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Objectives: To standardly assess and describe nailfold videocapillaroscopy (NVC) assessment in children and adolescents with juvenile rheumatic and musculoskeletal diseases (jRMD) vs healthy controls (HCs). Material and methods: In consecutive jRMD children and matched HCs from 13 centres worldwide, 16 NVC images per patient were acquired locally and read centrally per international consensus standard evaluation of the EULAR Study Group on Microcirculation in Rheumatic Diseases. A total of 95 patients with JIA, 22 with JDM, 20 with childhood-onset SLE (cSLE), 13 with juvenile SSc (jSSc), 21 with localized scleroderma (lSc), 18 with MCTD and 20 with primary RP (PRP) were included. NVC differences between juvenile subgroups and HCs were calculated through multivariable regression analysis. Results: A total of 6474 images were assessed from 413 subjects (mean age 12.1 years, 70.9% female). The quantitative NVC characteristics were significantly lower or higher in the following subgroups compared with HCs: for density: lower in jSSc, JDM, MCTD, cSLE and lSc; for dilations: higher in jSSc, MCTD and JDM; for abnormal shapes: higher in JDM and MCTD; for haemorrhages: higher in jSSc, MCTD, JDM and cSLE. The qualitative NVC assessment of JIA, lSc and PRP did not differ from HCs, whereas the cSLE and jSSc, MCTD, JDM and cSLE subgroups showed more non-specific and scleroderma patterns, respectively. Conclusions: This analysis resulted from a pioneering registry of NVC in jRMD. The NVC assessment in jRMD differed significantly from HCs. Future prospective follow-up will further elucidate the role of NVC in jRMD. VC The Author(s) 2022.

Aim: To identify the frequency of scleroderma-type capillaroscopic patterns and evaluate the association of capillaroscopic patterns with clinical parameters, functional status, fatigue, and pain in systemic sclerosis (SSc). Material and Method: This cross-sectional study included SSc patients consecutively between January 2017 and January 2019. Cutaneous involvement was evaluated with the modified Rodnan skin score (mRSS). The presence of digital ulcers, Raynaud phenomenon, interstitial lung disease, pulmonary hypertension, cardiac, gastrointestinal system (GIS), renal, joint and muscle involvement were recorded. The severity of the Raynaud phenomenon, fatigue, pain, and patient global assessment (PGA) was assessed on the Visual Analogue Scale (VAS). The Health Assessment Questionnaire (HAQ) and the Duruoz Hand Index (DHI) were used to assess physical disability and hand function, respectively. Nailfold videocapillaroscopic examinations of the patients were performed, and they were classified into four groups, including normal/non-specific, early, active, and late scleroderma patterns. Results: The mean age of 32 patients with SSc (31 female, one male) was 48.93±12.77. Anormal capillaroscopic examination findings were detected in 93.7% of the patients, and the most common capillaroscopic pattern was the active pattern. The comparison of scleroderma pattern groups revealed no difference in age (p=0.224), but disease duration was shorter in the early pattern group (p=0.005). The duration and severity of the Raynaud phenomenon, and mean mRSS were lower in the early pattern group (p=0.004, p=0.009, and p=0.001, respectively). The digital ulcer (p=0.011) and diffuse cutaneous SSc (p=0.016) were more common in the late pattern group. The percentage of pulmonary hypertension (p=0.011), GIS involvement (p

Videocapillaroscopy is a simple, non-invasive investigation that allows the “in vivo” study of the nailfold capillaries. This method is inexpensive, easily accepted by patients and the results can be easily interpreted. It is mainly used in patients with Raynaud’s phenomenon and systemic sclerosis, but this examination can also be performed on patients who are suspected of having microcirculation alterations, such as rheumatoid arthritis and psoriatic arthritis. It may aid in the diagnosis, evaluation and prognosis of other rheumatic diseases, besides systemic sclerosis. The aim of this study is to identify the nailfold videocapillaroscopic abnormalities in rheumatoid arthritis and psoriatic arthritis patients and analyze the correlation between their evolution and 12 months of anti-TNF-α therapy. The abnormal capillaroscopic findings comprised widened, dilated or giant capillaries and the distortion of the normal nailfold architecture, avascular areas, hemorrhages and neoangiogenesis. Overall, capillary density, dilated capillaries, giant capillaries, elongated capillaries and angiogenesis significantly improved after 12 months. Moreover, no avascular areas were found after 12 months of anti-TNF treatment.

Microcirculation in a subject can be examined and pathological changes can be assessed by utilizing capillaroscopy, which is a very safe, convenient and non-invasive approach. Using a microscope, doctors view the capillaries by looking through nailfold epidermis. Nailfold anatomy is ideal to evaluate the microcirculation and detect various diseases caused by vascular damages. Rheumatologists evaluate systemic diseases which involve damage in vasculature, by analyzing the red blood cells within the capillaries. Sometimes, capillary morphology may be useful as an early indicator while, severity of damage in capillary architecture may indicate internal organ involvement. Thus, in a capillaroscopic assessment, the doctor examines modifications in morphological and functional aspects of capillaries. These comprise of capillary diameter, visibility, distribution, length, micro-hemorrhages, blood flow and density. In this paper, a novel object detection algorithm is proposed based on deep learning architectures for detecting and locating various capillary loops in the nailfold region. Various characteristic features are extracted from the capillaries through image processing algorithms and in turn an attempt is made to differentiate between images of diseased subjects and healthy controls.

Objectives: To standardly assess and describe nailfold videocapillaroscopy (NVC) assessment in children and adolescents with juvenile rheumatic and musculoskeletal diseases (jRMD) vs healthy controls (HCs). Material and methods: In consecutive jRMD children and matched HCs from 13 centres worldwide, 16 NVC images per patient were acquired locally and read centrally per international consensus standard evaluation of the EULAR Study Group on Microcirculation in Rheumatic Diseases. A total of 95 patients with JIA, 22 with JDM, 20 with childhood-onset SLE (cSLE), 13 with juvenile SSc (jSSc), 21 with localized scleroderma (lSc), 18 with MCTD and 20 with primary RP (PRP) were included. NVC differences between juvenile subgroups and HCs were calculated through multivariable regression analysis. Results: A total of 6474 images were assessed from 413 subjects (mean age 12.1 years, 70.9% female). The quantitative NVC characteristics were significantly lower or higher in the following subgroups compared with HCs: for density: lower in jSSc, JDM, MCTD, cSLE and lSc; for dilations: higher in jSSc, MCTD and JDM; for abnormal shapes: higher in JDM and MCTD; for haemorrhages: higher in jSSc, MCTD, JDM and cSLE. The qualitative NVC assessment of JIA, lSc and PRP did not differ from HCs, whereas the cSLE and jSSc, MCTD, JDM and cSLE subgroups showed more non-specific and scleroderma patterns, respectively. Conclusions: This analysis resulted from a pioneering registry of NVC in jRMD. The NVC assessment in jRMD differed significantly from HCs. Future prospective follow-up will further elucidate the role of NVC in jRMD. VC The Author(s) 2022.

Up to 30 % of patients with psoriasis (PsO) develop psoriatic arthritis (PsA), and diagnosis can be difficult. Nailfold capillaroscopy (NC) is an easily applicable, non-invasive procedure to assess skin microcirculation. This systematic review investigates NC as diagnostic tool for PsO and PsA, including correlations between NC outcome measures to clinical and laboratory outcome measures. This systematic review was built on the PICO and PRISMA guidelines. In total 22 relevant studies were found Searching in the Web of Science, PubMed and Embase, latest update June 13th, 2022. The following NC outcome measures are found to be significantly more prevalent in PsO patients than healthy controls: reduced density, reduced length and more abnormal morphology. Likewise, in PsA patients, reduced density, more abnormal morphology, more microhaemorrhages and fewer hairpin shapes are found to be significantly more prevalent. Results were non-conclusive in terms of disease activity and duration with NC findings. Random-effects meta-analysis showed a significant reduction of density in PsO patients compared to healthy controls (studies: 6, n = 249; SMD = -0.91; 95 % CI [−1.41, −0.40], p = 0.0058, heterogeneity I2=74 %, AUC = 0.740) and in PsA patients compared to healthy controls (studies: 5, n = 130; SMD = -1.22; 95 % CI [−2.38, −0.06], p = 0.0432, heterogeneity I2=89 %, AUC = 0.806). No NC outcome measures were overall conclusive in differentiating PsO from PsA. Considering the conflicting results and small sample sizes further large-scale research on the identification of capillaroscopic changes in PsO and PsA and correlations with standardised clinical and laboratory outcome measures are necessary.

Objectives: To standardly assess and describe nailfold videocapillaroscopy (NVC) assessment in children and adolescents with juvenile rheumatic and musculoskeletal diseases (jRMD) vs healthy controls (HCs). Material and methods: In consecutive jRMD children and matched HCs from 13 centres worldwide, 16 NVC images per patient were acquired locally and read centrally per international consensus standard evaluation of the EULAR Study Group on Microcirculation in Rheumatic Diseases. A total of 95 patients with JIA, 22 with JDM, 20 with childhood-onset SLE (cSLE), 13 with juvenile SSc (jSSc), 21 with localized scleroderma (lSc), 18 with MCTD and 20 with primary RP (PRP) were included. NVC differences between juvenile subgroups and HCs were calculated through multivariable regression analysis. Results: A total of 6474 images were assessed from 413 subjects (mean age 12.1 years, 70.9% female). The quantitative NVC characteristics were significantly lower or higher in the following subgroups compared with HCs: for density: lower in jSSc, JDM, MCTD, cSLE and lSc; for dilations: higher in jSSc, MCTD and JDM; for abnormal shapes: higher in JDM and MCTD; for haemorrhages: higher in jSSc, MCTD, JDM and cSLE. The qualitative NVC assessment of JIA, lSc and PRP did not differ from HCs, whereas the cSLE and jSSc, MCTD, JDM and cSLE subgroups showed more non-specific and scleroderma patterns, respectively. Conclusions: This analysis resulted from a pioneering registry of NVC in jRMD. The NVC assessment in jRMD differed significantly from HCs. Future prospective follow-up will further elucidate the role of NVC in jRMD.

Most of the recent literature regarding rotator cuff tear etiology identifies in peripheral microcirculation disorders the probable main cause of tissue degeneration, and consequently of tendon rupture. Nailfold capillaroscopy is a practical and inexpensive diagnostic technique used to evaluate the health status of peripheral microcirculation, and recently, its use has found other indications in addition to that of diagnosing connective tissue diseases and Raynaud phenomenon. We verified the possible indirect contribution of nailfold capillaroscopy in the identification of peripheral microcirculation disturbances in a group of patients with rotator cuff tear and whether these possible alterations could be related to rotator cuff tear size.A case-control study was performed. One hundred patients (56 male, 44 female; mean age ± standard deviation [SD]: 60.46 ± 5.46 years) with different-sized posterosuperior cuff tears and 100 healthy controls (38 male, 62 female; mean age ± SD: 60.40 ± 6.34 years) were submitted to capillaroscopic examination. The following parameters were examined: capillary morphology and density, avascular areas, visibility of the subpapillary venous plexus, enlarged and giant capillaries, ectasias and microaneurysms, neoangiogenesis, hemosiderin deposits, pericapillary edema, and capillary blood flow. Severe exclusion criteria were applied. Statistical analysis was performed.Visibility of subpapillary venous plexus (P .001), pericapillary edema (P .001), capillary blood flow (P .001), ectasias and microaneurysms (P .001), and neoangiogenesis (P = .04) were significantly associated with presence of a rotator cuff tear.Our results support the hypothesis that microcirculation disorder has a relevant role in the genesis of cuff degeneration and, consequently, of tendon rupture. However, these alterations do not seem to be related to rotator cuff tear size.

Background: Hand osteoarthritis (OA) can be a distressing disorder that impairs a patients' quality of life. There is a relation between OA and vascular damage, especially atherosclerosis, and OA are both common chronic conditions associated with aging and obesity. Aim of the study: The objective of this research was to examine nailfold microcirculation in individuals with hand OA Patients and Methods: a cross-sectional, observational study, about 56 patients with hand OA were enrolled. All subjects underwent a standard radiology X-Ray examination of the hands and a Nailfold vascular examination was performed. Results: of the 56 patients studied were females, and 12 were males. Regarding the Kellgren and Lawrence scoring, it was mild (grade II) in one patient (50%) and severe (grade IV) in one patient (50%) in the male patients without pain, while in those with hand pain, it was moderate (grade III) in eight patients (80%) and severe (grade IV) in two patients (20%).it was mild (grade II) in eight patients (22.2%), moderate (grade III) in 21 patients (58.3%) and severe (grade IV) in seven patients (19.4%). Nailfold examination for studied subjects showed that there were about 41 patients (73.2%) with low capillary density and 32 patients (57.1%) with morphological changes. Conclusion: Our findings provided a general description of the NFC findings most likely to be seen in OA patients; Vascular anomalies may play a role in the illness progression and may assist in elucidating because the condition is frequently found in the distal digit joints.

Funder: Wellcome Trust, SIGNIFICANCE: The capillaries are the smallest blood vessels in the body, typically imaged using video capillaroscopy to aid diagnosis of connective tissue diseases, such as systemic sclerosis. Video capillaroscopy allows visualization of morphological changes in the nailfold capillaries but does not provide any physiological information about the blood contained within the capillary network. Extracting parameters such as hemoglobin oxygenation could increase sensitivity for diagnosis and measurement of microvascular disease progression. AIM: To design, construct, and test a low-cost multispectral imaging (MSI) system using light-emitting diode (LED) illumination to assess relative hemoglobin oxygenation in the nailfold capillaries. APPROACH: An LED ring light was first designed and modeled. The ring light was fabricated using four commercially available LED colors and a custom-designed printed circuit board. The experimental system was characterized and results compared with the illumination model. A blood phantom with variable oxygenation was used to determine the feasibility of using the illumination-based MSI system for oximetry. Nailfold capillaries were then imaged in a healthy subject. RESULTS: The illumination modeling results were in close agreement with the constructed system. Imaging of the blood phantom demonstrated sensitivity to changing hemoglobin oxygenation, which was in line with the spectral modeling of reflection. The morphological properties of the volunteer capillaries were comparable to those measured in current gold standard systems. CONCLUSIONS: LED-based illumination could be used as a low-cost approach to enable MSI of the nailfold capillaries to provide insight into the oxygenation of the blood contained within the capillary network.

Nailfold capillaroscopy (NFC) is now one of the main imaging tools in systemic sclerosis and imposed over time as an easy, non-invasive method for the nailfold microvascular bed assessment. In qualitative NFC normal pattern is characterized by homogeneous, parallel fashion arrangement of the last capillaries row as well as by capillaries with hairpin or non-specific variations like tortuous and/ or crossing shape. Nailfold capillaroscopy is strongly recommended for evaluation of all patients with Raynaud phenomenon. Appearance of giant capillaries is chronologically the first relevant finding for scleroderma spectrum disorders development (systemic sclerosis, dermatomyositis, undifferentiated and mixed connective tissue disease). Collapses of the giant loops generate microhemorrhages and further capillary loss with subsequent hypoxia, and neoangiogenesis seen as ramified/ bushy capillaries. Nailfold capillaroscopy is indicated especially in systemic sclerosis, being also included in the classification criteria. Based on these major NFC pathologic findings (giant capillaries, microhemorrhages, avascularity and neoangiogenesis), three evolutive stages were described in systemic sclerosis, namely the early, active, and late scleroderma pattern. In other connective tissue diseases than those scleroderma-related, like systemic lupus erythematosus, psoriatic arthritis, or antiphospholipid syndrome, the interest for capillaroscopy is growing, but the attempts of defining specific characteristics failed until now. Besides qualitative NFC, semiquantitative and quantitative capillaroscopic assessments were proposed for more accurate evaluation. Lately, automated systems are under development. There is still need of more studies to sustain the nailfold capillaroscopy validity as diagnostic and prognostic test.

Introduction Mixed connective tissue disease (MCTD) is a rare disease with clinical picture consisted of multiple organ manifestations, including skin changes resembling systemic lupus erythematosus (SLE), systemic sclerosis (SSc), or dermatomyositis (DM). On the background of these manifestations are microvascular changes — alteration of endothelial function and impairment of endothelial progenitor cell. Nailfold capillaroscopy (NFC) is a simple, non-invasive technique for investigating microvascular involvement in rheumatic diseases. Objectives To describe the relationship between type of skin lesions and NFC pattern in MCTD patients. Methods We analyzed the clinical picture and NFC patterns in 79 patients with MCTD. The NFC changes were classified into Normal, “Early,” “Active,” and “Late” scleroderma-like patterns (SD-like pattern) based on Cutolo classification. In all patients, subjective and physical examinations were carried out, specifically the occurrence of skin lesions in the course of MCTD was assessed (systemic sclerosis-like (Ssc-like), systemic lupus erythematosus-like (SLE-like), dermatomysitis-like (DM-like)). Results Skin changes were present in 64 (81%) patients, involving 43 (54%) SLE-like, 48 (61%) SSc-like, and 4 (5.1%) DM-like. NFC changes were observed in a total of 55 (69.6 %) patients with predominance of the “Early” pattern — 41 (51.9 %) patients. According to skin change phenotypes, NFC changes were observed in 31 (72%) patients with SLE-like and in 32 (66.7%) patients with SSc-like skin phenotypes. The “early” pattern predominated in both group. Conclusions We did not find any correlation between NFC pattern and the type skin changes. Key Points• The study did not show a correlation between the presence and absence of skin lesions and NFC pattern.• Scleroderma-like patterns were found in over 60% of patients with mixed connective tissue disease.• The “early” pattern is dominant regardless of the occurrence or absence of skin lesions in patients with MCTD.• Skin lesions, regardless of their type (SLE or SSc), do not correlate with type of lesion found in the NFC examination.

Nailfold capillaroscopy is currently one of the highly informative methods for assessing the state of microcirculation. The advantages are ease of implementation, non-invasiveness, accessibility of the research object, instant visualization of the microvasculature, painlessness. The object of the study is the nailfold capillaries of the fingers or toes, due to their horizontal position in relation to the surface of the body, which makes it possible to evaluate the capillary throughout. Many authors have shown pronounced microvascular anomalies in type 1 and type 2 diabetes mellitus: tortuosity and dilation of capillaries, a decrease in their density, microhemorrhages, avascular zones, ectasias and aneurysms, newly formed capillaries; a relationship with the duration and compensation of diabetes mellitus as well as the severity of diabetic retinopathy was revealed. Thus, nailfold capillaroscopy can be used as a method for diagnosing and predicting diabetic retinopathy in patients with diabetes mellitus with the aim of early detection of initial changes in the microvasculature and timely retinopathy treatment. Key words: capillaroscopy; nailfold; diabetes mellitus.

Anti-U1RNP antibody is associated with distinct organ involvement in patients with systemic lupus erythematosus (SLE). Nailfold capillaroscopy (NFC) allows non-invasive assessment of microvascular abnormalities in several connective tissue diseases. The objective of this study is to determine the association of anti-U1RNP antibody with microvascular changes by NFC in RNP-positive SLE patients in comparison with RNP-negative SLE patients (negative disease controls) and mixed connective tissue disease (MCTD) cases (positive disease controls). NFC examination was performed in consecutive patients with SLE with or without anti-U1RNP positivity. MCTD patients were recruited as disease controls. Abnormalities noted in the three groups were compared using non-parametric tests. Ordinal logistic or linear regression was used wherever applicable. 81 patients were studied, of whom 28 were diagnosed as RNP-positive SLE (age 30.0 ± 10.37; 26 females), 26 were RNP-negative SLE (age 29.42 ± 9.20; 25 females) and 27 had MCTD (age36.5 ± 9.70; 25 females). RNP-positive SLE patients had more frequent giant capillaries, enlarged capillaries and ramified capillaries as compared to RNP-negative SLE (p = 0.05

BACKGROUND The nailfold capillaroscopy (NFC) has been used to analyse capillary microarchitecture in diseases like Raynaud’s disease, scleroderma, and other collagen disorders earlier but recently researchers have documented capillaroscopic findings in diabetic patients which correspond to their ophthalmoscopic findings. Every diabetic patient after ten years of the disease is at risk of diabetic retinopathy and should thereby be referred to an ophthalmologist for screening. But for various factors the referral to ophthalmologists is poor and the patients usually present to the ophthalmologist when their vision is threatened. Our study intends to correlate the nailfold capillaroscopic microvascular changes with duration of diabetes, severity of diabetic retinopathy (DR), compare the changes of patients with DR and without DR and determine its role as a screening tool. METHODS This was a hospital based cross-sectional study for over 18 months in patients (15 - 75 yrs. of age) with diabetes mellitus. Patients with other vascular disorders were excluded. The study subjects were evaluated for diabetic retinopathy by indirect ophthalmoscopy, fundus pictures and optical coherence tomography. The nailfold capillaroscopy findings were recorded and co-related with fundus findings. Statistical analysis was done by using the STATA software version 15.1. RESULTS Two hundred and fifty patients were recruited with 125 patients in each group, patients with and without DR respectively. Poor glycaemic control was seen most commonly in patients with proliferative diabetic retinopathy. The reduced capillary density, tortuosity, microhaemorrhages, neoangiogenesis and avascular areas were seen more frequently in proliferative diabetic retinopathy than nonproliferative diabetic retinopathy and patients without DR. (P value 0.00). CONCLUSIONS Changes in nailfold capillaroscopy in diabetics have significant association with severity of DR, duration of diabetes, and glycaemic control. It could be used as screening and early diagnostic tool for non-ophthalmology medical fraternities to refer to ophthalmologist for follow-up and treatment of diabetic retinopathy. KEYWORDS Diabetes Mellitus, Diabetic Retinopathy, Nailfold Capillaroscopy, Microangiopathy

espanolIntroduccion La capilaroscopia periungueal (CP) es util en la evaluacion del fenomeno de Raynaud tanto primario como secundario, y en el seguimiento de los pacientes con esclerosis sistemica. Nuestro estudio evalua el impacto de la CP en el diagnostico, en funcion del motivo de solicitud y el perfil de autoanticuerpos en la practica clinica diaria. Material y metodos Se incluyeron todos los pacientes con al menos una CP entre junio de 2012 y diciembre de 2017. Se revisaron las historias clinicas y se determino de forma dicotomica (si/no) si la CP contribuyo al diagnostico realizado en la consulta posterior a la realizacion de la CP. Se recogieron datos demograficos, clinicos y de laboratorio, motivo de solicitud de la CP y su relacion con los patrones CP. Resultados De 530 pacientes con una primera CP, 266 se realizaron como estudio de un fenomeno de Raynaud. De estos, en 20 pacientes (3,8%) se realizo un diagnostico de enfermedad del tejido conectivo en la consulta posterior a la CP; 15 fueron diagnosticados de esclerosis sistemica, 4 de conectivopatia indiferenciada y uno de enfermedad mixta. Salvo un paciente diagnosticado de conectivopatia indiferenciada, el resto tenia anticuerpos antinucleares positivos y 11 de ellos, ademas, anticuerpos especificos (9 anticentromero, uno anti-Scl70 y otro, anti-RNPC). La positividad de anticuerpos antinucleares se asocio con una mayor probabilidad de presentar una CP de esclerodermia, y ningun paciente diagnosticado de una enfermedad reumatica tras la CP tenia un patron normal. Conclusion La CP es una tecnica util, pero con impacto limitado en el diagnostico de enfermedades del tejido conectivo. La positividad de los anticuerpos se relaciona con una mayor probabilidad de presentar patrones patologicos en la CP. EnglishIntroduction Nailfold capillaroscopy (NC) is useful in the evaluation of Raynaud's phenomenon, associated with some connective tissue diseases and in the follow-up of patients with systemic sclerosis. Our study evaluates the impact of NC in the diagnosis, according to the reason for the request and profile of autoantibodies in daily clinical practice. Material and methods All patients that undergone at least one NC between June 2012 and December 2017 were included. Clinical records were reviewed and analysed in a dichotomous way (yes/no), to see whether the NC contributed to a change of diagnosis in subsequent consultations. In addition, demographic, clinical and laboratory data were collected, and the relationship with NC patterns evaluated. Results Of the 530 patients who had undergone at least one NC, 266 had Raynaud's phenomenon as primary indication for the technique. Of those, 20 patients (3.8%) had a diagnostic change in the post-NC consultation; 15 were diagnosed with systemic sclerosis, 4 with undifferentiated connective tissue disease and one with mixed connective tissue disease. All patients had, except for one patient diagnosed with undifferentiated connective tissue disease, positive antinuclear antibodies titres, 11 of them had disease specific antibodies (9 anti-centromere, one anti-Scl70 and other anti-RNPC). The positivity of antinuclear antibodies titres was associated with a higher probability of presenting a scleroderma pattern in the NC, and all patients with a specific rheumatological diagnosis had an abnormal NC. Conclusion NC is a useful technique, but with limited impact in the diagnosis of connective tissue diseases. Autoantibody positivity is associated with a greater likelihood of presenting pathological NC patterns.

Amaç: Tırnak kapilloskopisi ve modifiye Rodnan skoru sistemik sklerozda (SS) çeşitli durumlarda klinik progresyon ile ilişkili bulunmuş olmasına rağmen sağ ventrikül fonksiyonları ile ilişkileri yeterince çalışılmamıştır. Bu çalışmada aşikar pulmoner hipertansiyonu olmayan sistemik sklerozlu hastalarda sağ ventrikül fonksiyonları ile modifiye Rodnan skoru, tırnak kapillaroskopisi gibi klinik SS klinik parametreleri ile arasındaki ilişki araştırılmıştır.Gereç ve Yöntem: Otuz iki SS’li hasta ve yirmi sağlıklı birey kontrol grubu için çalışmaya alınmıştır. Hastalar romatoloji uzmanı tarafından dijital ülserler, tırnak kapillaroskopisi ve deri tutulumu için değerlendirilmiştir. Tüm katılımcılara ekokardiyografik değerlendirme kardiyoloji uzmanı tarafından yapılmıştır. Ekokardiyografik parametreler Amerikan Ekokardiyografi Birliğinin kılavuzlarına göre hesaplanmıştır.Bulgular: Sistolik pulmoner arteriyel SS grubunda istatiksel olarak anlamlı şekilde yüksekti (26,4±3,2 vs 30,8±3,6 mmHg, p=0,001). Triküspit anüler plan sistolik hareketi (TAPSE), pulmoner akselerasyon zamanı ve ortalama sağ ventrikül strain değeri SS grubunda anlamlı olarak daha düşüktü (p=0,003, p=0,001, p=0,001). Kapillaroskopik olarak aktif ve geç patern ve geç paterne sahip hastalarda erken ve aktif paterne ve erken paterne sahip hastalara göre TAPSE, ventriküler isovolumik akselerasyon ve sağ ventrikül ortalama serbest duvar straini anlamlı olarak daha düşüktü (p=0,002, p=0,005, and p=0,001, sırasıyla) Univariat doğrusal regresyon analizde, SS grubunda modifiye Rodnan cilt skoru ile sağ ventrikül serbest duvar ortalama strain arasında anlamlı ilişki vardı (R2=0,192; p=0,007).Sonuç: Bu çalışmada SS’li hastalarda sistolik pulmoner arteriyel basınçta ve sağ ventrikül fonksiyonlarında erken dönemde bozulma olduğu gösterilmiştir. Ayrıca yüksek modifiye Rodnan cilt skoru ve geç dönem kapillaroskopik değişikliklerin sağ ventrikül fonksiyonlarındaki bozulmayla ilişkili olduğu bulunmuştur., Objective: Although nailfold capillaroscopy (NC) and modified Rodnan skin score (mRSS) have already been studied in a variety of contexts related to Systemic Sclerosis (SSc) progression, there is limited data about the relationships between NC, mRSS, and right ventricle (RV) function in SSc patients without overt pulmonary arterial hypertension (PAH). In this study, we examined the relationship between RV function and clinical SSc parameters such as NC pattern and mRSS. Material and Method: Thirty two patients with SSc and twenty healthy participants as a control group were enrolled in this study. Patients with SSc were assessed for digital ulcers, Raynaud’s phenomenon, and severity of skin involvement by a rheumatology specialist. Also, all participants underwent echocardiographic examinations by cardiology specialists. The echo parameters were measured considering the criteria of the American Society of Echocardiography guidelines. Results: Systolic pulmonary arterial pressure (sPAP) was statistically higher in the SSc group (26.4±3.2 vs 30.8±3.6 mmHg, p

Contains fulltext : 287595.pdf (Publisher’s version ) (Closed access) OBJECTIVES: Haematopoietic stem cell transplantation (HSCT) is a treatment option for patients with severe systemic sclerosis (SSc), but the efficacy of the procedure in remodelling the nailfold microvascular array is largely unknown. Therefore, this study aimed to evaluate the effect of HSCT on microangiopathy assessed through nailfold capillaroscopy (NC) and to compare the results with findings in patients receiving conventional immunosuppression. METHODS: We included SSc patients with severe SSc and whose pre- and post-treatment NC images were available. Findings in patients treated with HSCT were compared with patients not treated with HSCT. Images were scored by two independent observers blinded for clinical data and treatment history. Capillary pattern was determined and semiquantitative scores from 0 (no changes) to 3 (>66% alterations per millimetre) were used to quantify the degree of specific microvascular characteristics. Changes in severity of microangiopathy between baseline and post-treatment were compared between groups. RESULTS: Images of 18 HSCT patients and 21 controls were scored. From baseline to follow-up, 33% of HSCT patients showed improvement from scleroderma pattern to normal NC, compared to 6% of controls (p=0.15). Pre- to post-treatment differences in semiquantitative scores showed significant improvement in HSCT patients compared to controls regarding capillary loss (-0.5 vs. 0.0, p

Background Limited joint mobility (LJM), previously known as cheiroarthropathy, refers to the presence of reduced extension at the finger joints in people with diabetes and may be associated with scleroderma-like syndromes such as diabetic sclerodactyly. While scleroderma-like syndromes and LJM have been observed in patients with long-term diabetes and associated complications, the coexistence of diabetes with Juvenile systemic sclerosis (jSSc) is rarely described. Case presentation We describe the case of a 14-year-old boy with long-lasting type 1 diabetes (T1D) and suspected LJM associated with Raynaud phenomenon, sclerodactyly and tapering of the fingertips. A comprehensive work-up showed positive autoantibodies (ANA, anti-Ro-52, anti-Mi-2b), abnormal nailfold capillaroscopy with a scleroderma pattern, interstitial lung disease and cardiac involvement. The overall clinical picture was consistent with the diagnosis of jSSc. Conclusions LJM can be the initial sign of underlying systemic sclerosis. Nailfold capillaroscopy may help differentiate jSSc from classical LJM in pediatric patients with T1D and finger contractures or skin induration of no clear origin. This case report provides a starting point for a novel hypothesis regarding the pathogenesis of jSSc. The association between T1D and jSSc may be more than a coincidence and could suggest a relationship between glucose metabolism, fibrosis and microangiopathy.

IntroductionSystemic sclerosis (SSc) is a connective tissue disease affecting the skin and multiple organs. Most of the evidence on ocular involvement comes from small studies. We evaluated the retinal vascular changes in patients with SSc and its associations with types, disease duration, skin score, and nailfold capillaroscopy changes.Material and methodsWe evaluated the demographic, clinical and nailfold capillaroscopy data of 52 patients referring to the clinic of scleroderma with SSc according to the 2013 ACR/EULAR SSc criteria. In addition, full ophthalmic examination in 52 patients and fundus photography in 40 patients were done in the ophthalmology clinic.ResultsThere were 52 (49 women and 3 men, 17 diffuse and 35 limited) patients with SSc with mean disease duration of 8 ±5 years and mean age of 40 ±9 years. Retinal changes in the ophthalmologic examination were seen in 30.7% as increased vascular tortuosity. None of the patients had cotton wool spot, hemorrhage or hard exudate. Forty patients underwent fundus photography and 22.5% of them had vascular tortuosity. Only the presence of hemorrhage in the nailfolds was correlated with retinal tortuosity, and the other characteristics of nailfold capillaroscopy did not have any association with retinal changes.ConclusionsRetinal vascular changes were seen in about one third of our patients. There was no correlation between nailfold capillaroscopy, disease duration, type and skin score of SSc patients and retinal vascular changes. These findings suggest that the mechanisms or the quality of changes in the retinal vessels and nailfold vasculature may be different.

We present the case of a 50‐year‐old woman with febrile myalgia, chilblains‐like lesions, and cough. Antinuclear antibodies and anti‐PL‐12 antisynthetase autoantibodies were found in complementary examinations. Interstitial lung disease was found on chest computed tomography. Nailfold capillaroscopy showed microangiopathic lesions. Antisynthetase syndrome is a recently described entity in inflammatory myopathies, with specific clinical criteria. Interstitial lung disease is very common, especially in anti‐PL‐12 associated antisynthetase syndrome. Raynaud's phenomenon is another well‐defined criterion. However, microangiopathic damage is probably underestimated and the role of nailfold capillaroscopy in the diagnosis has not been established yet., Antisynthetase syndrome (ASS) is a syndrome with various clinical manifestations. The diagnosis is often difficult and needs a broad range of clinical and serological arguments to be thought of. Future studies are needed to define the role of capillaroscopy in the diagnosis and the prognosis of the disease.

Nailfold video-capillaroscopy (NVC) is a useful diagnostic tool, used to early detect abnormalities in micro-circulation, providing a qualitative description of microvascular anomalies in Raynaud's phenomenon. NVC role in the diagnosis of Systemic Sclerosis is well known. In other rheumatic conditions such as connective tissue diseases, vasculitis, and arthritis, the NVC anomalies are often included in a scleroderma like pattern. The use of NVC in non-rheumatic diseases (NRD), with remarkable microvascular damage, as diabetes, is not standardized yet, although several research studies are carrying on. The aim of this article is to provide a resume of published results in order to lay the groundwork for the employment of NVC both in the diagnosis and follow up of microvascular complication in NRD. Furthermore, we mention NVC findings in pathologies without well recognize microvascular damages in their pathogenesis : micro-vessels abnormalities may suggest a different point of view. J. Med. Invest. 68 : 6-14, February, 2021.

Purpose of reviewTo identify clues to disease activity and discuss therapy options.Recent findingsThe diagnostic evaluation includes documenting symmetrical proximal muscle damage by exam and MRI, as well as elevated muscle enzymes—aldolase, creatine phosphokinase, LDH, and SGOT—which often normalize with a longer duration of untreated disease. Ultrasound identifies persistent, occult muscle inflammation. The myositis-specific antibodies (MSA) and myositis-associated antibodies (MAA) are associated with specific disease course variations. Anti-NXP-2 is found in younger children and is associated with calcinosis; anti-TIF-1γ+ juvenile dermatomyositis has a longer disease course. The diagnostic rash—involving the eyelids, hands, knees, face, and upper chest—is the most persistent symptom and is associated with microvascular compromise, reflected by loss of nailfold (periungual) end row capillaries. This loss is associated with decreased bioavailability of oral prednisone; the bioavailability of other orally administered medications should also be considered. At diagnosis, at least 3 days of intravenous methyl prednisolone may help control the HLA-restricted and type 1/2 interferon–driven inflammatory process. The requirement for avoidance of ultraviolet light exposure mandates vitamin D supplementation.SummaryThis often chronic illness targets the cardiovascular system; mortality has decreased from 30 to 1–2% with corticosteroids. New serological biomarkers indicate occult inflammation: ↑CXCL-10 predicts a longer disease course. Some biologic therapies appear promising.

Nailfold capillaroscopy is a non-invasive method for visualising the nailfold capillaries under a stereomicroscope or videocapillaroscope. The examination is used for differential diagnosis of Raynaud`s phenomenon and microcirculation disorders in secondary Raynaud’s phenomenon associated with systemic connective tissue diseases [1]. Capillaroscopy is particularly important for early diagnosis of systemic sclerosis (SSc) for assessing the disease activity and the extent of internal organ involvement as well as for monitoring and evaluating the effectiveness of the therapy applied. In SSc, the capillaroscopic examination shows characteristic microcirculation disturbance called microangiopathy typical of SSc “scleroderma pattern”. The term microangiopathy includes the following pathological changes: dilated capillary loops leading to formation of megacapillaries, decreased numbers of vessels, the areas of avascularisation, and the presence of ramified loops and microhaemorrhages [2].

We review the exciting potential (and challenges) of quantitative nailfold capillaroscopy, focusing on its role in systemic sclerosis. Quantifying abnormality, including automated analysis of nailfold images, overcomes the subjectivity of qualitative/descriptive image interpretation. First we consider the rationale for quantitative analysis, including the potential for precise discrimination between normal and abnormal capillaries and for reliable measurement of disease progression and treatment response. We discuss nailfold image acquisition and interpretation, and describe how early work on semi-quantitative and quantitative analysis paved the way for semi-automated and automated analysis. Measurement of red blood cell velocity is described briefly. Finally we give a personal view on ‘next steps’. From a clinical perspective, increased uptake of nailfold capillaroscopy by general rheumatologists could be achieved via low-cost hand-held devices with cloud-based automated analysis. From a research perspective, automated analysis could facilitate large-scale prospective studies using capillaroscopic parameters as possible biomarkers of systemic sclerosis-spectrum disorders.

Objectives: This study aims to evaluate nailfold capillaroscopic pattern in patents with fibromyalgia and to assess the relation of capillaroscopic parameters with clinical variables and disease-related measures. Patients and methods: This cross-sectional, case-control study included 60 participants (4 males, 56 females; mean age: 44.0±8.2 years; range, 26 to 64 years) between August 2019 and November 2019. All participants were divided into two groups as the primary fibromyalgia group (n=30) who met the 2016 modified American College of Rheumatology Diagnostic Criteria for Fibromyalgia and the control group (n=30) consisting of age- and sex-matched healthy individuals. Nailfold capillaroscopy was performed by a digital microscope under a magnification of 200X. Capillary density, capillary loop diameter, number of dilated, giant and neoangiogenic capillaries, capillary shape, number of avascular areas, micro-aneurysms and micro-hemorrhages were evaluated by an assessor who was blind to the group allocation. In the fibromyalgia group, Widespread Pain Index, Symptom Severity Scale scores, and Fibromyalgia Severity scores were calculated. Health status and presence of benign joint hypermobility syndrome (BJHS) were evaluated using the Fibromyalgia Impact Questionnaire (FIQ) and revised Brighton criteria, respectively. Results: Of the capillaroscopic parameters, the mean capillary loop diameter, number of micro-aneurysms, avascular areas, and neoangiogenic capillaries were significantly higher in the patient group compared to the controls (p Conclusion: Patients with fibromyalgia have distinct capillaroscopic patterns than healthy population. Capillaroscopic features, in general, are not related to clinical variables and disease-related measures.

Background: Microcirculation in the skin of psoriasis patients significantly differs from unaffected individuals. Vascular changes precede skin lesions and occur even in nonlesional skin. Aims: The primary aim of this work was to study the nailfold capillary changes in psoriasis patients, and to compare it with that of controls. The secondary aim of this work was to compare the nailfold capillary changes in psoriasis patients with and without nail changes. Methods: A comparative cross-sectional study of 40 psoriasis patients and 40 age and sex-matched controls analyzed the capillaries of the proximal nailfold of all fingers using a dermoscope. The mean capillary loop density/mm, tortuous capillaries, capillary hemorrhages, and avascular areas were assessed. Results: The mean nailfold capillary loop density in psoriasis (6.98 ± 0.54 per mm) was significantly less than that in controls (8.01 ± 0.61 per mm) (P < 0.001). Avascular areas in the nailfold of psoriasis patients (55%) were significantly more than the same in controls (22.5%) (P = 0.003). Of 40 psoriasis patients, 26 had nail psoriasis. Of this, 19 (73%) had avascular areas (P = 0.002). No significant association of nailfold capillary density or avascular areas with disease duration or severity was noted. An increase in tortuous capillaries and nailfold hemorrhages noted in psoriasis was not significant. Limitations: Small sample size, not having participants with psoriatic arthropathy, and lack of information on capillary diameter and capillary changes in hyponychium were the limitations. Conclusion: The reduced mean capillary loop density, and higher frequency of avascular areas noted in cases compared to controls, points to a pathogenic role for microvascular damage in psoriasis. Whether the lack of association of these changes with disease duration and severity suggests the possibility of these being early disease markers for psoriasis, needs further analysis in larger prospective studies.

Aims/Introduction Crossing capillaries in the finger nailfold might potentially be a novel diabetic retinopathy (DR) biomarker that could be assessed non‐invasively in the clinical setting. However, the association between crossing capillaries and DR is controversial. This study aimed to investigate the association between the percentage of crossing capillaries in the finger nailfold and DR in patients with type 2 diabetes mellitus. Materials and Methods This cross‐sectional study enrolled 108 type 2 diabetes mellitus patients (aged 40–75 years) who visited the outpatient diabetic clinic at Osaka University Hospital, Osaka, Japan, between May and October 2019. Capillary morphology was assessed using nailfold capillaroscopy based on the simple capillaroscopic definitions of the European League Against Rheumatism Study Group. Details of DR and other laboratory data were obtained from medical records. The association between the tertile of the percentage of the crossing capillary and DR was analyzed using multivariable logistic regression. Results After adjusting for age, sex, diabetes duration, glycated hemoglobin, systolic blood pressure, body mass index, and use of renin–angiotensin system inhibitor and antihyperlipidemic medication, the percentage of crossing capillaries was significantly associated with DR (multivariable‐adjusted odds ratios for increasing tertiles of the percentage of crossing capillary: 1 [reference], 2.05 [95% confidence interval 0.53–7.94], and 4.33 [95% confidence interval 1.16–16.21]; P‐trend = 0.028). Conclusions A higher percentage of crossing capillaries in the nailfold was associated with a higher risk of DR, independent of traditional risk and inhibiting factors, in patients with type 2 diabetes mellitus., Crossing capillaries in the finger nailfold might potentially be a novel diabetic retinopathy biomarker that could be assessed non‐invasively in a clinical setting. We investigated the association between crossing capillaries and diabetic retinopathy in patients with type 2 diabetes mellitus. A higher percentage of crossing capillaries in the nailfold was associated with a higher risk of diabetic retinopathy in patients with type 2 diabetes mellitus, independent of traditional risk and inhibiting factors.

Background: Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with increased risk of atherosclerosis. Despite the relative youth of lupus patients, they have risk for the development of premature cardiovascular atherosclerotic diseases. Objective: This work aimed to study the detection of atherosclerosis and the cardiovascular risk in SLE patients by nail fold capillaroscopy and the intima media thickness of the carotid artery. Patients and methods: This was a cross sectional study included 60 SLE patients. Patients were collected from the Outpatient Clinic and Inpatient of Internal Medicine and Rheumatology Departments within 6 months. Results: 22 patients (36.7%) had atherosclerosis, with higher SLE disease activity index (SLEDAI) score (p 0.002). 45.5 % of them had severe disease activity and 54.5% had moderate disease activity. Atherosclerotic patients had statistically significant lower capillary density (P 0.001), higher capillary length (P 0.008), capillary width (P < 0.001) and arterial limb diameter (P< 0.001) with higher prevalence of tortuous (P= 0.022), meandering (p=0.014) and disorganized capillaries (P= 0.008). There was statistically significant positive correlation between Intima media thickness (IMT) and disease duration, SLEDAI score, capillary width and arterial limb diameter, while there was statistically significant negative correlation with capillary density. The Arterial limb diameter had highest diagnostic performance in diagnosing atherosclerosis. Conclusion: Atherosclerosis is common in SLE & is associated with higher diseases activity. By capillaroscopy, lower density, longer, wider and disorganized capillaries and tortuous & meandering capillaries were associated with the presence of atherosclerosis.

Background: Nailfold capillaroscopy (NFC) has been described to be a valuable instrument used in diagnosis of systemic sclerosis (SSc) and juvenile dermatomyositis (JDM) patients. Though, NFC has been infrequently assessed in adult dermatomyositis (DM) patients. Objectives: Our aim was to visualize nailfold capillary changes in dermatomyositis (DM) and polymyositis (PM) Egyptian patients and to identify a specific diagnostic capillary pattern and to assess its relevance to disease activity, clinical and laboratory findings. Patients and Methods: This study included 20 patients (12 DM, 5 PM, and 3 overlap syndrome). Routine laboratory assessment was done, disease and skin activity scores were evaluated. Assessment of the nailfold capillary circulation using the videocapillaroscope was done and capillary density score was assessed. Results: The mean age was 39±12 years and their mean disease duration was 24±19 months. The mean muscle disease activity score was 3.8±3.3, and global disease activity score (DAS) 3.92±3.09. The mean skin activity score was 2±3. The global DAS was significantly higher in patients with branched capillaries (p=0.041). Skin activity score was significantly higher in patients with capillary hemorrhage (p=0.024). More severe capillaroscopic findings were prominent in DM patients rather than PM or overlap patients. Conclusion: Capillary branching is more common in patients with higher global DAS and capillary hemorrhage is more frequent in patients with higher skin activity score. Capillaroscopic changes are evident in DM patients rather than PM or overlap patients.

Background: Pulmonary arterial hypertension (PAH) represents one of the main clinical pictures of microvascular damage in systemic sclerosis (SSc). Objective: To assess the role of nailfold capillaroscopy (NFC) changes as a screening tool for the existence of PAH in patients with systemic sclerosis. Patients and methods: Forty SSc patients were studied, included: 20 patients with PAH (diagnosed by transthoracic echocardiography and confirmed by right heart catheterization) and another 20 without PAH. History taking, clinical examination, and laboratory investigations including serological biomarkers (anti-centromere and anti-topoisomerase1 antibodies) have been recorded. NFC was performed for all patients according to the standard method. Results: Scleroderma patients with PAH had significantly a longer disease duration (p=0.002) and higher frequencies of positive anticentromere and anti-topoisomerase 1 antibodies (p=0.011 and p=0.010 respectively). As regard NFC picture, SSc patients without PAH had significantly higher frequencies of early scleroderma pattern changes in 73.7% (p= 0.003), avascular area score 0 in 52.6% (p=0.014) and total NFC score 0 in 57.9% (p=0.022). In contrast, SSc patients with PAH showed higher frequencies of late scleroderma pattern changes in 50% (p=0.005) with lower capillary density (p

Background: Behcet’s disease is a systemic autoimmune disease. Treatment mainly is immunosuppression. It has no sure diagnostic tests and relies mainly on clinical diagnosis. There are few scores to assess Behcet's disease activity (Behcet's Disease Current Activity Form) BDCAF score. Nailfold Capillaroscopy is a technique to visualize capillaries in nailfold area. It can correlate many diseases with the abnormalities in shape of blood capillaries. Objectives: The aim of the work was to find out Nail Fold Capillaroscopy abnormality in Behcet's Disease and its relation to the disease activity. Patients and Methods: A cross sectional-case control study which was done on 50 Behcet’s patients and 30 healthy controls. We assessed nailfold capillaroscopic changes in Behcet’s patients in comparison to controls and the correlation of BDCAF score with nailfold capillary abnormalities. Results: we found that 70% of Behcet's Disease patients had nailfold capillaroscopy abnormalities , a highly statistically significant changes in the capillaries in the form of capillary dilatation (24%), hemorrhage (54%), tortuosity (64%) in comparison to the control group (P

Background. Nailfold capillaroscopy is a noninvasive technique to recognize peripheral microangiopathy, which is an important feature in systemic lupus erythematosus (SLE). The aims of the present study were to investigate the prevalence of nailfold capillaroscopy (NFC) changes in juvenile systemic lupus erythematosus (JSLE), find out patterns of these changes, and correlate findings with clinical and laboratory parameters. Methods. Forty-nine patients with SLE, all meeting the 1997 revised ACR criteria for SLE classification, and 30 healthy controls were included. A digital video camera was used to capture nailfold capillary images. Computerized image processing was used for analysis. Results. Different abnormal NFC changes were observed with abnormal morphology, the increased diameter and abnormal loop densities in 55.1%, 93.9%, and 26.5% of the patients, respectively. No statistically significant differences were depicted between capillaroscopy with age, gender, autoantibodies (APLs, anti-ds DNA), antiphospholipid antibody syndrome, thrombotic angiopathy, renal function tests (Bun, Cr), and abnormal urine analysis. However, a significant correlation was found between the branched pattern and the CNS involvement group (P value Conclusions. Different abnormal NFC changes are quite common among patients with SLE, and nailfold capillaroscopy is an effective method to monitor such changes. Treatment strategies may change in the branched pattern of nailfold capillaroscopy due to CNS involvement.

Raynaud’s phenomenon (RP) is a chronic episodic attack of digital ischemia provoked by exposure to cold or emotional stres. This phenomenon affects 3-5% of the population, with female ratio more than men and is categorized into a primary and secondary form. Primary form of RP is idiopathic, meanwhile the secondary form is associated with underlying diseases and other condition such as connective tissue disease, obstructive arterial disease, neurologic disorders, drugs and toxin, hyperviscosity disorders, infections, endocrine disturbance, neoplasms, and occupation or environmental exposure. Until today, to classified and build the diagnosis of RP is still a clinical challenge. Therefore, a careful history and a physical examination, together with laboratory tests and nailfold capillaroscopy are mandatory. A combination of conservative measures and medications can help in the management of RP. The approach to the RP patients requires therefore a coordinated care of specialists including dermatology.

The term "microcirculation" refers to the terminal vascular network of the body, which includes arterioles, capillaries, venules as well as initial lymphatic vessels. Additionally, it insinuates to their unique function in thermoregulatior, fluid balance, maintenance of cellular exchange, and metabolism. Disturbances of microvascular function were identified to precede macrovascular involvement in the presence of cardiovascular risk factors and is the hallmark of terminal disease stages like critical limb or acral ischemia. Nevertheless, despite its obvious significance in vascular medicine assessment of microvascular function became increasingly neglected in the clinical institutions during the last decades and seems to play a subordinary role in medical education. We therefore provide an overview over relevant and clinically practicable methods to assess microcirculation in vascular medicine with critical estimations of their pros and cons and their perspectives in the future.

Objectives This study aims to assess the nailfold capillary changes in patients with systemic lupus erythematosus (SLE), particularly among those with Raynaud's phenomenon (RP), and the correlation between nailfold capillary changes and autoantibodies and disease activity. Patients and methods A total of 85 patients (9 males, 76 females; median age 31 years; range, 15 to 58 years) with newly diagnosed SLE were selected between July 2016 and July 2018 from our hospital. Disease activity was scored by the SLE Disease Activity Index. Nailfold capillaroscopy (NFC) was performed in all patients. Results Normal pattern, non-specific pattern, and scleroderma pattern were found in 13 (15.3%), 64 (75.3%), and eight (9.4%) patients, respectively. There was no significant difference between anti-double stranded deoxyribonucleic acid, anti-Smith antibodies, and low complements (all p>0.05), while significant differences of NFC pattern were found between low disease activity and high disease activity (p=0.002). RP was present in 31.7% of SLE patients, and the NFC findings in SLE patients with and without RP were significantly different in dilatation (81.5% vs. 14.0%). Conclusion The results of our study showed that capillary changes were very common in patients with SLE, which seem to associate with disease activity and RP condition.

Summary. The term “microcirculation” refers to the terminal vascular network of the body, which includes arterioles, capillaries, venules as well as initial lymphatic vessels. Additionally, it insinuates to their unique function in thermoregulation, fluid balance, maintenance of cellular exchange, and metabolism. Disturbances of microvascular function were identified to precede macrovascular involvement in the presence of cardiovascular risk factors and is the hallmark of terminal disease stages like critical limb or acral ischemia. Nevertheless, despite its obvious significance in vascular medicine assessment of microvascular function became increasingly neglected in the clinical institutions during the last decades and seems to play a subordinary role in medical education. We therefore provide an overview over relevant and clinically practicable methods to assess microcirculation in vascular medicine with critical estimations of their pros and cons and their perspectives in the future.

Background: The development of graft vasculopathy may play a role in the long-term deterioration of hand grafts. The aim of study was to examine the patterns of the nailfold capillaries in hand transplant recipients. Methods: the study was performed on six patients who received hand transplantation. To normalize for the effect of immunosuppression an age- and sex-matched group of 12 patients with active kidney transplant was selected. As an additional control group, 12 healthy volunteers were recruited. Nailfold videocapillaroscopy was performed in all participants. Additionally, serum concentrations of vascular endothelial growth factor (VEGF) were measured. Results: Videocapillaroscopic examination of the hand allografts revealed significant abnormalities: including capillary disorganization and microhaemorrhages. The number of capillaries was reduced, the vessels were enlarged and branched. Surprisingly, similar, albeit slightly less pronounced, changes were seen in the nailfolds of healthy hands of the limb transplant recipients. In kidney transplant recipients the capillaroscopic pattern was general normal and comparable to healthy individuals. Moreover, serum concentrations of VEGF in all participants correlated with average capillary diameter in capillaroscopy. Conclusions: in hand transplant recipients advanced microvascular abnormalities are found in nailfold capillaroscopic pattern in both transplanted and own extremities connected with elevated levels of VEGF.

Introduction Mixed connective tissue disease (MCTD) is a chronic immune-mediated disorder defined by the combined presence of serum anti-RNP antibodies and distinct clinical features including progressive lung fibrosis. The aim of this study was to evaluate potential associations between lung fibrosis in MCTD and specific clinical and laboratory findings including results of nailfold capillaroscopy (NFC) examination. Material and methods Patients with MCTD who were admitted to the Departments of Allergy and Immunology or Dermatology at the University Hospital of Krakow (Poland) in 2015–2018 were identified based on comprehensive individual record review. Diagnosis of MCTD required fulfilment of at least one of the four widely accepted sets of diagnostic criteria. Clinical and laboratory data were collected, and statistical analysis was performed to identify potential predictors of interstitial lung disease (ILD). Results Thirty patients (90% females) aged 22–81 years met the study inclusion criteria. The mean duration of symptoms associated with MCTD was 7.3 months. Photosensitivity and Raynaud’s phenomenon were the most common clinical manifestations (90% and 70%, respectively). Hand oedema, sclerodactyly and the presence of giant capillaries in NFC correlated significantly with the risk of lung involvement. In multivariate analysis, the presence of enlarged loops in NFC (giant capillaries) was identified as an independent factor for ILD (R 2 = 0.82, p < 0.0000001). Conclusions The NFC examination should be considered in all patients with a diagnosis of MCTD. The presence of giant capillaries may be a promising marker for interstitial lung disease in these patients, especially among those with a short duration of disease (i.e. < 1 year).

Intravenous iloprost is currently recommended in the treatment of Raynaud's phenomenon (RP) refractory to oral therapy and of digital ulcers (DUs) related to systemic sclerosis (SSc). In real-life practice there is a huge heterogeneity about the Iloprost regimens used.A survey was carried out on SSc patients that interrupted Iloprost infusion to compare acral vascular symptoms just before Iloprost withdrawal and just after the missed infusion. Severity, and frequency of RP, new DUs onset or aggravation of those pre-existing were reported. Last available capillaroscopic images were also evaluated.The analysis includes 50 patients. After iloprost withdrawal, 11 patients reported a RP worsening because of enhanced intensity (p = 0.007). Only 8 patients of them also complained of an increased frequency (p = 0.07). None of the patients experienced digital ulcers for the first-time during quarantine. Among the 27 patients with a history of digital ulcers, 9 reported worsening and 7 recurrence of DUs. Overall, 17 patients (34.0 %) complained of a worsening of SSc vascular acral manifestations, namely RP or DUs. Reduced capillary density was associated with RP worsening, in particular, each unit increase of capillary density corresponds to an average 44 % decrease in the odds of RP worsening (OR 0.56, CI 95 % 0.36-0.97, p = 0.037). As for RP worsening, the aggravation of DU was associated with a lower capillary density.Low capillary density can predict a worsening of both RP and DUs in controlled quarantine conditions within a month after iloprost discontinuation in SSc patients.

Systemic sclerosis (SSc) is a connective tissue disease characterized by immune-system alterations, fibrosis involving the skin and internal organs and diffuse microangiopathy. Pulmonary arterial hypertension (PAH) is a severe complication of SSc affecting about 10–15% of the patients and it is a leading cause of mortality. Due to the devastating nature of SSc-PAH, there is a clear need to systematically adopt appropriate screening programs. Nail fold videocapillaroscopy (NVC) studies have shown a more severe peripheral microvascular dysfunction in SSc patients with PAH suggesting that abnormalities in peripheral microcirculation may correlate with pulmonary microangiopathy. This is a cross-sectional study involving four tertiary University Rheumatology Units in the Center-North of Italy. Seventy patients, 35 adults with SSc and PAH confirmed by RHC (F/M 34/1; median age 65.2 ± 8.9 SD yrs), and 35 SSc patients without PAH were enrolled (F/M 3471; median age 63.3 ± 10.3 SD yrs). Clinical, laboratoristic and instrumental data were collected and NVC was performed in all patient. Specific NVC parameters were evaluated and a semi-quantitative rating scale was adopted to score these changes. Finally, patients were distributed into the suitable NVC pattern belonging to the scleroderma pattern. Our aim was to compare the peripheral microangiopathy changes in SSc patients with and without PAH, and to investigate the relationship between NVC findings and the main hemodynamic parameters of pulmonary vasculopathy. Patients with SSc-PAH+ showed a significant higher frequency of interstitial lung disease (ILD). No significant differences regarding clinical and laboratoristic parameters were observed. NVC abnormalities, avascular areas were more frequent in SSc patients with PAH, respect to those without (p = 0.03), and capillary density was significantly lower when considering grade 3 (p = 0.02). A higher NVC semiquantitative mean was found in SSc-PAH+ patients and a greater rate of the “late” pattern was detected in SSc-PAH+ subjects in respect to PAH- (57.1% vs. 25.7%) (p = 0.03). A significant correlations between pulmonary pressure values (sPAP by TTE and mPAP by RHC) and the capillary density (Spearman's rho 0.35, p = 0.04 for both). Our findings provide additional evidence to the literature data, confirming that a higher degree of peripheral nailfold microangiopathy is more common in SSc-PAH patients, and further strengthening the concept that NVC changes may run parallel with similar abnormalities inside pulmonary microcirculation.

Introduction Microvasculopathy is characterized by progressive structural and functional damage to the microvessels and plays a key role in the pathogenesis of various connective tissue diseases (CTD). Nailfold videocapillaroscopy is an optimal and validated method for analysis of microvascular abnormalities and is able to differentiate secondary Raynaud’s phenomenon (RP) of CTD from primary RP and healthy subjects. Aim To assess and analyze nailfold capillaroscopic findings in Indian subjects with secondary Raynaud and to compare with findings in healthy subjects. Methods A total of 62 study participants including cases and controls underwent nailfold videocapillaroscopy. Capillary loop length, capillary width, capillary density, presence/absence of tortuosity, giant loops, neoangiogenesis, microhemorrhages, and avascular areas were the parameters studied. Results All the quantitative and qualitative parameters studied were significantly associated with secondary RP. Mean loop length in cases of connective tissue diseases was significantly less than in the controls (225.74 μm versus 282.97 μm) (P=0.002). Capillary density was also reduced significantly in the cases as compared to the controls (4.6 versus 7.39/mm) (P

Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by vascular complications, endothelial cell injury with subsequent dysfunction. Nailfold video capillaroscopy (NVC) is a non-invasive and reliable point-of-care method that can be used to directly visualize microscopic digital capillaries and monitor patients. In this review, we will describe NVC’s role in assessing SLE disease activity and prognosticating risk for developing vascular complications. In SLE, qualitative NVC changes like the presence of microhemorrhages and enlarged capillaries correlate with disease activity. These abnormalities are also associated with specific autoantibody profiles (e.g., anti-phospholipid and anti-U1-RNP antibodies). Recent studies show NVC changes may increase risks of digital ulceration, pulmonary arterial hypertension, and accelerated cardiovascular disease. NVC may be used in personalized medicine as an adjunct in assessing SLE activity which may help prognosticate risk of serious complications, but more studies are needed to further delineate its role in specific SLE disease subtypes.

Nailfold capillaroscopy (NFC) is a non-invasive tool validated for systemic sclerosis diagnosis. The role and interpretation of NFC in interstitial lung disease (ILD) patients for the diagnosis of connective tissue disease associated ILD (CTD-ILD) remains undefined. In a prospective study, quantitative and qualitative NFC by smartphone-dermatoscope (3M Dermlite-DL4ΤΜ attached to iPhone-6plusΤΜ) was performed in 96 patients with well-defined CTD-ILD (n=27) and non-CTD ILD (n=69; idiopathic interstitial pneumonia n=42, interstitial pneumonia with autoimmune features n=27) by ILD-multidisciplinary meeting. NFC scoring was performed by two independent, blinded specialist rheumatologists. Comprehensive baseline clinical, serological, physiological and radiological data were included. Multivariable models for CTD diagnosis in ILD, comprising nailfold characteristics at empirical thresholds determined by receiver operating characteristic curve analysis and clinical variables, were explored. In 94 patients with complete NFC data (total 687 images, median eight images per patient from eight digits), low capillary density (, Nailfold capillaroscopy by smartphone-dermatoscope is a safe, feasible tool that may improve the identification of connective tissue disease associated with interstitial lung disease beyond routine clinical assessment https://bit.ly/3iLjxUy

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 &lt, 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.

Machine learning can accurately classify the clarity of nailfold capillaroscopy (NC) images and be applied as a verification tool during the preliminary design stage of medical devices. The method can be beneficial for systems that require a myriad of image datasets. This study has focused on the design, integration, and verification of the NC. The study utilized the low-cost NC prototype to record two videos and extract 600 photos, including both blurry and sharp images. Then we used the Laplace operator method for blur detection among the pictures. Statistics has shown the Laplace score of each photo and lead to the distribution of clear photos in NC. Finally, t-SNE, an unsupervised nonlinear machine learning technique, was applied as a data visualization tool to determine clear images. The results showed similar cluster distribution of clear images in NC with the t-SNE method and the Laplace operator method.

In recent years, a novel Doppler ultrasonography (US) modality—superb microvascular imaging (SMI)—has been presented as a reliable method to evaluate small vessel blood flow with minimised motion artefacts. In this review, we present the challenges of incorporating SMI in daily practice with detailed and comparable US images of a fingertip. The main focus of this paper is the discussion of all tested US techniques, artefacts, and practical challenges for evaluating Raynaud’s syndrome in systemic sclerosis. Despite a few reports on SMI use in assessing nailfold capillaries, there is still a need for more evidence of its value and possibilities for its standardisation.

Objectives:The aim of this study was to determine the relationship between disease activity in adult patients with dermatomyositis (DM) and other biomarkers of disease activity such as C-reactive protein creatinine kinase and nailfold video capillaroscopy (NVC).Methods:We performed a prospective single center study of 15 adult patients with DM. Study participants underwent two assessments at least 9 months apart including clinical, laboratory and NVC evaluations. Patients received immunosuppressive medications for their dermatomyositis, and ongoing disease activity was measured by the Myositis Intention to Treat Index (MITAX). NVC evaluation included assessment of capillary density, capillary apical diameter (mm), and the number of microhemorrhages per digit.Results:Microvascular abnormalities were present in most DM patients. Of these, capillary density (4.71 vs. 6.84,p= 0.006) and mean apical diameter (56.09 vs. 27.79 μm,p= 0.003) significantly improved over the study period in concordance with improving disease control (MITAX 8.53 vs. 2.64,p= 0.002). Longitudinal analysis demonstrated that capillary density was independently associated with MITAX (β = −1.49 [CI −2.49, −0.33],p= 0.013), but not other parameters such as C-reactive protein and creatinine kinase.Conclusions:Nailfold capillary density is a dynamic marker of global disease activity in adult DM. NVC may be utilized as a non-invasive point-of-care tool to monitor disease activity and inform treatment decisions in patients with DM.