Clinical and capillaroscopic findings in patients with liver disease and proximal apparent leukonychia (Terry nails and its variants).

ABSTRACT: Terry nails and Lindsay nails are similar forms of proximal apparent leukonychia (PAL). A change in nail bed vascularity is thought to be responsible for PAL. The study was aimed at investigating the frequency of PAL in patients attending a liver disease clinic, the factors associated with its presence, its value for detecting cirrhosis, its prognostic value for mortality, and associated capillaroscopic findings.A total of 521 patients were included (age range, 18-94 years; 69% men). Systematic nail photographs were evaluated by 2 independent investigators. Disease-related data were obtained from the medical records. Mortality was evaluated after 7 years of follow-up. Nailfold capillaroscopy was performed on a subset of 80 patients.PAL was present in 228 patients (43.8%; Terry nails in 205, Lindsay nails in 20, and both in 3). The kappa-coefficient of interobserver agreement was 0.82. The presence of PAL was associated with cirrhosis and, accordingly, with portal hypertension and hepatocellular dysfunction. The positive likelihood ratio of PAL for the diagnosis of cirrhosis was 1.6 (95% CI 1.3-1.92). PAL was independently associated with chronic alcohol abuse and was not a significant predictor of mortality. Venous loop dilatation and prominence of the venous plexus were observed on capillaroscopy in patients with cirrhosis but were not significantly associated with PAL.In summary, PAL is a common finding in patients from a liver clinic; it is associated with liver cirrhosis and with alcohol abuse. PAL is not associated with specific capillaroscopic findings. We propose the generic term proximal apparent leukonychia instead of classic eponymous titles to avoid confusion in the literature.

Reporting items for capillaroscopy in clinical research on musculoskeletal diseases: a systematic review and international Delphi consensus.

OBJECTIVES: The level of detail included when describing nailfold videocapillaroscopy (NVC) methods varies among research studies, making interpretation and comparison of results challenging. The overarching objective of the present study was to seek consensus on the reporting standards in NVC methodology for clinical research in rheumatic diseases and to propose a pragmatic reporting checklist. METHODS: Based on the items derived from a systematic review focused on this topic, a three-step web-based Delphi consensus on minimum reporting standards in NVC was performed among members of the European League against Rheumatism (EULAR) Study Group on Microcirculation in Rheumatic Diseases and the Scleroderma Clinical Trials Consortium. RESULTS: A total of 319 articles were selected by the systematic review, and 46 items were proposed in the Delphi process. This Delphi exercise was completed by 80 participants from 31 countries, including Australia and countries within Asia, Europe, North America and South America. Agreement was reached on items covering three main areas: patient preparation before NVC (15 items), device description (5 items) and examination details (13 items). CONCLUSION: Based on the available evidence, the description of NVC methods was highly heterogeneous in the identified studies and differed markedly on several items. A reporting checklist of 33 items, based on practical suggestions made (using a Delphi process) by international participants, has been developed to provide guidance to improve and standardize the NVC methodology to be applied in future clinical research studies.

Analysis of the first 5 years of an interdisciplinary Rheumatology-Dermatology clinic.

BACKGROUND: Multispeciality clinics, such as combined psoriasis-psoriatic arthritis clinics, have shown improved outcomes in various diseases. At Massachusetts General Hospital, we are entering our ninth year of having an interdisciplinary Rheumatology-Dermatology (R-D) clinic. AIM: To evaluate the contribution of an R-D clinic by comparing care of patients pre- and post-evaluation in the combined clinic. As proxies of care, rates and comprehensiveness of evaluations (capillaroscopic examination, skin and joint examination) were compared between the combined clinic and standard Rheumatology or Dermatology clinic. METHODS: This was a retrospective chart review of patients at the R-D clinic in Massachusetts General Hospital during the period November 2012 to December 2017. RESULTS: Prior to the patients visiting the R-D only 5% of capillaroscopic examinations were documented, only 5% of rheumatologists specifically described a rash even when present, and pruritus was documented in only 6% of rheumatology notes. By contrast, in the R-D clinic, capillaroscopic, skin and joint examinations were documented in 100% of visits, and 19% of patients were given a different or a refined diagnosis. Although all our patients had cutaneous manifestations of their disease (hair loss, rash, itch, Raynaud phenomenon, ulcerations, calcinosis) only 34% had seen a dermatologist prior to the combined clinic and only 5% of those had had their concerns addressed by the rheumatologist. This suggests that 95% had a more complete evaluation and management of all aspects of their disease by attendance at the R-D clinic. CONCLUSION: Despite this study being limited by its retrospective nature, we found that it is an efficient model to achieve more comprehensive and potentially lower medication costs. Collaboration between dermatologists and rheumatologists in a combined clinic led to more complete skin and joint examinations, consistent tracking of capillaroscopic examination, better description of rash and improved management. Having this clinic helped in reaching a diagnosis and overall better disease control and outcome.

Capilaroscopia periungueal en el Grupo Español de Enfermedades Autoinmunes Sistémicas (GEAS). Resultados de una encuesta electrónica / Nailfold capillaroscopy in the Spanish Group of Systemic Autoimmune Diseases (GEAS). Results of an electronic survey

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Reproducibility of capillaroscopic classifications of systemic sclerosis: results from the SCLEROCAP study.

Objectives: Subgroups of capillaroscopic scleroderma landscape have been correlated with stages of SSc: two groups for Maricq's classification (slow and active), and three for Cutolo's classification (early, active and late). We report inter- and intra-observer agreement for these classifications as a preliminary step in the multicentre prospective SCLEROCAP study, which aims to assess the classification and single capillaroscopic items as prognostic tools for SSc. Methods: SCLEROCAP included 385 patients. Agreement was studied in the first 100 patients, who were independently rated twice by two observers, blind to patients' characteristics; 30 of the patients were rated once by six observers. After consensus meetings, these ratings were held again. Kappa and intraclass correlation coefficients were used to assess agreement. Results: Interobserver agreement on 100 patients was moderate for Maricq and Cutolo classifications [κ 0.47 (0.28, 0.66) and 0.49 (0.33, 0.65), respectively], and became substantial after consensus meetings [0.64 (0.50, 0.77) and 0.69 (0.56, 0.81)]. Intra-observer agreement between two observers was moderate to substantial: κ 0.54 (0.33, 0.75) and 0.70 (0.57, 0.83) for Maricq's classification; 0.57 (0.38, 0.77) and 0.76 (0.65, 0.87) for Cutolo's. Thirty patients were rated once by each of six observers, and agreement was moderate to substantial: κ 0.57 ± 0.10 (Maricq) and 0.61 ± 0.12 (Cutolo). Agreement was substantial for bushy, giant capillaries and microhaemorrhages, moderate for capillary density and low for oedema, disorganization and avascular areas. Conclusion: The moderate reproducibility of Maricq and Cutolo classifications might hamper their prognostic value in SSc patients. Consensus meetings improve reliability, a prerequisite for better prognostic performances. A focus on giant capillaries, haemorrhages and capillary density might be more reliable.

Reproducibility of the scleroderma pattern assessed by wide-field capillaroscopy in subjects suffering from Raynaud's phenomenon.

Objectives: The aim of this work was to study inter- and intra-observer agreement for the diagnosis of scleroderma pattern by wide-field capillaroscopy. Methods: Images were taken from 50 patients known to have SSc and 50 controls consulting for RP who did not have SSc. These images were rated simultaneously by 11 experienced vascular medicine physicians as scleroderma pattern or not. Two weeks later, 7 of the 11 observers again rated the same images. Results: Inter-observer agreement was almost perfect between the 11 observers (κ 0.86 ± 0.01), and the proportion of concordant observations was 79% (70-87). When each observer was compared with the reference, agreement was also almost perfect: κ coefficient 0.92 ± 0.03 and proportion of concordant observations 79% (70-87). Intra-observer agreement was also almost perfect: median κ coefficient 0.94 (0.78-0.96) and median proportion of concordant observations 97% (89-98). Conclusion: Excellent inter- and intra-observer agreement was obtained in experienced vascular physicians for the diagnosis of capillaroscopic landscape by wide-field nailfold capillary microscopy.

Clinical usefulness of videocapillaroscopy and selected endothelial cell activation markers in people with Type 1 diabetes mellitus complicated by microangiopathy.

PURPOSE: Microvascular abnormalities are one of the most important causes of persistent diabetic complications. The aim of our study was to compare microvascular changes examined by nailfold videocapillaroscopy (NVC) examination with serum concentrations of vascular endothelial growth factor (VEGF), soluble thrombomodulin (sTM) and endothelin-1 (ET-1) in people with Type 1 diabetes with and without microangiopathy. MATERIAL/METHODS: The study included 106 people with Type 1 diabetes and 40 healthy controls. All participants were evaluated by extensive clinical, laboratory and capillaroscopic studies. NVC was performed using a stereomicroscope SZ 4045 (Olympus, Germany). The intensity of morphological changes was graded from 0 to 3. Serum levels of VEGF, sTM and ET-1 were determined by an enzyme-linked immunosorbent assay (ELISA). RESULTS: Morphological changes were observed by NVC in 86 out of 106 (81%) people with Type 1 diabetes mellitus. Severe capillaroscopic changes (score 3) were seen in 32 out of 54 (59%) people with microangiopathy, but in only seven out of 52 (13%) individuals without microangiopathy. Higher serum concentration of VEGF (p<0.001), ET-1 (p<0.001) and sTM (p<0.05) were demonstrated in people with diabetes complicated with microangiopathy compared to healthy controls. Moreover, comparison between people with and without microangiopathic complications showed a significantly higher capillaroscopic score and sTM serum concentration in the group with retinopathy (p<0.001) nephropathy (p<0.001) and neuropathy (p<0.01). CONCLUSIONS: Our results suggest that abnormalities in NVC may reflect the extent of microvascular involvement and associated with higher VEGF, sTM and ET-1 serum levels, as well as with microangiopathic complications in diabetic people.

[Gender-specific differences in capillaroscopy in healthy persons and patients with inflammatory rheumatic diseases]. / Geschlechtsspezifische Unterschiede bei Gesunden und Patienten mit entzündlich-rheumatischen Erkrankungen in der Kapillarmikroskopie.

BACKGROUND: Capillaroscopy is an established tool to assess morphological and functional findings of the microcirculation which have a confirmed association with disease activity and damage of inflammatory rheumatic diseases, especially systemic sclerosis. Hairpin-shaped capillaries with normal density predict a very low risk for collagenosis. METHODS: Own databases were evaluated with respect to the topic of the manuscript and the current literature was evaluated. RESULTS: The healthy population does not only demonstrate hairpin shape as capillaroscopic normal findings and morphological and functional abnormalities can also be found which show various patterns depending on gender and age. These can be found in healthy persons and also patients with collagenosis. Ectasia and sludge phenomenon are more common in women and tortuous capillaries are more common in men. Capillary filling is often decreased in women and increased in male patients. CONCLUSION: When assessing capillaroscopy findings, gender and the morphological or functional alterations which can be found in the normal healthy population should be taken into consideration. In further studies with capillaroscopy as the target parameter, the potential source of the disturbance should be known, especially in small populations, and if necessary balanced by weighted randomization.

[Capillaroscopy in rheumatological practice--one center experience]. / Kapilaroskopija u reumatoloskoj praksi--iskustvo jednog centra.

Capillaroscopy is a method for evaluating morphological characteristics of nailfold capillaries. The simplicity, noninvasiveness and easiness-to-perform make the method accessible in everyday rheumatological practice. Raynaud's phenomenon is the main indication for performing capillaroscopy (differentiating between primary and secondary Raynaud's phenomenon) and diagnosing early stages of systemic sclerosis. According to some authors capillaroscopy should be included in the work-up algorithm for patients with puffy fingers and Raynaud's phenomenon. Other autoimmune conditions (systemic lupus erythematosus, polymyositis/dermatomyositis, mixed connective tissue disease, antiphospholipid syndrome and other diseases which affect microvasculature - diabetes mellitus, thromboangiitis obliterans) can have some abnormalities of the capillaroscopic pattern. We present the results of the capillaroscopies performed in our center during the period of one year.

Nailfold capillaroscopy in systemic sclerosis: data from the EULAR scleroderma trials and research (EUSTAR) database.

OBJECTIVE: The aims of this study were to obtain cross-sectional data on capillaroscopy in an international multi-center cohort of Systemic Sclerosis (SSc) and to investigate the frequency of the capillaroscopic patterns and their disease-phenotype associations. METHODS: Data collected between June 2004 and October 2011 in the EULAR Scleroderma Trials and Research (EUSTAR) registry were examined. Patients' profiles based on clinical and laboratory data were obtained by cluster analysis and the association between profiles and capillaroscopy was investigated by multinomial logistic regression. RESULTS: 62 of the 110 EUSTAR centers entered data on capillaroscopy in the EUSTAR database. 376 of the 2754 patients (13.65%) were classified as scleroderma pattern absent, but non-specific capillary abnormalities were noted in 55.48% of the cases. Four major patients' profiles were identified characterized by a progressive severity for skin involvement, as well as an increased number of systemic manifestations. The "early" and "active" scleroderma patterns were generally observed in patients with mild/moderate skin involvement and a low number of disease manifestations, while the "late" scleroderma pattern was found more frequently in the more severe forms of the disease. CONCLUSION: These data indicate the importance of capillaroscopy in SSc management and that capillaroscopic patterns are directly related to the extent of organ involvement.

Computerized nailfold video capillaroscopy--a new tool for assessment of Raynaud's phenomenon.

OBJECTIVE: To develop a computer based nailfold video capillaroscopy system with enhanced image quality and to assess its disease-subgroup resolving power in patients with primary and secondary Raynaud's phenomenon (RP). METHODS: Using frame registration software, digitized video images from the microscope were combined to form a panoramic mosaic of the nailfold. Capillary dimensions (apex, arterial, venous, and total width) and density were measured onscreen. Significantly, the new system could guarantee analysis of the same set of capillaries by 2 observers. Forty-eight healthy control subjects, 21 patients with primary RP, 40 patients with limited cutaneous systemic sclerosis (lcSSc), and 11 patients with diffuse cutaneous SSc (dcSSc) were studied. Intra- and interobserver variability were calculated in a subset of 30 subjects. RESULTS: The number of loops/mm was significantly lower, and all 4 capillary dimensions significantly greater, in SSc patients versus controls plus primary RP patients (p < 0.001 for all measures). When comparing control (+ primary RP) patients with SSc patients (lcSSc + dcSSc) the most powerful discriminator was found to be the number of loops/mm. Results for intra- and interobserver reproducibility showed that the limits of agreement were closer when both observers measured the same capillaries. CONCLUSION: The key feature of the newly developed system is that it improves reproducibility of nailfold capillary measurements by allowing reidentification of the same capillaries by different observers. By allowing access to previous measurements, the new system should improve reliability in longitudinal studies, and therefore has the potential of being a valuable outcome measure of microvessel disease/involvement in clinical trials of scleroderma spectrum disorders.

[The evaluation by video capillaroscopy of the efficacy of a Ginkgo biloba extract with L-arginine and magnesium in the treatment of trophic lesions in patients with stage-IV chronic obliterating arteriopathy]. / Valutazione mediante videocapillaroscopia dell'efficacia dell'estratto di Ginkgo biloba con L-arginina e magnesio nel trattamento delle lesioni trofiche nei pazienti con AOC IV stadio.

BACKGROUND AND AIMS: This study aimed to evaluate the influence of Ginkgo biloba extract with arginine and magnesium used for the treatment of trophic lesions in the lower limbs caused by both diabetic and non-diabetic microangiopathy. METHODS: A comparative study was carried out in 20 patients who were divided into two groups: 10 were treated with ASA plus Ginkgo biloba extract with arginine and magnesium and 10 with ASA plus conventional hemorheology. The observation time was extended to 6 months, taking into consideration patients with trophic lesions to the lower limbs suffering from diabetic and non-diabetic peripheral arterial occlusive disease. The evaluation was performed by clinical, ultrasonographic and perilesional video capillaroscopy monitoring. Ultrasonographic and video capillaroscopy instrumental methods were used because they provide a full picture of macro and microcirculatory conditions around lesions. RESULTS: The study showed the undoubted efficacy of Ginkgo biloba extract with magnesium and arginine in relation to the following points: reduced healing times for the trophic lesion compared to the control group, improved painful symptoms, increased perilesional neoangiogenesis. No significant differences were observed from a Doppler ultrasonographic point of view or with regard to the claudication free interval. CONCLUSIONS: Ginkgo biloba extract with arginine and magnesium can improve the dynamics of cutaneous trophism in lesions caused by diabetic and non-diabetic microangiopathy.