Nailfold Videocapillaroscopy Findings Research Articles

P149 Association between nailfold videocapillaroscopy findings and sKL-6 levels in patients with idiopathic inflammatory myopathies-related interstitial lung disease

Abstract Background/Aims Idiopathic inflammatory myopathies (IIM) are a heterogeneous group of acquired muscle diseases with distinct clinical, pathological and histological features. Interstitial lung disease (ILD) is a frequent pulmonary manifestation in IIM (IIM-ILD) and considerably influences morbidity and mortality. Krebs von den Lungen 6 (sKL-6) has been proposed as a potential biomarker reflecting the severity of ILD in connective tissue diseases. Raynaud’s phenomenon is very frequent and the presence of microvascular changes in IIM have been described however, the role of nailfold videocapillaroscopy (NVC) in diagnosis and prognosis in IIM is not clearly established. We aim to determine if there is any association between NVC findings, sKL-6 levels and pulmonary involvement in patients with inflammatory myopathies. Methods We performed a retrospective study of IIM patients followed in a reference center and compared them according to the presence of ILD. Epidemiological, clinical and immunological data, pulmonary function tests (forced vital capacity and diffusing capacity for carbon monoxide), sKL-6 levels and NVC finding were retrieved. Statistical analysis was performed by T-test and Fisher’s exact test to compare qualitative and/or quantitative variables and multiple logistic regression modelling to identify correlation between pulmonary function tests, NVC findings and sKL-6 levels. Results 95 patients were included, 47 patients (49%) with ILD. 34% were male with a median age at inclusion of 55.3±24 years. Avascular areas and capillary lossshowed a significant association with the presence of ILD (OR 2.43, 95% CI 1.3-5.7, p 0.004) and (OR 1.7, 95% CI 1.48-3.1, p 0.04). A negative correlation between capillary loss and enlarged capillaries was also found with FVC% (β=-0.46, p 0.001 and β=-0.57, p < 0.0001) and DLCO% (β=-0.32, p 0.04 and β=- 0.23, p 0.03), respectively. When we studied the correlation between sKL-6 levels, positive correlations with the presence of ILD (β = 0.77, p 0.0004), the presence of hemorrhages (β = 0.21, p 0.04) and avascular areas in NVC (β = 0.64, p 0.03) and negative correlations with FVC% (β=-0.47, p 0.001) and DLCO% (β=-0.59, p, 0.005) were found. Male sex, respiratory symptoms, %FVC and %DLCO, sKL-6 levels, anti-Jo1 positivity and the presence of avascular areas and enlarged capillaries in NVC were identified as IIM-ILD predictors (R2=0.974, p = 0.006). Conclusion Capillary loss and avascular areas showed a significant association with the presence of ILD, worse FVC and DLCO values and sKL-6 levels. We identified nine predictors for developing ILD in IIM. NVC assessment and sKL-6 levels can have a predictive role for studying pulmonary function and assessing the prognosis of IIM-ILD. Disclosure C. Sieiro Santos: None. J. Tandaipan: None. D. Castillo: None. L. Martínez Martínez: None. H. Codes: None. L. Sainz: None. B. Magallares: None. P. Moya: None. H. Corominas: None. E. Díez Álvarez: None. I. Castellvi: None.

Microvascular status in juvenile Sjögren’s disease: the first nailfold videocapillaroscopy investigation

IntroductionJuvenile Sjögren’s disease (jSjD) is a rare autoimmune disease characterized by exocrine gland involvement and systemic manifestations, including small vessel vasculitis and Raynaud’s phenomenon (RP). We aimed to investigate the microvascular status in jSjD patients by nailfold videocapillaroscopy (NVC) and the potential correlations with clinical and serological features.MethodsClinical data from thirteen consecutive jSjD patients (11 females and 2 males), with a mean age of 16 ± 4 years, diagnosed before 16 years of age (mean age at diagnosis 12 ± 3) according to the 2016 American College of Rheumatology/EULAR criteria for adult SjD, were collected including age- and sex-matched healthy controls (HCs). Clinical, laboratory, and instrumental data were collected, together with NVC examination. Non-specific and specific NVC parameters were investigated, such as capillary density, capillary dilations, giant capillaries, microhaemorrhages and abnormal shapes. Associations between NVC findings and clinical/serological features were explored and analysed using parametrical and non-parametrical tests.ResultsCapillary density reduction correlated significantly with articular involvement (arthralgias) (p = 0.024). Microhaemorrhages correlated with lower C3 levels (p = 0.034). No specific NVC pattern for jSjD was identified, whereas abnormal capillary shapes were significantly higher in jSjD patients than HCs (p = 0.005). NVC abnormalities were not associated with SjD-specific instrumental tests (biopsy, imaging, Schirmer’s test). RP was present in 8% of jSjD patients.ConclusionsThe reduction of capillary density, as well as microhaemorrhages at NVC analysis, are significantly associated with some clinical aspects like articular involvement and serum biomarkers (C3 reduction). The NVC is suggested as safe and further analysis in jSjD patients.Key Points• Juvenile Sjögren’s disease (jSjD) exhibits a higher rate of abnormal shapes at nailfold videocapillaroscopy (NVC) compared to matched healthy controls.• Articular involvement in jSjD is significantly associated with a lower capillary number at NVC.• Microhaemorrhages on NVC are associated with lower C3 levels, prompting this finding as a putative novel negative biomarker/prognostic factor for jSjD.

Early improvement of nailfold videocapillaroscopy abnormalities in dermatomyositis patients with anti‐NXP‐2 antibody

AbstractThis study aimed to evaluate long‐term changes in nailfold videocapillaroscopy (NVC) findings in dermatomyositis patients with antinuclear matrix protein 2 (NXP‐2) antibody (Ab). All four patients with anti‐NXP‐2 Ab presented irregularly enlarged and reduced capillaries and hemorrhages at the initial assessment. After disease stabilization, irregularly enlarged capillaries and hemorrhages disappeared within the mean observation period of 6 months. These early improvements were not observed in patients with anti‐TIF1 Ab. The results of this study show that long‐term changes in NVC findings should be assessed using myositis‐specific Ab information.

Specific Autoantibodies and Microvascular Damage Progression Assessed by Nailfold Videocapillaroscopy in Systemic Sclerosis: Are There Peculiar Associations? An Update

Specific autoantibodies and nailfold videocapillaroscopy (NVC) findings are serum and morphological diagnostic hallmarks of systemic sclerosis (SSc) as well as useful biomarkers which stratify the microvascular progression and prognosis of patients. The aim of our narrative review is to provide an update and overview of the link between SSc-related autoantibodies, used in clinical practice, and microvascular damage, evaluated by NVC, by exploring the interaction between these players in published studies. A narrative review was conducted by searching relevant keywords related to this field in Pubmed, Medline and EULAR/ACR conference abstracts with a focus on the findings published in the last 5 years. Our search yielded 13 clinical studies and 10 pre-clinical studies. Most of the clinical studies (8/13, 61.5%) reported a significant association between SSc-related autoantibodies and NVC patterns: more specifically anti-centromere autoantibodies (ACA) were associated more often with an "Early" NVC pattern, whereas anti-topoisomerase autoantibodies (ATA) more frequently showed an "Active" or "Late" NVC pattern. Five studies, instead, did not find a significant association between specific autoantibodies and NVC findings. Among the pre-clinical studies, SSc-related autoantibodies showed different mechanisms of damage towards both endothelial cells, fibroblasts and smooth muscle vascular cells. The clinical and laboratory evidence on SSc-related autoantibodies and microvascular damage shows that these players are interconnected. Further clinical and demographic factors (e.g., age, sex, disease duration, treatment and comorbidities) might play an additional role in the SSc-related microvascular injury whose progression appears to be complex and multifactorial.

Microvascular capillaroscopic abnormalities and occurrence of antinuclear autoantibodies in patients with sarcoidosis

We described nailfold videocapillaroscopy (NVC) findings and estimated the prevalence of serum anti-nuclear (ANA) and extractable nuclear antigen autoantibodies (ENA) in a cohort of sarcoidosis patients, comparing them with adequate healthy controls (HCs) and with primary Raynaud’s phenomenon patients (PRPs). NVC findings were also correlated with the occurrence of autoantibodies, current treatment, laboratory parameters, variables of lung function and whole-body imaging data. Twenty-six patients with sarcoidosis were assessed through NVC, laboratory parameters, pulmonary function tests, chest-X ray and 18- fluorodeoxyglucose positron emission tomography/computed tomography. The NVC parameters and ANA/ENA dosage were recorded also in 30 PRPs and 30 HCs. Sarcoidosis patients showed a higher rate of capillary dilations and nonspecific abnormalities and a lower mean capillary absolute number than PRPs and HCs (p < 0.01 for all comparisons). The prevalence of ANA positivity was higher in patients with sarcoidosis compared with PRPs and HCs (p < 0.02 for both), whereas ENA positivity was detected in one sarcoidosis patient (Ro52). Among sarcoidosis patients, the mean capillary absolute number negatively correlated with the C-reactive protein concentrations and was positively associated with the forced vital capacity percentage. Instead, a negative correlation was detected between serum ACE levels and the presence of capillary dilations (all p < 0.05). Our findings suggest a microvascular involvement in sarcoidosis whose investigation by NVC might be useful for the follow-up of patients displaying RP. Autoantibody positivity in sarcoidosis might suggest autoimmune implications in the disease or the production of autoantibodies reactive to tissue damage.

Examination of nailfold videocapillaroscopy findings in ANCA-associated vasculitis.

The objective of this study was to evaluate nailfold videocapillaroscopy (NVC) as a useful tool for assessing the disease activity of ANCA-associated vasculitis (AAV). This study enrolled 51 patients with AAV and 21 healthy controls. We scored NVC findings semiquantitatively, and compared them between AAV patients and controls. We examined the association of NVC findings with disease activity indicators, histopathological findings of skin biopsies, and high-resolution CT (HRCT) scores in AAV. Additionally, we repeatedly rated the NVC findings 3 months after immunosuppressive therapy. Of the 51 enrolled patients, 36 (70.6%) showed a microangiopathy pattern and 4 (7.8%) showed a scleroderma pattern in AAV. The scores for microhaemorrhage, capillary loss, neoangiogenesis, and tortuosity were significantly higher in the AAV group than in the control group. NVC abnormalities correlated with the severity of skin, lung and kidney involvement. The scores of giant capillaries significantly correlated with the total BVAS and the chest BVAS; the scores of capillary loss correlated with the chest BVAS and the renal BVAS. The scores of microhaemorrhage significantly correlated with perivascular inflammatory cell infiltrations in the upper dermis of the purpura and tended to correlate with the total ground-glass opacity and consolidation scores on HRCT. In addition, capillary loss scores had a significant positive correlation with serum creatinine levels. Additionally, the microhaemorrhage scores were significantly reduced after 3 months of immunosuppressive therapy. In AAV patients, NVC abnormalities are significantly associated with disease severity. This result suggests that NVC is a useful tool for assessing the disease activity and treatment response in AAV.

Peripheral Microangiopathy Changes in Pulmonary Arterial Hypertension Related to Systemic Sclerosis: Data From a Multicenter Observational Study

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.

Capillaroscopic analysis of the microvascular status in mixed versus undifferentiated connective tissue disease.

Raynaud phenomenon (RP), typically, precede the clinical onset of systemic manifestations in several connective tissue diseases (CTDs). These autoimmune disorders usually share a microvascular damage whose alterations can be detected by nailfold videocapillaroscopy (NVC). The aim of the study was to compare the NVC microvascular status in Mixed Connective Tissue Disease (MCTD) versus the Undifferentiated Connective Tissue Disease (UCTD), and to search correlations between NVC findings and specific autoantibodies in UCTD patients. Clinical data and NCV patterns were retrospectively obtained from the files of 46 MCTD patients, 47 stable UCTD patients and 51 individuals with primary RP (PRP) as controls collected in a central database (VideoCap®, DS Medica, Milan, Italy). ANA and ENA Abs were tested respectively by indirect immunofluorescence and enzyme-linked immunosorbent assay. "Scleroderma-like" (SSc-like) NVC pattern was significantly more frequent in MCTD than in UCTD patients (48% vs 11%, p<0.001). Giant capillaries, abnormal shapes (i.e. neoangiogenesis) and lower capillary density were predominantly detected among MCTD versus UCTD patients (48% vs 11%, 49% vs 13%, 52% vs 9%, respectively, p<0.001). The absolute number of capillaries was significantly lower in MCTD versus UCTD patients (mean 7±1.7 SD vs mean 9.2±1.3 SD, respectively, p<0.001). Fully normal NVC pattern and non-specific NVC alterations were respectively observed in 6% and 46% of MCTD and in 6% and 83% of UCTD. Moreover, PRP patients showed normal NVC pattern and non-specific capillary abnormalities in 23% and in 77%, respectively. No statistically significant correlations were observed between NVC patterns and ANA patterns/specific ENA-Abs among the UCTD patients. The significant presence of the SSc-like NVC pattern and reduced number of capillaries seem the most typical NVC findings in MCTD in comparison to UCTD patients, suggesting a reflection of more complex and severe disease in MCTD ones.

Nailfold Videocapillaroscopy in Patients with COVID-19-associated Pneumonia in Intensive Care Units

To compare patients with COVID-19 in intensive care units (ICUs) to healthy controls using nailfold videocapillaroscopy (NVC), offering standardised findings about micro-circulation. A descriptive, cross-sectional study. Medical Intensive Care Unit, Kayseri City Education and Research Hospital, Kayseri, Turkey between January and May 2021. The NVC parameters-capillary morphology, loop diameter, capillary density, dilated capillaries, giant capillaries, avascular areas, microaneurysms, and micro-hemorrhages of 32 patients with COVID-19 and 29 controls were recorded. The most common capillary morphology in the COVID-19 group (18/32, 56.2%) was serpentine, which also characterised some (6/29, 20.7%) patients in the non-COVID-19 group (p <0.001). The median capillary loop diameter was 77.78 ± 3.63 µm in the COVID-19 group and 71.67 ± 2.19 µm in the non-COVID-19 group (p=0.030). Mean capillary density was 6.41 ± 1.21/1 mm in the COVID-19 group and 8.55 ± 1.12/1 mm in the non-COVID-19 group (p <0.001). The COVID-19 group had significantly more enlarged capillaries (p = 0.001), giant capillaries (p = 0.025), avascular areas (p = 0.028), micro-aneurysms (p <0.001), and micro-hemorrhages (p = 0.011). Mean capillary density was 5.50 ± 0.19/1 mm among deceased patients with COVID-19, but 6.71 ± 0.25/1 mm among survivors (p = 0.011). NVC findings differed between patients with COVID-19 and controls, and capillary density was less among deceased patients with COVID-19 than survivors. Capillaries, COVID-19, İntensive care unit, Micro-circulation, Nailfold videocapillaroscopy.

The effect of vitamin D3 and thyroid hormones on the capillaroscopy-confirmed microangiopathy in pediatric patients with a suspicion of systemic connective tissue disease-a single-center experience with Raynaud phenomenon.

Raynaud phenomenon (RP) may be the first manifestation of a systemic connective tissue disease (SCTD). Early detection of dysfunction of small vessels called microangiopathy is essential for the diagnostic process. The focus of this single-center, retrospective study was to investigate the potential dependencies between microvascular image and laboratory markers measured in children with RP. The study analyzed the nail-fold video-capillaroscopy (NVC) findings and laboratory results of 81 children between the ages 6 and 17 who were referred to pediatric rheumatologist with a suspicion of SCTD. Out of 52 patients presenting with RP at the time of evaluation, abnormalities in capillary microscopic imaging were found in 34. NVC findings were then compared to levels of specific biomarkers in serum. Vitamin D3 serum levels have been significantly decreased in patients with RP (23.4 ng/mL ± 8.76 vs. 30.0 ng/mL ± 12.66, P = 0.0148). There were positive significant correlations between levels of vitamin D3 and acute-phase reactants in serum, such as C-reactive protein (P = 0.0292). Furthermore, free thyroxine levels (fT4) in patients with both RP (P = 0.0126) and micro-angiopathy (P = 0.05496) persisted in the lower range of the normal limit (< 1.0 ng/dL). Regular oral supplementation of vitamin D3 should be always considered in children with RP if deficiency is found. Additionally, low fT4 level (< 1.0 ng/dL) should be considered as an indication to perform NVC in patients suspected of SCTD even when they do not present RP.

Nailfold capillaroscopy : a comprehensive review on common findings and clinical usefulness in non-rheumatic 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.

The comparison of nailfold videocapillaroscopy findings between anti-melanoma differentiation-associated gene 5 antibody and anti-aminoacyl tRNA synthetase antibody in patients with dermatomyositis complicated by interstitial lung disease

Dermatomyositis (DM) is frequently complicated by interstitial lung disease (ILD), which increases mortality. This study aims to elucidate the clinical significance of nailfold videocapillaroscopy (NVC) on assessing the disease activity and prognosis of DM-ILD. We compared the NVC findings between anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody-positive and anti-aminoacyl tRNA synthetase (anti-ARS) antibody-positive patients, the survival and ILD-related death groups, and examined the association of NVC findings with prognostic factors of DM-ILD. The median scores of microhemorrhage and capillary disorganization in the anti-MDA5 antibody-positive group were significantly higher than those in the anti-ARS antibody-positive group (P = 0.012 and 0.044, respectively). In contrast, the median scores of tortuous capillaries in the anti-ARS antibody-positive group were significantly higher than those in the anti-MDA5 antibody-positive group (P = 0.002). The median scores of microhemorrhage was significantly higher in the ILD-related death group than the survival group (P = 0.02). The scores of microhemorrhage, capillary disorganization, and neoangiogenesis correlated with known poor prognosis factors of DM-ILD. Additionally, the scores of microhemorrhage and capillary loss correlated significantly with the total fibrosis scores of chest high-resolution computed tomography. These findings suggest that NVC is a useful tool for assessing the disease activity and prognosis of DM-ILD.

Association between coronary artery disease severity and videocapillaroscopic findings of nail fold capillary circulation

Aim: Endothelial dysfunction plays a significant role in the etiology of coronary artery disease (CAD), which develops due to atherosclerosis, and is a major cause of morbidity and mortality. Nail fold videocapillaroscopy (NVC) assessment is a surrogate marker of endothelial dysfunction. To the best of our knowledge, the relationship between the microcirculation features on NVC and the severity of coronary atherosclerosis has not previously been investigated. In this study, we aimed to investigate the relationship between severity of coronary atherosclerosis (shown by SYNTAX and Gensini scores) and NVC findings which is an indirect indicator of systemic endothelial damage.Methods: The study was designed as a retrospective cohort. One-hundred consecutive patients who underwent coronary angiography (CAG) and had at least one lesion in the epicardial coronary arteries, narrowing the lumen by more than 50%, were included. Coronary angiographic images of the patients were evaluated by two experienced interventional cardiologists and SYNTAX and Gensini scores were calculated. Patients were divided into three groups according to the SYNTAX scores (SYNTAX score ≤22, between 23-32, and ≥33) and two groups according to Gensini scores (Gensini score ≥30 and ˂30). The NVC examinations were performed by a rheumatologist who was experienced in this field, and the reduction in capillary density, presence of dilated capillaries, giant capillary, microhemorrhage, branching, disorganization, tortuosity, avascular area, extravasation, and neoangiogenesis were investigated.Results: Dilated capillary vessels were more common in diabetic patients (85%) than in non-diabetics (66.7%) (P=0.041). Besides, there was no significant difference in NVC parameters between patients with and without diabetes mellitus (P>0.05 for all), with and without hypertension (P>0.05 for all). There was no significant difference between the SYNTAX groups in terms of NVC findings (P>0.05 for all). The NVC findings were similar between Gensini groups (P>0.05 for all). Conclusion: The severity of atherosclerosis surrogated by SYNTAX and Gensini scores was not related to the NVC findings. Therefore, NVC is inappropriate for determining the severity of CAD.

Nailfold Videocapillaroscopy Changes Are Associated With the Presence and Severity of Systemic Sclerosis-Related Interstitial Lung Disease.

The aim of this study was to evaluate the association of nailfold videocapillaroscopy (NVC) changes and the presence and severity of interstitial lung disease (ILD) in systemic sclerosis. This was a cross-sectional analysis of 48 systemic sclerosis patients (21 patients with ILD). The NVC characteristics considered were capillary organization, capillary loss (CL), avascular areas, enlarged and giant capillaries, hemorrhages, abnormally shaped capillaries, edema, and intermittent flux.We analyzed the association between NVC findings and (1) presence and extension of ILD and (2) percent predicted of forced vital capacity (FVC) and the carbon monoxide diffusing capacity (DLCO). Capillary loss and avascular areas showed a significant association with the presence of ILD (odds ratio, 18.57; 95% confidence interval [CI], 2.17-158.72 [p = 0.008]; and odds ratio, 4.64; 95% CI, 1.35-15.91 [p = 0.015], respectively). Receiver operating characteristic (ROC) curve analysis confirmed the association between CL and ILD (area under the ROC curve, 90.1%; 95% CI, 81.8-91.4). Avascular areas and CL were associated with a worse pulmonary function (FVC -18.1% [p = 0.034], DLCO -14.0% [p = 0.013]; and FVC -15.3% [p = 0.086], DLCO -12.3% [p = 0.049], respectively). No association was found between other NVC findings and ILD or lung function. Capillary loss and avascular area showed a significant association with the presence of ILD, supported by ROC curve analysis. These results may reinforce a prognostic role for NVC and a physiopathology mechanism for ILD based on vascular damage.

Nailfold Videocapillaroscopy as a Candidate Biomarker for Organ Involvement and Prognosis in Patients with Systemic Sclerosis.

Background:Systemic Sclerosis (SSc) is a rare, multisystemic connective tissue disease associated with significant morbidity. Early recognition of patients at risk for adverse prognosis may help towards optimized monitoring and treatment, thus improving disease outcome.Objective:To correlate nailfold videocapillaroscopy (NVC) findings (‘early’, ‘active’, ‘late’ scleroderma patterns and non-specific capillary abnormalities) with major organ involvement and prognosis in patients with systemic sclerosis (SSc).Methods:Patients from the Scleroderma cohort followed at the Rheumatology clinic of the University Hospital of Heraklion will be included. The study will include a prospective and a retrospective part. Prospective part: All newly diagnosed patients will undergo NVC at baseline and subsequently every six months. We will review demographics, clinical features and autoantibodies status. Major organ involvement will be monitored (Pulmonary Function Test, DLCO, heart echocardiogram, chest XR, modified Rodnan skin score) at baseline and then every 6–12 months. Retrospective part: Existing SSc patients with available NVC data at diagnosis will be included. We will correlate the NVC findings at the time of diagnosis with disease outcomes such as major organ involvement, end stage organ failure, need for hospitalization, and death. We will also correlate longitudinal changes of the NVC patterns with treatment responses and outcomes.

The evaluation of nailfold videocapillaroscopy findings in patients with type 2 diabetes with and without diabetic retinopathy.

OBJECTIVE:Diabetes mellitus (DM) is a chronic hyperglycemic state and is associated with microvascular structural alterations. This study aimed to investigate the diameters of capillary loops and morphostructural changes using nailfold video capillaroscopy (NVC) in patients with type 2 DM with and without diabetic retinopathy (DR).METHODS:This cross-sectional, single-center study was conducted in patients with type 2 DM who were followed in outpatient clinics of ophthalmology and internal medicine. General demographic data were collected from patients. An ophthalmologist examined all patients in terms of DR. A rheumatologist blinded to the clinical data performed NVC. The diameters of apical, arterial, and venous loop of capillaries were measured, and the microvascular changes of capillaries were scored.RESULTS:In this study, 44 patients with type 2 DM with DR (47.7% males) and 20 patients with type 2 DM without DR (55% males) were included. In our study, patients with type 2 DM with DR had more frequent capillary hemorrhage, more frequent ectasia, more frequent giant capillary, and more frequent neo-angiogenesis than patients with type 2 DM without DR. However, these findings were not statistically significant.CONCLUSION:Further controlled studies with large sample size are needed to determine the characteristic NVC findings of DR in patients with type 2 DM.

Evaluation of nailfold videocapillaroscopy in central serous chorioretinopathy.

Nailfold videocapillaroscopy (NVC) is a diagnostic tool to evaluate micro-vasculature. The presence of choroidal vasculopathy is apparent in central serous chorioretinopathy (CSCR). This study was aimed at assessing capillaroscopic nailfold findings in patients with CSCR. To the best of our knowledge, there is no study assessing NVC findings in CSCR in the literature. Sixty-one patients with CSCR who met the inclusion criteria, and 82 age- and sex-matched healthy controls were included to the study. A videocapillaroscopy device with 200× magnification was used for capillaroscopic assessment. The mean age was 48.79 ± 11.15years in the patient group (13 female, 48 male) and 49.38 ± 9.02years in the control group (17 female, 65 male). The age and gender were comparable in the patient and control groups (p = 0.727 and p = 0.933, respectively). The capillary count was found to be decreased in the patient group compared to control group. No significant correlation was found between capillary count and choroidal thickness (p = 0.551; r = -0.081). In the patient group, the frequencies of major capillaroscopic findings including capillary ectasia, aneurysm, micro-hemorrhage, avascular area, tortuosity, neo-formation, bizarre capillary, bushy capillary, meander capillary and extravasation were found to be increased in the patient group. However, no significant correlation was detected between capillaroscopic findings and disease type and presence of attacks. This is first study in which nailfold capillary assessment was performed in patients with CSCR, and we detected major capillaroscopic changes. These findings suggest that CSCR can be a systemic microvasculopathy. Further studies are needed to clarify the diagnostic and prognostic value of capillaroscopy in CSCR.

New insights into systemic sclerosis related microcirculatory dysfunction by assessment of sublingual micr\\ocirculation and vascular glycocalyx layer. Results from a preliminary study

BackgroundInitial morphological and functional markers of systemic sclerosis (SSc) are evidenced in microvascular structural damage. However, nailfold videocapillaroscopy (NVC) explores only morphological abnormalities. Sidestream Dark Field (SDF) imaging of sublingual microcirculation enables assessment of both morphological and functional capillary impairment and allows measurement of the glycocalyx layer, which is an indicator of endothelial dysfunction. ObjectiveTo describe and validate sublingual abnormalities assessed by SDF device in comparison with NVC findings and to measure the thickness of the glycocalyx layer. MethodsFrom February to May 2014, 26 subjects (16 SSc patients and 10 healthy controls) underwent standardised NVC and SDF imaging of sublingual microcirculation. Glycocalyx thickness was also measured. ResultsCapillary density and percentage of perfused vessels were significantly reduced in patients with SSc (n=13) compared to controls. Correlation between nailfold capillary density assessed by NVC and sublingual capillary density assessed by SDF was observed (r2=0.59; P=0.023). According to the NVC pattern, patients with “active” disease experienced greater reduction in capillary density than patients with “late” disease as suggested by the de Backer score (9.17±0.81 vs 10.86±1.19; P=0.03). Additionally, the decrease in glycocalyx thickness was measured in SSc patients (n=13) compared to controls (n=10) (0.41±0.03 versus 0.76±0.29 P=0.003). ConclusionOur results suggest for the first time in SSc, that sublingual microcirculation and glycocalyx are impaired and that SDF imaging findings correlate with those of NVC. Nevertheless, further studies are required for the validation of our preliminary results.

Nailfold capillaroscopic changes in dermatomyositis and polymyositis.

Inflammatory myopathies (IM) are a group of muscle diseases occurring both in children and adults. Nailfold videocapillaroscopy (NVC) alterations are described in IM, but available data are discordant, including differences between polymyositis (PM) and dermatomyositis (DM). The aim of this study was to describe the capillaroscopic differences between PM and DM patients and possible correlation with clinical and serological features. We analyzed 52 unselected patients with IM in a cross-sectional study in a 6-month period. NVC findings of 29 DM and 23 PM patients were compared with those of 52 patients with primary Raynaud's phenomenon. Tortuosities, capillary loss, enlarged and giant capillaries, microhemorrhages, and ramified capillaries were scored by a semiquantitative rating; disorganization of the vascular array, avascular areas, and scleroderma pattern were scored as presence/absence. Sex, mean age, and mean disease duration were similar in both groups. Disorganization of the vascular array, enlarged and giant capillaries, capillary loss, and scleroderma-like pattern were observed almost only in IM patients. Significant differences were observed between PM and DM with higher frequency and mean score of NVC changes in DM. In DM patients with disease duration ≤6 months (14/29 patients), capillary density was significantly reduced (P = 0.039) and giant capillaries more frequent (P = 0.027), compared with patients with longer disease duration, while a scleroderma pattern tended to be more frequent in patients with a disease duration of less than 6 months. On the contrary, no differences were observed for ramified capillaries with regard to disease duration. Capillaroscopic alterations are identified only in DM patients as expression of diffuse microangiopathy; surprisingly, more severe changes were associated with shorter disease duration, while persistence of ramified capillaries with long-standing disease.

Intrarenal Hemodynamic Parameters Correlate with Glomerular Filtration Rate and Digital Microvascular Damage in Patients with Systemic Sclerosis

To evaluate intrarenal arterial stiffness by Doppler ultrasound and examine the correlation between renal Doppler indices, glomerular filtration rate, and digital microvascular damage in systemic sclerosis patients. Thirty systemic sclerosis patients and 30 healthy controls were enrolled in this study. Doppler indices of intrarenal arterial stiffness, peak systolic flow velocity, end diastolic flow velocity, resistive index, pulsative index, and systolic/diastolic (S/D) ratio were measured on the interlobar artery of both kidneys. Glomerular filtration rate was measured using Tc(99m) diethylenetriamine pentaacetic acid (DTPA). Equation 7 from the Modification of Diet in Renal Disease was used to estimate glomerular filtration rate. Nailfold videocapillaroscopy findings were classified as early, active, and late patterns. The intrarenal arterial stiffness, evaluated by Doppler indices, was higher in systemic sclerosis patients than healthy controls. In systemic sclerosis patients pulsative index (r = -0.69), resistive index (r = -0.75), and S/D ratio (r = -0.74) showed a negative correlation with measured glomerular filtration rate (P < 0001). High correlation (P = 0008) was observed between measured and estimated glomerular filtration rate (r = 0.55). Pulsative index, resistive index, and S/D ratio significantly increased with progression of capillaroscopic damage. Conversely, measured glomerular filtration rate significantly decreased with capillaroscopic damage progression. Doppler indices of intrarenal arterial stiffness are noninvasive diagnostic tests to evaluate renal damage in SSc patients. Intrarenal arterial stiffness and glomerular filtration rate correlate with capillaroscopic microvascular damage.