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.

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.

Raynaud's Phenomenon: Its Prevalence in Systemic Sclerosis and Its Relationship with the Onset and with the Subsets of Systemic Sclerosis - An Experience from the Kashmir Valley, India / Fenómeno de Raynaud: su prevalencia en la esclerosis sistémica y sus relación con el inicio y los subconjuntos de la esclerosis sistémica - una experiencia del Valle de Kashmir, India

ABSTRACT Background: Raynaud's phenomenon (RP) is a very common clinical sign in patients with systemic sclerosis (SSc). Within the same country, its prevalence may vary depending on climactic changes. Usually, it predates the onset of cutaneous involvement in SSc, but in rare cases, it can follow the skin changes in these patients. Its evolution differs in the two subsets of SSc (limited and diffuse) and can serve as a clinical pointer to differentiate between the two disease subsets. Objective: To study the prevalence of RP in SSc and report its relationship with the onset and with the subsets of SSc. Methods: A prospective observational study of 56 patients with SSc was carried out at the Postgraduate Department of Dermatology, STDs and Leprosy of Shri Maharaja Hari Singh Hospital, Kashmir, India. Results: Of the 56 patients, 40 (71.4%) had limited SSc (lSSc) and 16 (28.6%) had diffuse SSc (dSSc). Raynaud's phenomenon was seen in 54 (96.4%) of the 56 patients, comprising 39 (97.5%) of the 40 patients with lSSc and 15 (93.8%) of the 16 patients with dSSc. Thirteen (81.3%) patients with dSSc and two (5%) patients with lSSc had a short history (less than one year) of RP preceding the skin changes. Twenty-six (65%) patients with lSSc and only two (12.5%) patients with dSSc had a long history (more than one year) of RP preceding the cutaneous manifestations. Six (15%) of the 40 patients with lSSc had a simultaneous onset of RP and skin changes. In five (12.5%) of the 40 patients with lSSc, RP followed the skin changes. Conclusion: Raynaud's phenomenon was very common in these patients with SSc. Patients with lSSc had a longer history of RP compared with those with dSSc. It could occur simultaneously with skin changes or even postdate the onset of skin changes.

RESUMEN Antecedentes: El fenómeno de Raynaud (FR) es una manifestación clínica muy común en pacientes con esclerosis sistémica (ES). Dentro del mismo país, su prevalencia puede variar en función de los cambios climáticos. Generalmente, precede al inicio de la afección cutánea en la ES, pero en raros casos puede seguir a los cambios de piel en estos pacientes. Su evolución difiere en los dos subconjuntos de ES (limitada y difusa), y puede servir como indicador clínico para poder diferenciar entre estos dos subconjuntos. Objetivo: Estudiar la prevalencia de FR en la ES y reportar sus relación con el inicio y los subconjuntos de ES. Métodos: Se realizó un estudio observacional prospectivo de 56 pacientes con ES en el Departamento de Posgrado de Dermatología, Enfermedades de Transmisión Sexual y Lepra del Hospital Shri Maharaja Hari Singh, India. Resultados: De los 56 pacientes, 40 (71.4%) tenían ES limitada (ESL) y 16 (28.6%) tenían ES difusa (ESD). El fenómeno de Raynaud se observó en 54 (96.4%) de los 56 pacientes, abarcando 39 (97.5%) de los 40 pacientes con la variante ESL y 15 (93.8%) de los 16 pacientes con la variante ESD. Trece (81.3%) pacientes con ESD y dos (5%) pacientes con ESL tenían una historia corta (menos de un año) de FR, que precedía a los cambios cutáneos. Veintiséis pacientes (65%) con ESL y solamente dos (12.5%) pacientes con ESD tenían una historia larga (más de un año) de FR, que precedía a las manifestaciones cutáneas. Seis (15%) de los 40 pacientes con ESL tuvieron un inicio simultáneo de FR y cambios de piel. En cinco (12.5%) de los 40 pacientes con ESL, FR siguió a los cambios de la piel. Conclusión: El fenómeno de Raynaud fue muy común en estos pacientes con ES. Los pacientes con ESL tuvieron una historia más larga de FR, a diferencia de aquellos con ESD. El fenómeno de Raynaud podía ocurrir simultáneamente con cambios cutáneos o incluso presentarse tras el comienzo de los cambios cutáneos.

Esclerose sistêmica e fenômeno de Raynaud: manejo terapêutico / Systemic sclerosis and Raynaud's phenomenon: therapeutic management

O fenômeno de Raynaud (FRy) caracteriza- se por episódios reversíveis de vasoespasmos de extremidades, que ocorrem usualmente após estresse ou exposição ao frio. O FRy pode ser primário ou secundário a uma série de condições, principalmente a doenças do espectro da esclerose sistêmica (ES). Na ES o FRy costuma ser mais grave, e lesões isquêmicas de extremidades são frequentes. Nos últimos anos, avanços no estudo da fisiopatologia do FRy e da doença vascular na ES propiciaram o surgimento de novas opções terapêuticas para esta manifestação. Os bloqueadores de canal de cálcio devem ser utilizados como tratamento de primeira escolha para o FRy. Novas drogas, como os inibidores da fosfodiesterase V e os prostanoides, podem ser utilizadas em pacientes com FRy grave, e a bosentana (antagonista do receptor da endotelina-1) é indicada para a prevenção de úlceras digitais recorrentes.

Raynaud?s phenomenon (RP) is characterized by episodic vasospasm of the extremities, usually in response to stress or cold exposure. It can be primary or secondary to several conditions, especially systemic sclerosis-related diseases. In systemic sclerosis (SSc), RP is usually more severe and digital ischemic lesions are a frequent problem. In recent years, advances in the understanding of the pathophysiology of RP and of SSc vasculopathy led to the development of new therapeutic options for this condition. Calcium-channel blockers are the first choice for the treatment of RP. New drugs, including phosphodiesterase type V inhibitors and prostanoids, can be used for severe RP, and bosentan (endothelin-1 receptor antagonist) for prevention of recurrent digital ulcers.

N-acetilcisteína oral no tratamento do fenômeno de Raynaud secundário à esclerose sistêmica: ensaio clínico randomizado, placebo-controlado e duplo-cego / Oral N-acetylcysteine in the treatment of Raynaud's phenomenon secondary to systemic sclerosis: A randomized, double-blind, placebo-controlled clinical trial

Objetivo Avaliar a segurança e a eficácia da N-acetilcisteína (NAC) por via oral sobre o fluxo sanguíneo da microcirculação digital em pacientes com fenômeno de Raynaud (FRy) secundário à esclerose sistêmica (ES). Métodos Este foi um estudo randomizado, duplo-cego e placebo-controlado, no qual 42 pacientes com ES receberam NAC oral na dose de 600 mg, três vezes ao dia (21 pacientes, idade média 45,6±9,5 anos) ou placebo (21 pacientes, idade média 45,0±12,7 anos) durante quatro semanas. O desfecho primário do estudo foi: melhora no fluxo sanguíneo da microcirculação cutânea antes e após estímulo frio avaliado pelo laser Doppler imaging (LDI) nas semanas 0 e 4. A frequência e a gravidade do FRy e o número de úlceras digitais também foram avaliados nas semanas 0 e 4. Os efeitos adversos foram registrados na quarta semana. Resultados Não houve mudança significativa no fluxo sanguíneo digital avaliado pelo LDI antes ou depois do estímulo frio após quatro semanas de NAC ou placebo. Ambos os grupos apresentaram melhora significativa na frequência e gravidade dos ataques de FRy, sem diferença entre os dois. O grupo placebo apresentou três úlceras digitais enquanto o grupo NAC não apresentou úlceras ao final do estudo. NAC foi bem tolerada e nenhum paciente descontinuou o tratamento. Conclusões NAC por via oral na dose de 1.800mg/dia não demonstrou efeito vasodilatador sobre a microcirculação das mãos após quatro semanas de tratamento em pacientes com FRy secundário à ES. .

Objective To evaluate the safety and efficacy of oral N-acetylcysteine (NAC) on digital microcirculation blood flow in patients with Raynaud's phenomenon (RP) secondary to systemic sclerosis (SSc). Methods This was a randomized, double-blind, placebo-controlled trial in which 42 patients with SSc received oral NAC at a dose of 600mg tid (21 patients, mean age 45.6±9.5 years) or placebo (21 patients, mean age 45.0±12.7 years) for four weeks. The primary endpoint was the change in cutaneous microcirculation blood flow before and after cold stimulation measured by laser Doppler imaging (LDI) at weeks 0 and 4. The frequency and severity of RP and the number of digital ulcers were also measured at weeks 0 and 4. The adverse events were recorded in the fourth week. Results There was no significant change in digital blood flow assessed by LDI before or after cold stimulus after four weeks of NAC or placebo. Both groups showed significant improvement in the frequency and severity of RP attacks, with no difference between the two groups. At the end of the study, the placebo group had three digital ulcers, while the NAC group showed no ulcers. NAC was well tolerated and no patient discontinued the treatment. Conclusions NAC orally at a dose of 1800mg/day showed no vasodilator effect on hands’ microcirculation after four weeks of treatment in patients with RP secondary to SSc. .

Mixoma: manifestaciones neurológicasy reumatológicas. Informe de casos / Myxoma: neurological and rheumatological manifestations. Case Report

Objetivo: informe de tres casos de mixoma cardíaco auricular izquierdo en pacientes de sexo femenino. Diseño de estudio: informe de casos. Lugar: Hospital Universitario San José de Popayán (HUSJ) - Facultad Ciencias de la Salud. Departamento de Medicina Interna. Universidad del Cauca. Pacientes: tres del sexo femenino, dos presentaron compromiso neurológico de tipo ataque vascular cerebral agudo embólico y un caso con manifestaciones reumatológicas. Intervención: ecocardiogramas. Tomografía axial computarizada (TAC) cerebral. Resección de masa cardíaca. Confirmación anatomopatológica. Resultado: tratamientos exitosos. Conclusiones: los pacientes con mixoma generalmente auricular producen síntomas cardíacos, pero, dada su variabilidad clínica, pueden presentarse como cuadros con compromiso cerebral y simular síndromes de origen reumatológico. Una vez identificada la causa, en los tres casos, se practicó resección tumoral.

Objective: To report three cases of left atrial cardiac myxoma in female patients.Study Design: Case reports.Location: University Hospital San José de Popayan (HUSJ), Faculty of Health Sciences. Department of Internal Medicine. Universidad del Cauca. 3 female patients. 2 had neurological involvement due to embolic acute cerebral vascular attack and 1 had rheumatologic manifestations.Intervention: Echocardiograms. Brain computed tomography (CT scan). Resection of cardiac mass. Pathologic confirmation.Result: successful treatment.Conclusions: Patients with atrial cardiac myxoma usually produce cardiac symptoms but, given its clinical variability, can manifest with brain involvement or simulate rheumatic syndromes. Once the cause was identified, in all three cases, tumor resection was performed.

Patogenia del fenómeno de Raynaud / Pathogenesis of Raynaud's phenomenon

El fenómeno de Raynaud consiste en una respuesta vascular exagerada al frío o al estrés emocional, que se manifiesta por un cambio trifásico de coloración en la circulación terminal. Esta reacción afecta a un 3 por ciento a 20 por ciento de la población, presenta un predominio femenino, y puede ser primario o secundario a otra patología. Este fenómeno puede ser clasificado en dos grupos, según su etiología y complicaciones: Raynaud primario y Raynaud secundario, o síndrome de Raynaud. El eje del Raynaud primario se basa en la existencia de un sistema de retroalimentación positiva de los fenómenos regulatorios neuronales, endoteliales y musculares. En el síndrome de Raynaud, la secuencia de eventos, en cambio, depende fundamentalmente de la etiología basal. en el caso de la esclerodermia, principal causante del Raynaud secundario, se describen niveles aumentados de factores endoteliales, tales como la endotelina 1, óxido nítrico y el anión superóxido, los que ejercen sus efectos mediante sus propiedades vasoactivas profibróticas. Además, se describen otros factores citotóxicos, como autoanticuerpos, citoquinas y factores del complemento que llevan a la activación del sistema inmunológico y daño secundario.

Raynaud's phenomenon is an exaggerated vascular response to cold or emotional stress, and is manifested by a triphasic change in the color of the terminal circulation. This reaction affects 3 percent to 20 percent of the population, has a female predominance, and may be primary or secondary to other pathology. This phenomenon can be classified into two groups according to their etiology and complications: primary and secondary Raynaud, also called Raynaud's syndrome. The pathogeny of primary Raynaud is centered on a positive feedback loop between the neuronal, endothelial and muscle regulatory systems. The sequence of events in the secondary Raynaud, on the other hand, depends primarily on its etiology. In the case of scleroderma, the main cause of secondary Raynaud, there have been reported increased levels of endothelial factors such as endothelin 1, nitric oxide and superoxide anion, which exert their effects through its vasoactive and pro-fibrotic properties. Also there have been described other cytotoxic factors such as autoantibodies, cytokines and complement factors that lead to activation of the immune system and secondary damage.

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.

Fenómeno de Raynaud por exposición a cloruro de vinilo / Raynaud's phenomenon secondary to vinyl chloride exposure

El policloruro de vinilo (PVC) es el polímero que ocupa el tercer puesto en el mercado de producción de plásticos a nivel mundial. Como consecuencia de la exposición crónica, los operarios pueden desarrollar cambios óseos degenerativos, Raynaud, trastornos circulatorios en extremidades, trombocitopenia y lesiones cutáneas semejantes a esclerodermia; esto se conoce como “enfermedad por cloruro de vinilo”. Presentamos un paciente masculino de 24 años de edad que presenta fenómeno de Raynaud, cefaleas, malestar en manos y pies, sensación de frío, fatiga y pérdida de apetito asociado a exposición a policloruro de vinilo. El estudio de la microcirculación cutánea periungueal por videocapilaroscopía muestra alteraciones estructurales y funcionales características. Se recomienda un seguimiento multidisciplinario estricto de los trabajadores expuestos a PVC.

Síndrome de Sharp o conectivitis mixta / Sharp syndrome or mixed connectivitis

El Síndrome de Sharp es una conectivitis en cuyo cuadro clínico se combinan manifestaciones de varias de las mesenquimopatias más frecuentes. Entre sus principales síntomas destacan el Síndrome de Raynaud; las poliartralgias de manos y las mialgias. El Síndrome de Sharp, generalmente no compromete la piel ni las vísceras y entre ellas especialmente el riñón. En el aspecto inmunológico, es característica la presencia, en el suero, y a título elevados de anticuerpos anti RNP. El pronóstico del Síndrome de Sharp es habitualmente benigno y se describe incluso evoluciones regresivas.

Pulmonary hypertension not a major feature of early mixed connective tissue disease: a prospective clinicoserological study.

BACKGROUND: Mixed connective tissue disease (MCTD) has features common to lupus, scleroderma and myositis with high levels of antibodies to U1 ribonucleoprotein (U1 RNP). Identification of a high incidence of pulmonary artery hypertension (PAH) has changed its prospect. We report the largest series from India. SETTINGS AND DESIGN: Rheumatology unit of a tertiary care centre in India; prospective. MATERIALS AND METHODS: Patients seen between January 2002 and June 2004, satisfying the Kasukawa criteria were enrolled. All patients had a complete laboratory work-up including pulmonary function test, 2-D echocardiography, and Schirmer's test, antinuclear antibodies (ANA) and antibodies to extractable nuclear antigens. HRCT of chest was done where indicated. All patients were given standard treatment and followed up regularly. RESULTS: Out of 1500 patients, thirteen (one male) were diagnosed to have MCTD. The median follow-up period was 18 months [Interquartile range (IQR) 12-22]. The median age of onset of symptoms was 36 years (IQR 22-39) and the median duration of disease was three years (IQR 1.75-4). The most common manifestation was polyarthritis followed by puffy fingers. Sjogren's syndrome, dysphagia and interstitial lung disease, was present in four, three and two patients respectively. Two patients each had myositis and migraine. None had PAH, serositis or renal involvement. Arthritis, puffy fingers and Raynaud's phenomenon were the most common manifestations at onset. All patients were positive for ANA and anti U1 RNP. Two patients each had antibodies to Sm and SSA. Response to treatment also was noted. CONCLUSION: Pulmonary artery hypertension is not common in early MCTD.

Severe systemic effects of Merrem's hump-nosed viper bite.

OBJECTIVE: To report unpredictable severe systemic effects of hump-nosed viper envenomation. SETTING: Medical unit, General hospital, Anuradhapura. METHODS: The clinical outcome of seven patients bitten by Merrem's hump-nosed viper were monitored until recovery or death. Limited autopsies were performed on the latter. Offending snakes were positively identified by medical officers and in one instance by a herpetologist. CONCLUSION: Merrem's hump-nosed viper bites, caused an array of potentially fatal systemic manifestations. One patient developed neurological effects, severe Raynaud's syndrome leading to ascending gangrene of distal limbs and adult repiratory distress syndrome (ARDS), in addition to the known complications of severe renal cortical necrosis and haemostatic dysfunction. Two patients who developed acute renal failure and prolonged coagulopathy recovered completely. The combination of extensive renal cortical necrosis, disseminated intravascular coagulation, and ARDS proved fatal in three.

Raynaud's phenomenon and gangrene following snake envenomation.

Local necrosis and gangrene at the site of the bite are commonly observed after snake envenomation. However, Raynaud's phenomenon and gangrene occurring in a limb other than that bitten by the snake is encountered rarely. So far, there is only one report of such an episode in literature. Here we are reporting a case of patient with snake bite on the right foot following which he developed Raynaud's phenomenon of the left upper limb and dry gangrene of the tips of the left index and middle fingers. The snake was identified as Russel's viper.

Raynaud miocárdico en esclerosis sistémica / Myocardial raynaud in systemic sclerosis

En un estudio prospectivo evaluamos 28 pacientes con Esclerosis Sistémica con Ecocardiografía bidimensional y un test de frío. Durante el test de frío encontramos zonas de hipoquinesia (16 casos) o de aquinesia ventricular (3 casos), transitorias y reversibles luego de cesar el estímulo frío. Estos cambios de la función ventricular inducido por el test de frío, son compatibles con vasoespasmo coronario: "Raynaud miocárdico". Este fenómeno se demostró en 9 de los 10 casos con formas difusa de ES y en 9 de 18 con la variedad más localizada de Acroesclerosis (p < 0.05)

Doença adjuvante humana após injeçäo de parafina e silicone: primeiro relato no Brasil / Human adjuvant disease after injection of paraffin and silicone: first report in Brazil

A estimulaçäo antigênica provocada pela injeçäo de substâncias estranhas, como a parafina líquida ou o silicone, pode desencadear um espectro de manifestaçöes auto-imunes. As colagenoses, mais freqüentemente a esclerodermia, estäo entre as mais descritas. Quando estäo presentes alguns elementos clínico-laboratoriais, mas näo há dados suficientes para definir uma colagenose, surge a entidade denominada doença adjuvante humana. É descrito um caso que apresentou poliartrite, fenômeno de Raynaud e púrpura higerglobulinêmica como complicaçäo de injeçäo prévia de parafina e silicone em procedimento estético para as mamas

Comprometimento esofagiano na esclerodermia: registro de um caso de longa sobrevida / Esophageal involvement in systemic scleroderma: report of a case the long survical

Os autores relatam o registro de uma paciente portadora de esclerose sistêmica progressiva que após 30 anos de doença na forma cutânea, estacionária, passa a apresentar sinais de comprometimento sistêmico, especificamente fenômeno de Raynaud e disfagia, caracterizando forma de evoluçäo banigna e conseqüente sobrevida muito além da média