Esclerosis sistémica sine esclerodermia en una mujer con anticuerois antinucleares centroméricos, afectación pulmonar y digestiva: reporte de caso

Las entidadessineson poco comunes en reumatología. En la esclerosis sistémica progresiva, hay una variante de la forma cutánea limitada llamada esclerosis sistémicasineesclerodermia, cuya característica central es la falta de afectación cutánea, pero compromiso visceral presente. La positividad a los anticuerpos antitopoisomerasa o anticentromérico confirman el diagnóstico. El caso de este estudio es una mujer de 63 años con enfermedad intersticial pulmonar, afectación del tránsito intestinal y fenómeno de Raynaud, con ANA a títulos elevados con patrón centromérico y positividad para anticuerpos antitopoisomerasa. Ante un paciente con fenómeno de Raynaud, afectación visceral y ANA elevado, se le debe pedir anticuerpos específicos para diagnóstico de esclerosis sistémica en su variedadsine.

Sine entities are rare in rheumatology. In progressive systemic sclerosis there is a variant of the limited cutaneous form called systemic sclerosis sine scleroderma, whose central feature is the lack of skin involvement, but visceral involvement is present. Positive anti-topoisomerase or anti-centromere antibodies confirm the diagnosis. We present the case of a 63-year-old woman with interstitial lung disease, intestinal transit involvement and Raynaud's phenomenon, with high titers of ANA with a centromeric pattern and positivity for anti-topoisomerase antibodies. When faced with a patient with Raynaud's phenomenon, visceral involvement and elevated ANA, specific antibodies should be requested for the diagnosis of systemic sclerosis in its sine variety.

Real-world nailfold videocapillaroscopy in a referral centre in north-western Colombia: A real-life cohort

Introduction: Nailfold videocapillaroscopy is a non-invasive tool for the assessment of peripheral microcirculation, the main indication is the study of Raynaud's phenomenon, poorly standardized outside of this context. There is no clear information in real-life about the rea-sons for referral, the presence of clinical findings of autoimmune diseases, the frequency of patterns of autoantibodies, and specific capillaroscopic findings. Objective: The purpose of this survey is to describe the sociodemographic, clinical, paraclinical, and angioscopy findings of a cohort of subjects referred to a capillaroscopy service in North-western Colombia. Methods: A retrospective study was conducted, from 2015 to 2018. Categorical variables were expressed in frequency and percentage and quantitative variables in mean and standard deviation or median with interquartile range, depending on the distribution of the data. Results: A total of 318 capillaroscopies were performed for the first time. The main referral reason was Raynaud's phenomenon (n = 134; 42.1%). The most frequent baseline capillaroscopic pattern found was normal (n = 123; 38.7%). Of the 12 capillaroscopies that presented a non-specific pattern at a 6-month follow-up, only one (8.3%) progressed to a scleroderma pattern. In the subjects with systemic sclerosis, the most frequent clinical finding was sclerodactyly (n = 34; 37.8%), and 42/44 individuals (95.4%) had positive antinuclear antibodies; the most frequent pattern was centromere (n = 27; 64.3%) Conclusions: In a real-world setting, the main referral reason for capillaroscopy was Raynaud's phenomenon; more than a third of the subjects had normal capillaroscopic findings. Sclerodactyly was the most frequent clinical finding in patients with scleroderma capillaroscopic pattern.

Introducción: La videocapilaroscopia del lecho ungular es una herramienta no invasiva para la evaluación de la microcirculación periférica; la indicación principal es el estudio del fenómeno de Raynaud. Luego de una revisión de la literatura, no hay información clara sobre los motivos de remisión, presencia de hallazgos clínicos de enfermedades autoinmunes, frecuencia de patrones de autoanticuerpos y hallazgos capilaroscópicos específicos. Objetivo: Describir los hallazgos sociodemográficos, clínicos, paraclínicos y capilaroscópicos de sujetos remitidos a un servicio de capilaroscopia en el noroccidente colombiano. Métodos: Estudio retrospectivo de 2015 a 2018. Las variables categóricas se expresaron en frecuencias absolutas y porcentajes, y las variables cuantitativas en media y desviación estándar o mediana con rango intercuartílico, dependiendo de la distribución de los datos. Resultados: Se realizaron 318 capilaroscopias por primera vez. El principal motivo de remisión fue el fenómeno de Raynaud (n = 134; 42,1%). El patrón capilaroscópico basal más frecuente fue el normal (n = 123; 38,7%). De las 12 capilaroscopias que presentaron un patrón no específico en un seguimiento de seis meses, solo una (8,3%) progresó a un patrón de esclerodermia. En los sujetos con esclerosis sistémica, el hallazgo clínico más frecuente fue la esclerodactilia (n = 34; 37,8%), y 42/44 individuos (95,4%) tenían anticuerpos antinucleares positivos; el patrón más frecuente fue el centromérico (n = 27; 64,3%). Conclusiones: La razón principal de remisión para realizar una capilaroscopia fue el fenómeno de Raynaud; más de un tercio de los sujetos tenían hallazgos capilaroscópicos normales. La esclerodactilia fue el hallazgo clínico más frecuente en pacientes con patrón capilaroscópico de esclerodermia.

Simpatectomía de la arteria radial y ulnar en contexto de úlcera digital secundaria a esclerosis sistémica cutánea localizada / Radial and ulnar artery sympathectomy in the context of digital ulcer secondary to localized cutaneous systemic sclerosis

SPeripheral sympathectomy is a procedure which has shown high rates of decreasing ischemic pain, recover functionality and wound healing, preventing the progression of the disease and further complications. We present a female patient with severe Raynaud´s phenomenon secondary to localized cutaneous systemic sclerosis complicated who presented digital ulcer treated with a sympathectomy of the radial and ulnar artery at the wrist level, undergoing post-operative follow-up.

Characterization of adult patients with systemic sclerosis in a reference center from northwestern Colombia: A descriptive survey / Caracterización de pacientes adultos con esclerosis sistémica en un centro de referencia del noroccidente de Colombia: un estudio descriptivo

ABSTRACT Background: Systemic sclerosis is an autoimmune and multisystemic disease characterized by vascular involvement and fibrosis that mainly affects the skin and internal organs; its morbidity and mortality are the highest of rheumatic diseases. Objective: To determine the sociodemographic, clinical, and paraclinical characteristics of adult patients with systemic sclerosis in a reference center in rheumatology in northwestern Colombia, between 2006 and 2016. Methods: A retrospective descriptive study was conducted. Information on sociodemographic, clinical, and paraclinical variables was collected from the review of medical records. The data were processed with the IBM SPSS 22 statistical package. The qualitative variables were expressed in absolute and relative frequencies and the quantitative variables in mean and standard deviation or median and interquartile range, according to their distribution. Results: 44 patients were included, predominantly women (90.9%), with an average age of 59 years; the most common variety was the limited (61.3%). One hundred percent of the subjects presented Raynaud's phenomenon and the most common compromised systems were: cutaneous (100%), musculoskeletal (59.1%), and gastrointestinal (50%). 95.5% of the patients fulfilled the diagnostic criteria ACR / EULAR 2013. Regarding the immunological profile, 72.7% of the individuals had positive antinuclear antibodies, of which 53.1% had a centromeric pattern. Conclusions: Sociodemographic, clinical and similar characteristics were found in this cohort, according to what was published in the literature. The most frequent extracutaneous systems involved were: musculoskeletal, gastrointestinal, and pulmonary, which cause a large part of the associated morbidity, as well as a decrease in quality of life.

RESUMEN Introducción: La esclerosis sistémica es una enfermedad autoinmune y multisistémica, caracterizada por vasculopatía y fibrosis que afecta principalmente la piel y órganos internos; su morbilidad y mortalidad son las más altas de las enfermedades reumatológicas. Objetivo: Determinar las características sociodemográficas, clínicas y paraclínicas de pacientes adultos con esclerosis sistémica en un centro de referencia en reumatología del noroccidente de Colombia, entre el 2006 y el 2016. Métodos: Estudio descriptivo retrospectivo. La información sobre las variables sociodemográficas, clínicas y paraclínicas se recogió de la revisión de registros médicos. Los datos se procesaron con el paquete estadístico IBM SPSS 22. Las variables cualitativas se expresaron en frecuencias absolutas y relativas y las cuantitativas en media y desviación estándar o mediana y rango intercuartílico, según su distribución. Resultados: Se incluyeron 44 pacientes, predominantemente mujeres (90,9%), con una edad promedio de 59 arios; la variedad más común fue la limitada (61,3%). El 100% de los sujetos presentaron el fenómeno de Raynaud y los sistemas comprometidos con mayor frecuencia fueron: cutáneo (100%), musculoesquelético (59,1%) y gastrointestinal (50%). El 95,5% de los pacientes cumplieron con los criterios diagnósticos ACR/EULAR 2013. Con respecto al perfil inmunológico, el 72,7% de los individuos tenía anticuerpos antinucleares positivos, de los cuales, un 53,1% tenía un patrón centromérico. Conclusiones: Se encontraron características sociodemográficas y clínicas similares en esta cohorte, de acuerdo a lo publicado en la literatura. Los sistemas extracutáneos más frecuentemente involucrados fueron: musculoesquelético, gastrointestinal y pulmonar, que causan una gran parte de la morbilidad asociada, así como una disminución en la calidad de vida.

Raynaud's phenomenon, capillaroscopy, and digital ulcers as sentinel events in systemic sclerosis / Fenómeno de Raynaud, capilaroscopia y úlceras digitales como eventos centinela en la esclerosis sistémica

ABSTRACT Raynaud's phenomenon is a sentinel event in systemic sclerosis; it is generally long- lasting, and repeated attacks produce, ultimately, structural consequences and complications, such as digital ulcers. Nailfold videocapillaroscopy is a non-invasive tool that allows assessing accurately these changes. Digital ulcers are the most aggressive and frequent microvascular complication in this disease, causing severe pain and significant functional sequelae. This literature review focuses on Raynaud's phenomenon as a central event in systemic sclerosis, its peculiarities in this entity, the role of capillaroscopy as a biomarker in this vasculopathic phenomenon, as well as a on a clinical and pharmacological approach to digital ulcers in this illness.

RESUMEN El fenómeno de Raynaud es un evento centinela en la esclerosis sistémica, por lo general es de larga duración y los ataques repetidos producen, en última instancia, consecuencias estructurales y complicaciones tales como úlceras digitales. La videocapilaroscopia del lecho ungueal es una herramienta no invasiva que permite evaluar en forma precisa estos cambios. Las úlceras digitales son la complicación microvascular más agresiva y frecuente en esta enfermedad, causando dolor severo y secuelas funcionales importantes. Esta revisión de la literatura se centra en el fenómeno de Raynaud como evento central en la esclerosis sistémica, sus peculiaridades en esta entidad y el papel de la capilaroscopia como biomarcador para este fenómeno vasculopático, así como en un enfoque clínico y farmacológico de las úlceras digitales en esta enfermedad.

Raynaud's phenomenon in the occupational context

SUMMARY OBJECTIVE To review articles that evaluated the prevalence of Raynaud's phenomenon of occupational origin. METHODS The search for articles was carried out in the Medline (via PubMed), Embase, Web of Science, Scientific Electronic Library Online (SciELO), and Latin America and Caribbean Health Sciences Literature (Lilacs) databases. RESULTS 64 articles were obtained from the electronic search; 18 articles met the eligibility criteria. All studies discussed the exposure to vibrations in the upper limbs. In 6 of them, the thermal issue was directly or indirectly addressed. No studies have addressed exposure to vinyl chloride. CONCLUSIO In general, a higher prevalence of Raynaud's phenomenon was found among vibratory tool operators compared to non-exposed workers, with an increase in the number of cases the higher the level of vibration and the time of exposure. Cold is a triggering and aggravating factor of the Raynaud phenomenon and seems to play an important role in the emergence of vascular manifestations of the hand-arm vibration syndrome.

RESUMO OBJETIVO Realizar um estudo de revisão dos artigos que avaliaram a prevalência do fenômeno de Raynaud de origem ocupacional. MÉTODOS A busca pelos artigos foi realizada nas bases de dados Medline (via PubMed), Embase, Web of Science, Scientific Eletronic Library Online (SciELO) e Literatura Latino-Americana e do Caribe em Ciências da Saúde (Lilacs). RESULTADOS Sessenta e quatro artigos foram obtidos a partir da busca eletrônica, dos quais 18 cumpriram os critérios de elegibilidade. Todos os estudos discutiram sobre a exposição a vibrações localizadas em membros superiores. Em seis deles, a questão térmica foi direta ou indiretamente abordada. Nenhum estudo abordou a exposição ao cloreto de vinila. CONCLUSÃO De maneira geral, constatou-se maior prevalência do fenômeno de Raynaud entre operadores de ferramentas vibratórias em comparação aos não expostos, com aumento do número de casos quanto maior o nível de vibração e tempo de exposição. O frio é fator desencadeante e agravante do fenômeno de Raynaud e parece exercer papel importante para o surgimento das manifestações vasculares da síndrome de vibração de mãos e braços.

Características de la videocapilaroscopia del lecho ungular y de los anticuerpos antinucleares en una cohorte de pacientes con enfermedad autoinmune sistémica con fenómeno de Raynaud / Characteristics of nailfold videocapillaroscopy and antinuclear antibodies in a cohort of patients with systemic autoimmune disease with Raynaud's phenomenon

Resumen Introducción: Entre un 15 y un 20% de los pacientes con fenómeno de Raynaud progresarán a una enfermedad autoinmune sistémica. La presencia de autoanticuerpos o alteraciones capilaroscópicas es fundamental para el diagnóstico temprano. Objetivos: Determinar las características de la videocapilaroscopia del lecho ungular y de los anticuerpos antinucleares en una cohorte de pacientes con enfermedad autoinmune sistémica. Materiales y métodos: Se realizó un estudio transversal en sujetos con fenómeno de Raynaud. Estos fueron evaluados con videocapilaroscopia y anticuerpos antinucleares. Las variables cualitativas se describieron con frecuencias absolutas y relativas; las variables cuantitativas, según la distribución de los datos, se reportaron como media o mediana, con desviación estándar y rango intercuartílico, respectivamente. Resultados: Se incluyeron 58 individuos; el 91,4% eran mujeres. La edad promedio fue 40,9 ± 14,1 arios. En 41 sujetos, los anticuerpos antinucleares fueron positivos; el patrón más común fue el moteado (41,5%), con una mediana de dilución de 1:640 (rango inter-cuartílico 1:320-1:1.280). Se encontró enfermedad autoinmune sistémica en 10 individuos (19,2%), 8 de ellos con esclerosis sistémica. Las alteraciones capilares más frecuentes fueron: megacapilares (n = 10), microhemorragias (n = 10) y zonas avasculares (n = 8). Conclusiones: En este grupo de pacientes con fenómeno de Raynaud sometidos a video-capilaroscopia, el diagnóstico de enfermedad autoinmune sistémica fue realizado en un porcentaje similar a lo reportado en la literatura. Se encontró una mayor dilución de anticuerpos antinucleares que la descrita.

Abstract Introduction: Between 15 and 20% of patients with Raynaud's phenomenon will progress to a systemic autoimmune disease. The presence of autoantibodies or capillaroscopy alterations are fundamental for early diagnosis. Objectives: To determine the characteristics of nailfold videocapillaroscopy and antinuclear antibodies in a cohort of patients with systemic autoimmune disease. Materials and methods: A cross-sectional study was conducted in subjects with Raynaud s phenomenon. These were evaluated with videocapillaroscopy and antinuclear antibodies. The qualitative variables were described with absolute and relative frequencies. The quantitative variables, according to the distribution of data, were reported as mean or median, with standard deviation and interquartile range, respectively. Results: The study included 58 individuals, of which 91.4% were women. The mean age was 40.9 ± 14.1 years. Antinuclear antibodies were positive in 41 subjects. The most common pattern was speckled (41.5%), with a median dilution of 1:640 (interquartile range 1:3201:1,280). A systemic autoimmune disease was found in 10 (19.2%) patients, 8 of them with systemic sclerosis. The most frequent capillary alterations were: mega-capillaries (n = 10), micro-haemorrhages (n = 10), and avascular zones (n = 8). Conclusions: In this group of patients with Raynaud's phenomenon subjected to videocapillaroscopy, a diagnosis of systemic autoimmune disease was made in a similar percentage to that reported in the literature. A higher dilution of antinuclear antibodies was found than that described.

Systemic lupus erythematosus and Raynaud's phenomenon

Abstract: BACKGROUND: Patients with systemic lupus erythematosus seem to belong to different serological and clinical subgroups of the disease. Genetic background can cause the appearance of these subgroups. OBJECTIVE: To determine whether Brazilian patients who have systemic lupus erythematosus and Raynaud's phenomenon differ from those who do not. METHODS: Retrospective analysis of 373 medical records of systemic lupus erythematosus patients studied for demographic, clinical and serological data. A comparative analysis was performed of individuals with and without RP. RESULTS: There was a positive association between Raynaud's phenomenon and age at diagnosis (p=0.02), presence of anti-Sm (p=0.01) antibodies and anti-RNP (p<0.0001). Furthermore, a negative association was found between Raynaud's phenomenon and hemolysis (p=0.01), serositis (p=0.01), glomerulonephritis (p=0.0004) and IgM aCL (p=0.004) antibodies. CONCLUSION: Raynaud's phenomenon patients appear to belong to a systemic lupus erythematosus subset with a spectrum of clinical manifestations located in a more benign pole of the disease.

Two-Step Incision for Periarterial Sympathectomy of the Hand

BACKGROUND: Surgical scars on the palmar surface of the hand may lead to functional and also aesthetic and psychological consequences. The objective of this study was to introduce a new incision technique for periarterial sympathectomy of the hand and to compare the results of the new two-step incision technique with those of a Koman incision by using an objective questionnaire. METHODS: A total of 40 patients (17 men and 23 women) with intractable Raynaud's disease or syndrome underwent surgery in our hospital, conducted by a single surgeon, between January 2008 and January 2013. Patients who had undergone extended sympathectomy or vessel graft were excluded. Clinical evaluation of postoperative scars was performed in both groups one year after surgery using the patient and observer scar assessment scale (POSAS) and the Wake Forest University rating scale. RESULTS: The total patient score was 8.59 (range, 6-15) in the two-step incision group and 9.62 (range, 7-18) in the Koman incision group. A significant difference was found between the groups in the total PS score (P-value=0.034) but not in the total observer score. Our analysis found no significant difference in preoperative and postoperative Wake Forest University rating scale scores between the two-step and Koman incision groups. The time required for recovery prior to returning to work after surgery was shorter in the two-step incision group, with a mean of 29.48 days in the two-step incision group and 34.15 days in the Koman incision group (P=0.03). CONCLUSIONS: Compared to the Koman incision, the new two-step incision technique provides better aesthetic results, similar symptom improvement, and a reduction in the recovery time required before returning to work. Furthermore, this incision allows the surgeon to access a wide surgical field and a sufficient exposure of anatomical structures.

Fenómeno de Raynaud. Revisión / A review of Raynaud's phenomenon

El fenómeno de Raynaud es una respuesta vascular exagerada al frío o al estrés que se manifiesta por cambios de coloración de la piel en diversas localizaciones; característicamente, presenta tres fases distintas que comprenden palidez, cianosis y rubor. Dependiendo de la ausencia o la presencia de enfermedad de base, el fenómeno se denomina como primario o secundario, siendo este último subtipo una manifestación importante de enfermedades graves, principalmente autoinmunes. El fenómeno de Raynaud primario tiene buen pronóstico y la posibilidad de manejo por médico primario; por el contrario, el secundario implica la necesidad de evaluación y manejo especializado. Es de importancia para el médico general conocer su clasificación, las bases del manejo para control de los signos y síntomas, además de identificar cuándo un paciente requiere valoración y manejo especializado de acuerdo con otras enfermedades de base que puedan coexistir.

Raynaud's phenomenon is an exaggerated vascular response to cold or stress manifested by skin colour changes at several locations, having three characteristic phases: pallor (white), cyanosis (blue) and rubor (red). When the cause is not known this phenomenon is termed primary Raynaud's phenomenon and secondary Raynaud's phenomenon when underlying disease is involved, autoimmune disease being the most frequent cause of the secondary form. Primary Raynaud's phenomenon has a good prognosis and can be treated medically without the need for specialist assessment, unlike secondary Raynaud's phenomenon which needs specialist assessment and treatment. A general practitioner needs to know the classification, the basis for treatment for signs and symptoms control and know when a patient requires specialist evaluation for treatment according to other diseases which may be present or coexist.

Clinical observation for treating Renault's disease by stellate ganglion block with ropivacaine / 中国医师杂志

Objective To investigate the therapeutic efficacy and safety of stellate ganglion block (SGB) with ropivacaine to treat Renault's disease.Methods Thirty seven patients with Renault's disease were randomly assigned into two groups:the lidocainegroup (L group,18 patients) treated by SGB with 5 ml of 1％ lidocaine,and the Ropivacaine-group (R group,19 patients) treated by SGB with 5 ml of 0.2％ ropivacaine.The efficacy,adverse reactions,and the recurrence after 3 months of two groups were observed and compared.Results The total effective rate of 89.5 ％ in group R was significantly higher that 55.6％ in group L (P ＜ 0.05).There were less times(22 times) presenting no Homers'sign in group R compared to group L (43 times).The incidence of adverse reaction and the recurrence after 3 months in group R were not statistically significantly different form group L (P ＞ 0.05).Conclu-sions SGB with 0.2％ ropivacaine could obtain better efficacy compared to lidocaine,and had a higher security.

Digital Thermography of the Fingers and Toes in Raynaud's Phenomenon

The aim of this study was to determine whether skin temperature measurement by digital thermography on hands and feet is useful for diagnosis of Raynaud's phenomenon (RP). Fifty-seven patients with RP (primary RP, n = 33; secondary RP, n = 24) and 146 healthy volunteers were recruited. After acclimation to room temperature for 30 min, thermal imaging of palmar aspect of hands and dorsal aspect of feet were taken. Temperature differences between palm (center) and the coolest finger and temperature differences between foot dorsum (center) and first toe significantly differed between patients and controls. The area under curve analysis showed that temperature difference of the coolest finger (cutoff value: 2.2degrees C) differentiated RP patients from controls (sensitivity/specificity: 67/60%, respectively). Temperature differences of first toe (cutoff value: 3.11degrees C) also discriminated RP patients (sensitivity/specificity: about 73/66%, respectively). A combination of thermographic assessment of the coolest finger and first toe was highly effective in men (sensitivity/specificity : about 88/60%, respectively) while thermographic assessment of first toe was solely sufficient for women (sensitivity/specificity: about 74/68%, respectively). Thermographic assessment of the coolest finger and first toe is useful for diagnosing RP. In women, thermography of first toe is highly recommended.

Poliarteritis nodosa en el niño: informe del primer caso en Camagüey

Fundamento: la poliarteritis nodosa es una vasculitis de vasos medianos, caracterizados por inflamación o necrosis de los vasos sanguíneos que conducen a su oclusión y por tanto a la isquemia del tejido que irrigan. Caso clínico: paciente de 15 años de edad, color de piel blanca, masculino, con historia de cuadros febriles prolongados sin diagnóstico, por lo que tuvo múltiples ingresos en diferentes instituciones del país con la sospecha de inicio de una histiocitosis o enfermedad linfoproliferativa, para lo cual se le realizaron diversos estudios que incluyeron a la médula ósea sin infiltración y biopsia de ganglios, los resultados descartaron estas enfermedades. En uno de esos episodios y luego de tres años es remitido al servicio de reumatología pediátrica de la provincia de Camagüey, con historia de fiebre de hasta 38.5 ºC durante 16 días, una úlcera infectada en el brazo derecho con un estafilococo en cultivo de la misma. Al examinarlo además se constató fenómeno de Raynaud y livedo reticulares, lo que sugirió la posibilidad diagnóstica de una vasculitis. Conclusiones: se realizaron los anticuerpos antinucleares que fueron positivos y confirmaron el diagnóstico de la poliarteritis nodosa, el cual se efectuó luego de tres años de evolución; en estos momentos bajo tratamiento de inmunosupresión extrema, con altas dosis de esteroides y ciclofosfamida con evolución favorable del cuadro general, desaparición de la fiebre, pero se mantiene daño a nivel de los vasos coronarios, renales y en las regiones distales de las cuatro extremidades ya establecidos previamente.

Bloqueio do receptor de leucotrieno CysLT1 em pacientes com fenômeno de Raynaud: evidência capilaroscópica do papel do leucotrieno / CysLT1 receptor inhibition in patients with Raynaud's phenomenon: capillaroscopic evidence of the role of leukotriene

OBJETIVO: Avaliar a ação do medicamento inibidor do receptor de leucotrieno CysLT1 (montelucaste) nas alterações vasculares das mãos em pacientes com fenômeno de Raynaud. MÉTODOS: Foram selecionadas pacientes com fenômeno de Raynaud secundário à doença inflamatória do tecido conjuntivo, excluindo tabagismo, hipertensão arterial e diabetes mellitus. As pacientes mantiveram a medicação prévia e iniciaram o uso de montelucaste 10 mg/dia por 60 dias. Foi realizada capilaroscopia periungueal dos dedos das mãos antes do uso da medicação e após 30 e 60 dias. A análise estatística foi feita por meio de porcentagem, média, desvio-padrão e teste exato de Fisher, com intervalo de confiança de 95 por cento. RESULTADOS: Foram estudadas cinco pacientes mulheres, brancas, com fenômeno de Raynaud secundário a doenças do tecido conjuntivo, das quais três apresentavam esclerodermia e duas apresentavam doença mista do tecido conjuntivo. A média de idade foi de 42,4 ± 12,4 anos, e a média de tempo de doença foi de 9,6 ± 4,8 anos. As pacientes estavam em uso de até 20 mg/dia de prednisona (pacientes com doença mista do tecido conjuntivo), nifedipina, pentoxifilina. As medicações foram mantidas. Após o uso de inibidor de receptor de leucotrieno por dois meses, o controle com capilaroscopia ungueal demonstrou diminuição do edema e da palidez e normalização do número, tamanho e distribuição dos capilares. CONCLUSÃO: O uso do montelucaste modificou as alterações capilares observadas na capilaroscopia periungueal de pacientes com fenômeno de Raynaud.

OBJECTIVE: To assess the effect of the leukotriene receptor inhibitor (montelukast) on vascular alterations in fingers of patients with Raynaud's phenomenon. METHODS: Patients with Raynaud's phenomenon of the hands secondary to inflammatory connective tissue disease were selected, and those with the following characteristics were excluded: smokers, arterial hypertension, and diabetes mellitus. All patients maintained their previous medications and started the use of montelukast, 10 mg/day, for 60 days. Naifold capillaroscopy of fingers was performed before the use of medication and after 30 and 60 days. Statistical analysis was performed with percentage, media, standard deviation, Fisher exact test, with 95 percent of confidence interval. RESULTS: The study assessed five Caucasian, female patients with Raynaud's phenomenon secondary to inflammatory connective tissue disease (three with scleroderma and two with mixed connective tissue disease), aged 42.4 ± 11.5 years, and with 9.6 ± 4.8 years of disease duration. Patients were on nifedipine and pentoxifylline, and those with mixed connective tissue disease were also on prednisone. The medications were maintained. After using montelukast for two months, nailfold capillaroscopy showed a reduction in edema and pallor, and normalization of capillary number, size, and distribution. CONCLUSION: The use of montelukast modified the capillary abnormalities observed on nailfold capillaroscopy of patients with Raynaud's phenomenon.

Percutaneous bipolar radiofrequency T3 sympathicotomy in Raynaud's disease: A case report / 대한마취과학회지

A 54-year-old female was suffering from cold-induced Raynaud's attacks in her both hands with symptoms most severe in her left hand. As the patient did not respond to previous medical treatments and endoscopic thoracic sympathectomy, we performed percutaneous bipolar radiofrequency thoracic sympathicotomy at the left T3 vertebral level. After the procedure, the patient obtained a long duration of symptom relief over 3 years. Percutaneous bipolar radiofrequency T3 sympathicotomy is minimally invasive and effective technique by creating large continuous strip lesion.

Clinical analysis of systemic sclerosis patients with digital ulcer: from Chinese EUSTAR database / 中华风湿病学杂志

ObjectiveTo investigate the clinical and laboratory characteristics of systemic sclerosis (SSc) patients with digital ulcer(DU) in China.MethodThe data of 166 consecutive SSc patients in EUSTAR DATABASE in Peking Union Medical Colloge Hospital from February 2009 to August 2010 were prospectively collected,and patients with DU were compared with those without DU.All patients fulfilled the ACR classification criteria for SSc in 1980.Results① Forty-nine patients (29.5％) had DU in 166 SSc patients.The disease onset age was(36±12) years(8.1-61.7 years) for those patients with DU.All had Raynaud's phenomenon(RP).② Demographic data:there were significant differences between patients with and without DU in sex (F/M 40/9 vs 112/5,P=0.005),age [(40±12) years old vs(46±12) years old,P=0.005],the onset age of RP [(33±12) years vs(39±13) years,P=0.005] and the duration from RP to the first non-RP presentaion[ (18±15) months vs(115±307) months,P=0.002 ].③ Clinical manifestations and laboratory findings:there were more diffuse SSc patients and more esophageal involvement in patients with DU (P＜0.05).ConclusionsDU in SSc patients is common,especially in man and patients with diffuse SSc.SSc patients with DU usually are younger when RP onsets and the non-RP manifestations usually present earlier when compared with those patients without DU.

Simpatectomía periférica periarterial cubital en el manejo de la enfermedad de Raynaud / Peripheral sympathectomy periarterial ulnar managing Raynaud disease

El Síndrome de Raynaud (SR) se define como una entidad, que afecta los vasos sanguíneos de las porciones dístales del cuerpo (dedos, orejas, nariz) y cuya característica clínica es la presencia de ataques episódicos de vasoespasmo, se explica por una hipersensibilidad local de las arterias digitales al frío, con una intensificación de esta anormalidad por la estimulación simpática normal, que generan isquemia tisular; aunque es una entidad de presentación alarmante, es importante reconocer el comportamiento benigno de ésta. Actualmente se han reconocido varios factores que influyen en la presentación del síndrome y que han permitido enfocar el manejo de estos pacientes. La simpatectomía periférica peri arterial es una alternativa para el manejo de estos pacientes sintomáticos que no responden con tratamiento conservador.

Raynaud’s syndrome is defined as an entity that affects the blood vessels of the distal portions of the body (fingers, ears, nose) and whose clinical feature is the presence of episodic attacks of vasospasm can be explained by a local hypersensitivity digital arteries to cold, with an intensification of this abnormality by normal sympathetic stimulation, which generates tissue ischemia, although a filing entity alarming, it is important to recognize this benign behavior. Have now been recognized several factors that influence the presentation of the syndrome, which allowed focus on the management of these patients. Periarterial peripheral sympathectomy is an alternative for the management of symptomatic patients who do not respond to conservative treatment.

Significance of the Raynaud phenomenon in systemic lupus erythemtosus / 中国医师进修杂志

Objective To study the clinical significance of Raynaud phenomenon (RP) in systemic lupus erythematosus(SLE).Methods One hundred and twelve cases with SLE were divided into two groups according to the condition of RP and were analyzed retrospectively.The similarities and differences were investigated in system injury and laboratory examination in two groups.Results The occurrence rates of pulmonary arterial hypertension (PAH),interstitial lung disease (ILD),pericarditis,fatigue,pulmonary embolism (PE) and deep venous thrombosis (DVT) in patients with RP were higher than those in patients without RP [89.3％(25/28) vs.2.4％(2/84),57.1％(16/28) vs.16.7％(14/84),75.0％(21/28) vs.39.3％ (33/84),67.9％(19/28) vs.33.3％(28/84),21.4％(6/28) vs.4.8％(4/84),14.3％(14/28) vs.1.2％(1/84) ](P ＜ 0.05 ).The positive rates of anti-cardiolipin (ACL) antibody,anti-RNP antibody,anti-Sm antibody and LA in patients with RP were higher than those in patients without RP [46.4％(13/28) vs.9.5％(8/84),75.0％(21/28) vs.33.3％(28/84),57.1％(16/28) vs.23.8％(20/84),17.9％(5/28) vs.2.4％(2/84)] (P ＜0.05),while leucopenia in patients with RP was lower than that in patients without RP [7.1％(2/28) vs.45.2％ (38/84)] (P =0.001).Conclusion Heart,lung and vascular lesions such as PAH,ILD,pericarditis,PE and so on are easier to occur in SLE with RP,furthermore the positive rates of ACL,anti-RNP antibody,anti-Sm antibody,and LA are higher than those without RP.

Sequential bipolar radiofrequency lumbar sympathectomy in Raynaud's disease: A case report / 대한마취과학회지

A 39-year-old female was suffering from cold-induced Raynaud's attacks in both hands and feet, with symptoms being most severe in her left foot. The patient did not respond to medical treatments and was referred to our department of pain medicine. We performed sequential bipolar radiofrequency lumbar sympathectomy to the patient, which offered a long duration of symptom relief. Sequential bipolar radiofrequency lesions could create continuous strip lesion, and thus, could achieve better results, while the potential risk of liquid neurolytic agents could be avoided.

Esclerodermia sistêmica: relato de caso clínico / Systemic sclerosis: a case report

A esclerodermia sistêmica é uma doença dermatológica que pode afetar os tecidos orais e periorais. Suaetiologia é desconhecida sendo associada a um mecanismo autoimune. A doença acomete preferencialmentemulheres negras, entre 30 e 50 anos de idade, e se caracteriza por modificações da microcirculaçãocausando fibrose e obliteração das veias da pele, pulmão, trato gastrointestinal, rins e coração. O fenômenode Raynaud e edema das mãos e dedos são sinais característicos da doença. As manifestações orofaciais incluemaumento do espaço do ligamento periodontal, limitação da função mastigatória, dificuldade no processode reparo, fibrose da pele, redução da abertura bucal, entre outros. O objetivo deste trabalho é relataro caso clínico de uma paciente portadora de Esclerodermia Sistêmica, visando auxiliar o cirurgião-dentistaa identificar os sinais e sintomas característicos dessa alteração sistêmica que pode acometer os pacientes

The Systemic Scleroderma is a dermatologic illness that can affect oral and dermal tissue. Its etiology isunknown and it?s commonly associated to an autoimmune mechanism. The illness affects preferentiallyafrican-american women, between 30 and 50 years old and it is characterized by modifications of the microcirculationthat causes fibrosis and obliteration of the veins of the skin, lung, gastrointestinal system,kidneys and heart. Raynaud?s phenomenon and edema of the hands and fingers are characteristics signalsof the illness. The orofacial manifestations include increase of the space of the periodontal ligament, limitationof the chewing function, difficulty in the wound healing process, fibrosis of the face skin and reductionof the buccal opening, among others. The objective of this work is to describe a case of a pacient withSystemic Scleroderma looking foward to help the dentist identify the signs and symptoms of this illness.The dental treatment will then be modified to suit these patients