Video-dermoscopic Assessment of Capillaroscopic Pattern in Hansen's Disease

Leprosy is a chronic granulomatous infectious disease with a proven role of Mycobacterium leprae invasion into endothelial cells. Animal studies have shown evidence of involvement of vasa nervorum in the process of nerve invasion. Capillaries act as the mirror image of vascular involvement in any rheumatic disorder and holds good for leprosy also. Nailfold capillaroscopy (NFC) is a non-invasive, easily reproducible technique to study proximal nailfold capillaries. The aim of this study is to investigate morphological nailfold capillaroscopic alterations in patients with leprosy in its various forms and comparison with the normal individual. Total 20 Leprosy patients and 20 normal age and sex matched individuals recruited for nailfold capillaroscopic examination using video dermoscopye. Among 20 normal individuals, 3(15%) individuals showed tortuous capillaries and microhemorrhages each, 2(10%) showed meandering vessels, 1(5%) each showed megacapillaries, dilated/ectatic capillaries and bizarre vessels. Out of 20 leprosy patients, 11 (55%) patients showed bizarre and meandering capillaries, 10(50%) showed dilated vessels and avascular areas, 9(45%) showed capillary dropouts and neovascularisation, 8(40%) showed tortuous vessels, 6(30%) haemorrhages and 4 (20%) showed megacapillaries. Findings like avascular areas, capillary dropouts, haemorrhages were more noticed in lepromatous and borderline lepromatous leprosy, whereas early capillary abnormalities like dilated, meandering, bizarre vessels and neoangiogenesis were noticed more in borderline tuberculoid leprosy. However, statistical significant difference between clinical and dermoscopic observations was not seen in this study. Further studies with a large sample size are required to find out the same. Morphological changes may denote micro-vascular invasion by Mycobacterium leprae and may act as warning signs of fore- coming complications like loss of sensation and trophic ulcers. Follow-up studies are required to understand such correlation, if any.

Characteristics of nailfold capillaroscopy examination of systemic sclerosis and its value in assessing the development of SSc / 中华风湿病学杂志

Objective:To identify the characteristics of nailfold capillaroscopy (NFC) of systemic sclerosis (SSc) and investigate whether more severe peripheral microangiopathy at NFC were related to the development of SSc.Methods:① The study included 115 patients (60 cases with SSc and 55 patients with other connective tissue diseases). All patients were treated with neither prednisone nor immunosuppressive drugs within 3 months before enrollment. We collected the following data: age, disease duration, disease onset, mRSS, high-resolution chest tomography (HRCT), echocardiography, pulmonary function, nailfold capillaroscopy and routine laboratory assessments. ② All the NFC definitions were used for semi-quantitatively scoring and Cutolo's qualitative assessment. ③ The relationship between NFC changes and joint, visceral involvement and autoantibodies in SSc patients was analyzed. ④ T test, Rank sum test and chi-square test were applied to analyze data. Results:① According to Cutoloqualitative assessment of NFC, patients of SSc with active/late pattern ( n=52) were very common than other CTD ( n=21) ( Z=-3.853, P<0.01). ② According to semiquantitative assessment, the scores of loss of capillaries [(1.67±0.60) vs (0.72±0.46), t=8.347, P<0.01)], irregular enlarged capillaries [(1.22±0.88) vs (0.74±0.50), t=3.178, P<0.01)], hemorrhage [(0.30±0.39) vs (0.10±0.21), t=3.090, P<0.01)], disorganization of the microvascular array [(0.38±0.38) vs (0.18±0.32), t=2.729, P<0.01)] were significantly higher than CTD. ③ The NFC of SSc patients was significantly different from CTD. The number of capillary loss ( Z=-4.194, P<0.01), input capillary dimensions ( t=3.704, P<0.01), output capillary dimensions ( t=3.913, P<0.01), wide diameter of capillary ( t=4.586, P<0.01), tortuous capillaries ( Z=-2.677, P<0.01), gaint capillary ( χ2=8.040, P=0.013), effusion ( Z=-2.278, P=0.023) were more increased than CTD. ④ The NFC pattern of SSc with lung involvement were mainly active and late (66%, 33/50), whereas early and active pattern (60%, 6/10) for those without respiratory system involvement ( Z=10.114, P=0.045) . The NFC pattern of SSc patients with joint involvement were mainly active and late (75%, 12/16), whereas early and active (66%, 29/44) for those without joint involvement ( Z=5.550, P=0.057) . Conclusion:The NFC of SSc patients is significantly different from CTD. NFC may be a suitable tool for disease evaluation.

A Diagnostic Roadmap for Raynaud's Phenomenon / 대한내과학회지

Raynaud's phenomenon (RP) is a reversible vasospasm that is aggravated by cold or emotional stress. Before confirming RP, it is essential to consider other possible causes including compressive neuropathy, sensori-neuropathy, thyroid disease, hematologic conditions and offending drugs. RP is typically characterized by the three-step color change that turns pallor (white), cyanosis (blue), and then erythema (red) of reperfusion. Once RP is diagnosed, it is important to determine whether it is primary or secondary RP. To distinguish primary from the secondary RP, the specialized tests performing in clinical practice are antinuclear antibody (ANA) and nailfold capillary microscopy (NFC). The combination of ANA and NFC is most helpful for discriminating secondary RP due to autoimmune rheumatic disease. Thereby, normal findings of NFC in primary RP distinguished from secondary RP should be understood. Patients with primary RP usually improves with symptomatic treatment focused on lifestyle modification and patient education, but those with secondary RP should be treated together with associated disease or causes.

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

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

Hallazgos capilaroscópicos patológicos previos al desarrollo de lupus eritematoso sistémico / Pathologic capillaroscopic findings before systemic lupus erythematous development

La capilaroscopía periungular es una técnica de estudio, de la microcirculación cutánea in vivo. Es de elección en el diagnóstico diferencial entre Raynaud primario y secundario. Presentamos la descripción de los hallazgos capilaroscópicos mediante imágenes, en una adolescente que padece fenómeno de Raynaud. Se hallan alteraciones estructurales y funcionales de los capilares. En los meses posteriores la paciente desarrolla lupus eritematoso sistémico. La presencia de estos hallazgos previos al desarrollo de enfermedad lúpica, no es frecuente.

Nailfold capillaroscopy is a technique to visualize the skin microcirculation in vivo. This is of the utmost importance to establish the differential diagnosis between primary and secondary Raynaud. We hereby present the capillaroscopic image of the alterations, both structural and functional, detected in a female adolescent with Raynaud's phenomenon. In the next months the patient developed a systemic lupus erythematosus. The presence of these prior to the development of lupus disease is rare findings.

Low vitamin D serum levels in diffuse systemic sclerosis: a correlation with worst quality of life and severe capillaroscopic findings / Baixos níveis séricos de vitamina D na esclerose sistêmica difusa: correlação com pior qualidade de vida e alterações capilaroscópicas graves

ABSTRACT Objective: The aim of this study was to analyze the correlation of vitamin D levels with clinical parameters, bone mineral density (BMD), quality of life (QoL) and nailfold capillaroscopy (NC) in patients with diffuse systemic sclerosis (SSc). Methods: Thirty-eight female patients with diffuse SSc were analyzed regarding 25-hydroxyvitamin D (25OHD) serum levels. At inclusion, organ involvement, autoantibodies, modified Rodnan skin score (mRSS), Medsger Disease Severity Index (MDSI), body mass index (BMI), BMD, NC, Short-Form-36 Questionnaire (SF-36), and Health Assessment Questionnaire (HAQ), were performed through a standardized interview, physical examination and electronic chart review. Results: Mean 25OHD serum level was 20.66 ± 8.20 ng/mL. Eleven percent of the patients had 25OHD levels ≤10 ng/mL, 50% ≤20 ng/mL and 87% ≤30 ng/mL. Vitamin D serum levels were positively correlated with BMI (r = 0.338, p = 0.038), BMD-total femur (r = 0.340, p = 0.037), BMD-femoral neck (r = 0.384, p = 0.017), SF-36-Vitality (r = 0.385, p = 0.017), SF-36-Social Function (r = 0.320, p = 0.050), SF-36-Emotional Role (r = 0.321, p = 0.049) and SF-36-Mental Health (r = 0.531, p = 0.0006) and were negatively correlated with HAQ-Reach (r = −0.328, p = 0.044) and HAQ-Grip Strength (r = −0.331, p = 0.042). A negative correlation with NC-diffuse devascularization (p = 0.029) and NC-avascular area (p = 0.033) was also observed. Conclusion: The present study provides novel evidence demonstrating that low levels of 25OHD have a negative impact in diffuse SSc QoL and further studies are needed to define whether vitamin D supplementation can improve health related QoL in these patients. The additional observation of a correlation with severe NC alterations suggests a possible role of 25OHD in the underlying SSc vascular involvement.

RESUMO Objetivo: O objetivo deste estudo foi analisar a correlação entre os níveis de vitamina D e parâmetros clínicos, densidade mineral óssea (DMO), qualidade de vida (QV) e capilaroscopia periungueal (CPU) em pacientes com esclerose sistêmica difusa (ES). Métodos: Mensuraram-se os níveis séricos de 25-hidroxivitamina D (25OHD) de 38 pacientes do sexo feminino com ES difusa. No momento da inclusão, analisaram-se o envolvimento de órgãos, autoanticorpos, escore cutâneo de Rodnan modificado (ERM), Medsger Disease Severity Index (MDSI), índice de massa corporal (IMC), DMO, CPU, Short-Form-36 Questionnaire (SF-36) e Health Assessment Questionnaire (HAQ) por meio de uma entrevista padronizada, exame físico e avaliação de prontuário eletrônico. Resultados: A média do nível sérico de 25OHD foi de 20,66 ± 8,20 ng/mL. Dos pacientes, 11% tinham níveis de 25OHD ≤ 10 ng/mL, 50% ≤ 20 ng/mL e 87% ≤ 30 ng/mL. Os níveis séricos de vitamina D estiveram positivamente correlacionados com o IMC (r = 0,338, p = 0,038), DMO-fêmur total (r = 0,340, p = 0,037), DMO-colo femoral (r = 0,384, p = 0,017), SF-36-Vitalidade (r = 0,385, p = 0,017), SF-36-Aspecto social (r = 0,320, p = 0,050), SF-36-Aspecto emocional (r = 0,321, p = 0,049) e SF-36-Saúde mental (r = 0,531, p = 0,0006) e se correlacionaram negativamente com o HAQ-Alcance (r = –0,328, p = 0,044) e HAQ-força de preensão (r = –0,331, p = 0,042). Também foi observada uma correlação negativa com a CPU- desvascularização difusa (p = 0,029) e CPU-área avascular (p = 0,033). Conclusão: O presente estudo fornece evidências novas de que níveis baixos de 25OHD têm um impacto negativo sobre a qualidade de vida de pacientes com ES difusa e que são necessários mais estudos para definir se a suplementação de vitamina D pode melhorar a qualidade de vida relacionada com a saúde desses pacientes. A observação adicional de uma correlação com alterações graves na CPU sugere um possível papel da 25OHD no envolvimento vascular subjacente da ES.

The Clinical Efficacy of Nailfold Capillaroscopy in Rheumatic Diseases / 대한내과학회지

Nailfold capillaroscopy (NFC) is a non-invasive morphological study that routinely used to differentiate between a primary and secondary Raynaud's phenomenon (RP). Secondary RP is a manifestation of an underlying rheumatic disease that occurs in 80-95% of patients with systemic sclerosis (SSc), 75% of patients with mixed connective tissue disease and in 20-25% of patients with systemic lupus erythematosus or rheumatoid arthritis. These RP frequently precedes the underlying disease by many years. The well-established roles of NFC are the early diagnosis of SSc, and potential for predicting clinical complication, as like digital ulcers. The following review will present a technique about how to perform NFC and optimal assessment with emphasis on its possible role as a reliable diagnostic tool.

Estudio de patrones capilaroscópicos en población sana / Capillarroscopic patterns in healthy population

La capilaroscopia es un método no invasivo y seguro que permite la visualización de los capilares a nivel del lecho periungueal de los dedos de las manos. Es útil en la evaluación del Fenómeno de Raynaud y de las colagenopatías, principalmente de la esclerodermia; sin embargo, se conoce poco acerca de la prevalencia y distribución de los cambios capilaroscópicos en sujetos sanos, siendo el objetivo de este estudio evaluar y describir las alteraciones capilaroscópicas en este grupo. A 100 participantes seleccionados por criterios de inclusión y exclusión se les realizó una videocapilaroscopia del lecho ungueal del cuarto y quinto dedo de la mano no dominante; el 86% de la población estudiada presentó hallazgos capilaroscópicos como: capilares tortuosos, entrecruzados y arborificados. Además, las alteraciones capilaroscópicas fueron más frecuentes en sujetos que consumían tabaco, se encontró una relación significativa entre el tabaco y la presencia de arborificaciones. Este es el primer estudio descriptivo de alteraciones capilaroscópicas en sujetos sanos en el Ecuador.

Capillaroscopy is a noninvasive and safe method that allows visualizationof capillaries in the nailbed. It is useful in the assesment ofRaynaud’s phenomenon and collagen diseases, especially scleroderma;however, little is known about the prevalence and distributionof capillaroscopic changes in healthy subjects, hence the aim ofthis study was to assess and describe the microvascular alterationsin this group. 100 participants selected by inclusion and exclusioncriteria underwent videocapillaroscopy of the nailfold of the fourthand fifth fingers of the nondominant hand; 86% of the study populationpresented capillaroscopic findings such as tortuous capillaries,crisscrossing and arborifications. Also, the microvascular alterationswere more frequent in subjects who had history of tobaccosmoking: a significant relationship between smoking and the presenceof arborifications was found. This is the first descriptive studyof microvascular alterations in healthy subjects in Ecuador.

The Efficacy of Nailfold Capillaroscopy in Patients with Raynaud's Phenomenon

Raynaud's phenomenon (RP) is characterized by reversible digital vasospasm triggered by exposure to cold or emotional stress. It manifests with unique 'triphasic' (pallor, cyanosis, erythema) or 'biphasic' (white, blue) color change. Clinically, RP is classified as primary RP, which does not have an underlying associated cause with a relatively benign course, and secondary RP, which predates various cause such as systemic connective tissue disease (CTD). Therefore, RP must be differentiated for detection of emerging CTD such as systemic sclerosis (SSc), systemic lupus erythematosus, and mixed conective tissue disease, ect. Nailfold capillaroscopy (NFC) is a non-invasive morphological study used routinely with antinuclear antibodies for parallel of secondary RP. A recent study showed NFC to be the best predictor of transition from a primary RP to secondary RP. The well-established role of NFC for early diagnosis of SSc, and potential for monitoring disease progression and predictive clinical complication makes NFC an important tool in research and clinical practice. In this paper, I present a recent update with emphasis on its possible role as a reliable diagnostic tool and biomarker in secondary RP.

Quantitative analysis of nailfold capillary morphology in patients with fibromyalgia

BACKGROUND/AIMS: Nailfold capillaroscopy (NFC) has been used to examine morphological and functional microcirculation changes in connective tissue diseases. It has been demonstrated that NFC patterns reflect abnormal microvascular dynamics, which may play a role in fibromyalgia (FM) syndrome. The aim of this study was to determine NFC patterns in FM, and their association with clinical features of FM. METHODS: A total of 67 patients with FM, and 30 age- and sex-matched healthy controls, were included. Nailfold capillary patterns were quantitatively analyzed using computerized NFC. The parameters of interest were as follows: number of capillaries within the central 3 mm, deletion score, apical limb width, capillary width, and capillary dimension. Capillary dimension was determined by calculating the number of capillaries using the Adobe Photoshop version 7.0. RESULTS: FM patients had a lower number of capillaries and higher deletion scores on NFC compared to healthy controls (17.3 +/- 1.7 vs. 21.8 +/- 2.9, p < 0.05; 2.2 +/- 0.9 vs. 0.7 +/- 0.6, p < 0.05, respectively). Both apical limb width (microm) and capillary width (microm) were significantly decreased in FM patients (1.1 +/- 0.2 vs. 3.7 +/- 0.6; 5.4 +/- 0.5 vs. 7.5 +/- 1.4, respectively), indicating that FM patients have abnormally decreased digital capillary diameter and density. Interestingly, there was no difference in capillary dimension between the two groups, suggesting that the length or tortuosity of capillaries in FM patients is increased to compensate for diminished microcirculation. CONCLUSIONS: FM patients had altered capillary density and diameter in the digits. Diminished microcirculation on NFC may alter capillary density and increase tortuosity.

El papel de la capilaroscopia del lecho ungueal en reumatología / No presenta

La capilaroscopia del lecho ungueal (CLU) es un método simple, inocuo y económico para el estudio detallado de la microcirculación en una amplia gama de enfermedades o síndromes de interés reumatológico. En el fenómeno de Raynaud (FR) no solo permite un preciso estudio de la circulación capilar sino que facilita la distinción entre FR primario y secundario. Posee un excepcional valor predictivo en la individuación de anormalidades morfoestructurales en fases tempranas de la esclerosis sistémica (ES), lo cual representa una de las principales ventajas de este método de imagen. El daño microvascular es una típica característica de la esclerosis sistémica que viene representada por desorganización arquitectónica, presencia de tortuosidades, aumento del diámetro de los capilares, neoangiogénesis, hemorragias/trombosis y reducción de la densidad de los capilares. Tales cambios capilaroscópicos delinean el “patrón esclerodérmico” que a su vez es la máxima expresión de la microangiopatía esclerodérmica propiamente dicha. Anormalidades capilaroscópicas están también presentes en otras enfermedades reumáticas sistémicas como: dermatomiositis, síndrome de Sjögren, lupus eritematoso sistémico, enfermedad indiferenciada del tejido conectivo y síndrome antifosfolípido. Esta revisión tiene como objetivo remarcar las aplicaciones y los límites de la CLU y sus respectivas aplicaciones en la práctica cotidiana reumatológica.

Nailfold capillaroscopy is a simple, non-invasive, inexpensive and useful method for the analysis of microvascular abnormalities that can be found in rheumatic disorders. The well-known Raynaud’s phenomenon represents a clinical condition that should promptly lead to a microvascular analysis, in order to distinguish its primary form from the secondary. Capillaroscopy has an exceptional predictive value for the diagnosis of early systemic sclerosis and this may be the best advantage this technique can offer. Microvascular damage is a typical feature of systemic sclerosis and a great number of this patients present architectural disorganization, tortuositis, giant capillaries, neovascularization haemorrhages, loss of capIllaries and avascular areas. These capillaroscopic changes characterize the “scleroderma pattern” and reflect the scleroderma microangiopathy. Microvascular abnormalities have also been found in other systemic rheumatic diseases such as dermatomyositis, Sjögren’s syndrome, systemic lupus erythematosus, undifferentiated connective tissue disease and antiphospholipid syndrome. The aim of this review is to describe the applications and limits of naifold capillaroscopy in the rheumatological clinical practice.