The role of nailfold capillaroscopy in pediatric patients with Kawasaki disease.

BACKGROUND: Nailfold capillaroscopy has been used as a non-invasive diagnostic method for microvasculature evaluation in various rheumatological disorders. The present study aimed to determine the utility of nailfold capillaroscopy in the diagnosis of Kawasaki Disease (KD). METHOD: In this case-control study nailfold capillaroscopy was performed in 31 patients with KD and 30 healthy controls. All nailfold images were evaluated for capillary distribution and capillary morphology such as enlargement, tortuosity, and dilatation of the capillaries. RESULT: Abnormal capillaroscopic diameter was identified in 21 patients from the KD group and 4 patients in the control group. The most common abnormality in capillary diameter was irregular dilatation in 11 (35.4%) KD patients and in 4 people (13.3%) in the control group. Distortions of the normal capillary architecture was commonly seen in the KD group (n=8). A positive correlation was observed between coronary involvement and abnormal capillaroscopic results (r=.65, P<.03). The sensitivity and specificity of capillaroscopy for the diagnosis of KD were 84.0% (95%CI: 63.9-95.5%) and 72.2% (95%CI: 54.8-85.8%), respectively. The PPV and NPV of capillaroscopy for KD were 67.7% (95%CI: 48.6-83.3) and 86.7% (95% CI: 69.3-96.2), respectively. CONCLUSION: Capillary alterations are more common in KD patients compared to control group. Thus, nailfold capillaroscopy can be useful in detecting these alterations. Capillaroscopy is a sensitive test for detecting capillary alternations in KD patients. It could be used as a feasible diagnostic modality for evaluating microvascular damage in KD.

The role of nailfold capillaroscopy in pediatric patients with Kawasaki disease / El papel de la capilaroscopia periungueal pacientes pediátricos con la enfermedad de Kawasaki

Background: Nailfold capillaroscopy has been used as a non-invasive diagnostic method for microvasculature evaluation in various rheumatological disorders. The present study aimed to determine the utility of nailfold capillaroscopy in the diagnosis of Kawasaki Disease (KD). Method: In this case–control study nailfold capillaroscopy was performed in 31 patients with KD and 30 healthy controls. All nailfold images were evaluated for capillary distribution and capillary morphology such as enlargement, tortuosity, and dilatation of the capillaries. Result: Abnormal capillaroscopic diameter was identified in 21 patients from the KD group and 4 patients in the control group. The most common abnormality in capillary diameter was irregular dilatation in 11 (35.4%) KD patients and in 4 people (13.3%) in the control group. Distortions of the normal capillary architecture was commonly seen in the KD group (n=8). A positive correlation was observed between coronary involvement and abnormal capillaroscopic results (r=.65, P<.03). The sensitivity and specificity of capillaroscopy for the diagnosis of KD were 84.0% (95%CI: 63.9–95.5%) and 72.2% (95%CI: 54.8–85.8%), respectively. The PPV and NPV of capillaroscopy for KD were 67.7% (95%CI: 48.6–83.3) and 86.7% (95% CI: 69.3–96.2), respectively. Conclusion: Capillary alterations are more common in KD patients compared to control group. Thus, nailfold capillaroscopy can be useful in detecting these alterations. Capillaroscopy is a sensitive test for detecting capillary alternations in KD patients. It could be used as a feasible diagnostic modality for evaluating microvascular damage in KD.(AU)

Antecedentes: La capilaroscopia periungueal se ha utilizado como un método diagnóstico no-invasivo para evaluación de la microvasculatura en varios trastornos reumatológicos. El presente estudio pretendió determinar la utilidad de la capilaroscopia periungueal en el diagnóstico de la enfermedad de Kawasaki (KD). Método: En este estudio de casos y controles se realizó la capilaroscopia periungueal para 31 pacientes con KD y 30 controles sanos. Todas las imágenes de la ungueal se evaluaron para la distribución capilar y la morfología capilar, como la ampliación, la tortuosidad y la dilatación de los capilares. Resultado: Se identificó un diámetro capilaroscópico anormal en 21 pacientes del grupo KD y en 4 pacientes del grupo control. La anomalía más común en el diámetro de los capilares fue la dilatación irregular en 11 (35,4%) pacientes con KD y 4 personas (13,3%) en el grupo control. Las distorsiones de la arquitectura capilar normal se observaron comúnmente en el grupo KD (n=8). Se observó una correlación positiva entre la afectación coronaria y los resultados capilares anormales (r=0,65, p <0,03). La sensibilidad y la especificidad de una capilaroscopia para el diagnóstico de KD fueron del 84,0% (IC del 95%: 63,9-95,5%) y del 72,2% (IC del 95%: 54,8-85,8%), respectivamente. El VPP y el VAN de la capilaroscopia para KD fueron del 67,7% (IC del 95%: 48,6-83,3) y del 86,7% (IC del 95%: 69,3-96,2), respectivamente. Conclusión: Las alteraciones capilares son más frecuentes en los pacientes con KD en comparación con el grupo control. Así, la capilaroscopia periungueal puede ser útil en la detección de estas alteraciones. La capilaroscopia es una prueba sensible para detectar las alteraciones capilares en pacientes con KD. Podría utilizarse como modalidad diagnóstica factible para evaluación el daño microvascular en la KD.(AU)

Impacto clínico de la capilaroscopia periungueal en la práctica clínica diaria / Clinical impact of nailfold capillaroscopy in daily clinical practice

Introducción: La capilaroscopia periungueal (CP) es útil en la evaluación del fenómeno de Raynaud tanto primario como secundario, y en el seguimiento de los pacientes con esclerosis sistémica. Nuestro estudio evalúa el impacto de la CP en el diagnóstico, en función del motivo de solicitud y el perfil de autoanticuerpos en la práctica clínica diaria. Material y métodos: Se incluyeron todos los pacientes con al menos una CP entre junio de 2012 y diciembre de 2017. Se revisaron las historias clínicas y se determinó de forma dicotómica (sí/no) si la CP contribuyó al diagnóstico realizado en la consulta posterior a la realización de la CP. Se recogieron datos demográficos, clínicos y de laboratorio, motivo de solicitud de la CP y su relación con los patrones CP. Resultados: De 530 pacientes con una primera CP, 266 se realizaron como estudio de un fenómeno de Raynaud. De estos, en 20 pacientes (3,8%) se realizó un diagnóstico de enfermedad del tejido conectivo en la consulta posterior a la CP; 15 fueron diagnosticados de esclerosis sistémica, 4 de conectivopatía indiferenciada y uno de enfermedad mixta. Salvo un paciente diagnosticado de conectivopatía indiferenciada, el resto tenía anticuerpos antinucleares positivos y 11 de ellos, además, anticuerpos específicos (9 anticentrómero, uno anti-Scl70 y otro, anti-RNPC). La positividad de anticuerpos antinucleares se asoció con una mayor probabilidad de presentar una CP de esclerodermia, y ningún paciente diagnosticado de una enfermedad reumática tras la CP tenía un patrón normal. Conclusión: La CP es una técnica útil, pero con impacto limitado en el diagnóstico de enfermedades del tejido conectivo. La positividad de los anticuerpos se relaciona con una mayor probabilidad de presentar patrones patológicos en la CP.(AU)

Introduction: 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.(AU)

Clinical impact of nailfold capillaroscopy in daily clinical practice. / Impacto clínico de la capilaroscopia periungueal en la práctica clínica diaria.

INTRODUCTION: 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.

Low vitamin D serum levels in diffuse systemic sclerosis: a correlation with worst quality of life and severe capillaroscopic findings.

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±8.20ng/mL. Eleven percent of the patients had 25OHD levels ≤10ng/mL, 50% ≤20ng/mL and 87% ≤30ng/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.

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.

Hiperhidrosis focal primaria asociada a eritromelalgia primaria / Primary focal hyperhidrosis associated to primary erythromelalgia

Paciente femenina de 30 años de edad que consulta por un cuadro de hiperhidrosis, asociado a sensación de calor desde su niñez. Sufre crisis de calor en cara, cuello, parte superior del tronco y los cuatro miembros, que la inducen a transpirar. Durante estos episodios nota sensación de calor, dolor y con aparición de rubor en manos y pies. Los síntomas ceden con aplicación de frío. Estos síntomas que presenta desde su adolescencia, dificultan el sueño, sus tareas habituales y el trato social. Sus antecedentes familiares respecto del mismo cuadro son floridos. Los estudios hematológicos, inmunológicos, la electromiografía y el ecodoppler de miembros superiores, resultaron dentro de límites normales. En el video sobre capilaroscopía periungueal de manos, se constata cambios micro vasculares inespecíficos. En la prueba de provocación mediante aumento de la temperatura, se obtiene resultado positivo a 39°C para miembros superiores. La paciente presenta signo-sintomatología compatible con hiperhidrosis focal primaria. Los episodios de calor, rubor y dolor de los cuatro miembros, que ceden a la exposición al frío fueron interpretados como eritromelalgia, en base a la clínica y los antecedentes familiares. Para la hiperhidrosis se indica solución de cloruro de aluminio hexa-hidratado al 20%, de aplicación tópica y para la eritromelalgia citrato de magnesio en grageas 528 mg, de dos a cuatro por día. Según la bibliografía por nosotros revisada, la asociación de hiperhidrosis focal primaria y eritromelalgia primaria familiar, no se ha registrado en la literatura.

Female patient, aged 30 years-old, attending a medical consultation with hyperhidrosis associated with episodes of warmth and sweating since childhood, located in face, neck, upper thorax and extremities. These episodes are accompanied by heat, pain and reddening in hands and feet. The symptoms disappear to cold exposure. These symptoms altered sleep, daily activities and social life. A positive family history with similar clinical characteristics was obtained. Hematological and immunological studies, as well as upper limbs electromyography and ecodoppler are within normal parameters. Unspecific microvascular alterations were observed with finger-periungueal video capillaroscopy. A provoking test yielded positive results when rising temperature to 39°C in upper limbs. The patient symptoms are compatible with focal primary hyperhidrosis. Heat, reddening and pain in the upper and low extremities responding to cold exposure and the positive family history led us to diagnose erythromelalgia. The hyperhidrosis treatment consisted of a topical application of 20% hexhydrate aluminum chloride. For erythromelalgia treatment 528 mg magnesium citrate pills, 2-4 daily were administered. Literature review to find out association of primary focal hyperhidrosis with primary familiar erythromelalgia was negative.

Hiperhidrosis focal primaria asociada a eritromelalgia primaria / Primary focal hyperhidrosis associated to primary erythromelalgia

Paciente femenina de 30 años de edad que consulta por un cuadro de hiperhidrosis, asociado a sensación de calor desde su niñez. Sufre crisis de calor en cara, cuello, parte superior del tronco y los cuatro miembros, que la inducen a transpirar. Durante estos episodios nota sensación de calor, dolor y con aparición de rubor en manos y pies. Los síntomas ceden con aplicación de frío. Estos síntomas que presenta desde su adolescencia, dificultan el sueño, sus tareas habituales y el trato social. Sus antecedentes familiares respecto del mismo cuadro son floridos. Los estudios hematológicos, inmunológicos, la electromiografía y el ecodoppler de miembros superiores, resultaron dentro de límites normales. En el video sobre capilaroscopía periungueal de manos, se constata cambios micro vasculares inespecíficos. En la prueba de provocación mediante aumento de la temperatura, se obtiene resultado positivo a 39°C para miembros superiores. La paciente presenta signo-sintomatología compatible con hiperhidrosis focal primaria. Los episodios de calor, rubor y dolor de los cuatro miembros, que ceden a la exposición al frío fueron interpretados como eritromelalgia, en base a la clínica y los antecedentes familiares. Para la hiperhidrosis se indica solución de cloruro de aluminio hexa-hidratado al 20%, de aplicación tópica y para la eritromelalgia citrato de magnesio en grageas 528 mg, de dos a cuatro por día. Según la bibliografía por nosotros revisada, la asociación de hiperhidrosis focal primaria y eritromelalgia primaria familiar, no se ha registrado en la literatura.(AU)

Female patient, aged 30 years-old, attending a medical consultation with hyperhidrosis associated with episodes of warmth and sweating since childhood, located in face, neck, upper thorax and extremities. These episodes are accompanied by heat, pain and reddening in hands and feet. The symptoms disappear to cold exposure. These symptoms altered sleep, daily activities and social life. A positive family history with similar clinical characteristics was obtained. Hematological and immunological studies, as well as upper limbs electromyography and ecodoppler are within normal parameters. Unspecific microvascular alterations were observed with finger-periungueal video capillaroscopy. A provoking test yielded positive results when rising temperature to 39°C in upper limbs. The patient symptoms are compatible with focal primary hyperhidrosis. Heat, reddening and pain in the upper and low extremities responding to cold exposure and the positive family history led us to diagnose erythromelalgia. The hyperhidrosis treatment consisted of a topical application of 20% hexhydrate aluminum chloride. For erythromelalgia treatment 528 mg magnesium citrate pills, 2-4 daily were administered. Literature review to find out association of primary focal hyperhidrosis with primary familiar erythromelalgia was negative.(AU)