The number of enlarged capillaries correlated positively with patient and physician visual analogue scales (VAS), the presence of a facial rash, giant capillaries, and microhemorrhages. Giant capillaries had a positive correlation with physician and cutaneous VAS, enlarged capillaries, avascular areas, microhemorrhages and bushy capillaries, and a negative correlation with capillary density. Microhemorrhages correlated positively with the "V-neck" sign and physician VAS. VEGF1 showed no relationship with the NC parameters with DM-related clinical and laboratory features. Additionally, 15 out of 23 patients were assessed prospectively after 3.21 years. All patients had a major clinical response with significant improvement in all NC parameters, except for enlarged and bushy capillaries. CONCLUSIONS: The NC may be a useful tool to assess disease activity in recent-onset DM, and it can also reinforce the role of ANG in the angiogenesis of this myopathy.

Risk of digital ulcers occurrence in systemic sclerosis: a cross-sectional study.

BACKGROUND/OBJECTIVE: Digital ulcers (DUs) represent a frequent complication of systemic sclerosis (SSc). The aim of this study was to evaluate clinical, serological and capillaroscopy features that are associated with DUs in patients with SSc. METHODS: In this bicentric cross-sectional study, 70 patients with SSc were consecutively selected from March 2016 to April 2017. Demographic and clinical features, including the presence of active DUs, were collected. Videocapillaroscopy was performed in all patients. RESULTS: Among the 70 patients included (mean age of 46.8 years, mean disease duration of 9.41 years), 14 (20%) had active DUs. Based on multivariate analysis, the presence of anti-Scl-70 antibodies, the HAQ-DI score, and the capillary loss score were independently associated with DUs with odds ratios of 7.96 (95% CI 1.32-47.99), 55.77 (95% CI 1.76-1764.28), and 16.66 (95% CI 2.07-133.81), respectively. CONCLUSIONS: The presence of avascular areas in capillaroscopy, elevation of HAQ-DI score and anti-Scl-70 antibodies were independent factors associated with DUs in patients with SSc.

Hallazgos capilaroscópicos en niños sanos / Capillary findings in healthy children

RESUMEN En el niño, la microcirculación cutánea (MCC) experimenta cambios desde sus primeros días de vida hasta la adolescencia. Mediante la video capilaroscopia (VC), se puede acceder a la visualización in vivo e incruenta de la MCC, en niños de cualquier edad. El sitio de elección es el pliegue peri ungular de los dedos de las manos. Se observan las asas capilares, dispuestas en corona sobre la matriz peri ungular y el plexo vascular superficial. Los recién nacidos presentan una red primitiva. Posteriormente, evolucionaalcanzando alrededor de los 10 años, los parámetros similares al adulto.El conocimiento de los parámetros de la MCC preservada en niños,será de gran importancia en la exploración diagnóstica de las patologías que afectan este territorio vascular, como las enfermedades reumáticas.

SUMMARY The child's skin microcirculation (MCC) undergoes changes from the first days of life until adolescence. Through video capillaroscopy (VC), in vivo and non-invasive visualization of the CCM is accessed in children of any age. The site of the election is the nail fold of the fingers of the hands. The capillary loops arranged in a crown over the nail matrix and the superficial vascular plexus are observed. The newborns present a primitive network, which later evolves reaching around 10 years the parameters similar to the adult. There are structural differences in MCC if the findings in children are compared with those of adults. In children the capillary density per linear millimeter is lower, the tortuosity index is higher, the superficial vascular plexus is more visible and bizarre capillary forms are found Knowledge of the parameters of MCC preserved in children will be of great importance in the diagnostic exploration of pathologies that affect this vascular territory such as rheumatic diseases.

Nailfold capillaroscopy as a risk factor for pulmonary arterial hypertension in systemic lupus erythematosus patients.

BACKGROUND: Pulmonary arterial hypertension (PAH) is a rare and severe complication of systemic lupus erythematosus (SLE). This study aimed to evaluate clinical and laboratory risk factors associated with PAH in SLE patients. METHODS: This was a retrospective case-control study in which patients with SLE with PAH (SLE-PAH) confirmed by right heart catheterization (RHC) were compared with SLE patients without PAH. Clinical and demographic variables related to SLE and PAH and nailfold capillaroscopy were evaluated by reviewing the medical records of the patients. RESULTS: Twenty-one patients with SLE-PAH and 44 patients with SLE without PAH matched for sex and disease duration were included. The scleroderma (SD) pattern on nailfold capillaroscopy was more frequently found in patients with SLE-PAH than in those without PAH (56.3% versus 15.9%, respectively, p = 0.002). By univariate analysis, Raynaud's phenomenon, history of abortion, and SD pattern on capillaroscopy were associated with PAH. Arthritis was a protective factor for PAH development. Multivariate analysis showed that the SD pattern on capillaroscopy was the only variable associated with a significantly higher risk of PAH, with an odds ratio of 6.393 (95% confidence interval, 1.530-26.716; p = 0.011). CONCLUSION: In this study, SD pattern was associated with a 6.3-fold increased risk for PAH development in SLE patients, suggesting that nailfold capillaroscopy might be useful as a screening method to identify SLE patients with a high risk of developing this severe complication.

The current role of capillaroscopy in vasculitides.

Nailfold capillaroscopy (NFC) has gained remarkable interest among rheumatologists because of its utility in both clinical practice and research activity. Nevertheless, there has been scarce attention on its potential in other rheumatic disorders such as vasculitis. We perform a systematic review of literature on NFC in noninfectious vasculitides, with the aim to provide an overview of the main NFC changes described, to discuss the current evidence supporting its clinical impact and applications in daily practice and to provide future research fields.

Position article and guidelines 2018 recommendations of the Brazilian Society of Rheumatology for the indication, interpretation and performance of nailfold capillaroscopy.

Nailfold capillaroscopy (NFC) is a reproducible, simple, low-cost, and safe imaging technique used for morphological analysis of nail bed capillaries. It is considered to be extremely useful for the investigation of Raynaud's phenomenon and for the early diagnosis of systemic sclerosis (SSc). The capillaroscopic pattern typically associated with SSc, scleroderma ("SD") pattern, is characterized by dilated capillaries, microhemorrhages, avascular areas and/or capillary loss, and distortion of the capillary architecture. The aim of these recommendations is to provide orientation regarding the relevance of NFC, and to establish a consensus on the indications, nomenclature, the interpretation of NFC findings and the technical equipments that should be used. These recommendations were formulated based on a systematic literature review of studies included in the database MEDLINE (PubMed) without any time restriction.

Position article and guidelines 2018 recommendations of the Brazilian Society of Rheumatology for the indication, interpretation and performance of nailfold capillaroscopy

Abstract Nailfold capillaroscopy (NFC) is a reproducible, simple, low-cost, and safe imaging technique used for morphological analysis of nail bed capillaries. It is considered to be extremely useful for the investigation of Raynaud's phenomenon and for the early diagnosis of systemic sclerosis (SSc). The capillaroscopic pattern typically associated with SSc, scleroderma ("SD") pattern, is characterized by dilated capillaries, microhemorrhages, avascular areas and/or capillary loss, and distortion of the capillary architecture. The aim of these recommendations is to provide orientation regarding the relevance of NFC, and to establish a consensus on the indications, nomenclature, the interpretation of NFC findings and the technical equipments that should be used. These recommendations were formulated based on a systematic literature review of studies included in the database MEDLINE (PubMed) without any time restriction.