Riesgo cardiovascular en pacientes con esclerosis sistémica / Cardiovascular Risk in Patients with Systemic Sclerosis

Introducción: El riesgo cardiovascular es importante en la evaluación de los pacientes con esclerosis sistémica. Objetivo: Determinar el riesgo cardiovascular en pacientes con esclerosis sistémica. Métodos: Se realizó un estudio transversal y descriptivo en pacientes protocolizados del Servicio de Reumatología, en el período de enero 2020 a enero 2022. Se recogieron variables demográficas, clínicas, y se aplicó la calculadora de riesgo cardiovascular Framingham. Resultados: Se incluyeron 105 pacientes con edad media de 48,6 ± 15,3 años, el grupo más frecuente de 50 a 59 años (36,2 por ciento), predominó el sexo femenino 92,2 por ciento el color de piel blanca (74,3 por ciento), el tiempo de evolución fue mayor a 5 años (66,7 por ciento) con una media de 10,5 ± 9,3. El valor promedio de la escala de gravedad modificada de Medsger fue 5,1 ± 2,7 y el 72,4 por ciento con afectación leve. El fenómeno de Raynaud y la fibrosis pulmonar fueron más frecuentes con un 89,5 por ciento y 55,2 por ciento. El índice de Rodnan en promedio fue de 13,1 ± 8,0 y los reactantes de fase aguda normales en la mayoría. Los factores de riesgo cardiovascular más frecuentes fueron la HTA (30,2 por ciento) y dislipidemia (19,9 por ciento). El índice de masa corporal que predominó fue de peso adecuado (54,3 por ciento). Predominó el riesgo cardiovascular bajo según score de Framingham (86 por ciento). Existieron diferencias significativas entre las medias del tiempo de evolución y el riesgo cardiovascular (10 ± 6,9 frente a 9,6 ± 8,8 frente a 16,9 ± 10,8; p = 0,032). Conclusiones: El riesgo cardiovascular en los pacientes con esclerosis sistémica fue bajo(AU)

Introduction: Cardiovascular risk is important in the evaluation of patients with systemic sclerosis. Objective: To determine the cardiovascular risk in patients with systemic sclerosis. Methods: A cross-sectional and descriptive study was carried out in protocolized patients of Rheumatology Service, from January 2020 to January 2022. Demographic and clinical variables were collected, and Framingham cardiovascular risk calculator was used. Results: One hundred five patients were included with a mean age of 48.6 ± 15.3 years, the most frequent group was 50 to 59 years (36.2percent), female sex (92.2percent) predominated, as well as white skin color (74.3percent). The evolution time was greater than 5 years (66.7percent) with a mean of 10.5 ± 9.3. The average value of modified Medsger severity scale was 5.1 ± 2.7 and 72.4percent had mild involvement. Raynaud's phenomenon and pulmonary fibrosis were more common at 89.5percent and 55.2percent. Rodnan index on average was 13.1 ± 8.0 and the acute phase reactants were normal in the majority. The most frequent cardiovascular risk factors were HBP (30.2percent) and dyslipidemia (19.9percent). The predominant body mass index was adequate weight (54.3percent). Low cardiovascular risk according to Framingham score prevailed (86percent). There were significant differences between the mean duration of evolution and cardiovascular risk (10 ± 6.9 vs. 9.6 ± 8.8 vs. 16.9 ± 10.8; p = 0.032). Conclusions: The cardiovascular risk in patients with systemic sclerosis was low(AU)

Multidisciplinary Treatment for Severe Secondary Raynaud's Phenomenon: A Case Report / 中国医学科学杂志(英文版)

Raynaud's phenomenon is a symptom complex manifested as intermittent fingertip ischemia caused by cold or other sympathetic drivers. Secondary Raynaud's phenomenon is often more severe and could even lead to finger ulceration, making it particularly complicated to treat. We describe a case of severe Raynaud's phenomenon secondary to subclinical hypothyroidism lasting for more than 6 hours in a 65-year-old woman. The patient was also diagnosed with hypothyroidism, epilepsy, and secondary soft tissue infection of the right middle and ring fingers. After careful multidisciplinary consultation and discussion, the patient received vasodilation, anticoagulation, thyroxine supplementation, stellate ganglion block, hyperbaric oxygen therapy and debridement. The patient responded well to the medication, avoiding amputation or obviously dysfunction. Multidisciplinary team gathering the doctors from different departments proposes appropriate strategies for patients with severe Raynaud's phenomenon and could improve the prognosis and satisfaction of patient effectively.

La capilaroscopia en reumatología: conceptos básicos de una valiosa técnica de imágenes / The capillaroscopy in rheumatology: basics of a valuable imaging technique

The capillaroscopy is an easy and non invasive tool that allows an accurate study of the microcirculation. It has gained over the years a space in the rheumatology community, being currently a fundamental method for the diagnosis and management of connective tissue disease (CTD), in particular systemic sclerosis. Currently it is possible to demonstrate a correlation between the microvascular alterations and the diagnosis and prognosis of the ETC. In this “point of view” work we provide a practical description of the utility of capillaroscopy in the ETC describing also the characteristic pathologic findings and mentioning technical details for a correct execution of the examination...

La capilaroscopia es una técnica sencilla y no invasiva que permite un correcto y minucioso estudio de los vasos de la microcirculación. Ésta ha ido ganando a lo largo de los últimos años un espacio en la comunidad reumatológica hasta el punto de ser, actualmente, considerada un método fundamental para el diagnóstico y manejo de las enfermedades del tejido conectivo (ETC), en particular la esclerosis sistémica. Actualmente es posible establecer y demostrar una correlación entre las alteraciones capilaroscópicas y el diagnóstico y pronóstico de las ETC. En este trabajo de “punto de vista” se provee una descripción práctica de la utilidad de la capilaroscopia en las ETC, describiendo los hallazgos patológicos característicos y mencionando detalles técnicos para una correcta ejecución del examen...

Pulmonary hypertension in systemic lupus erythematosus: an independent predictor of patient survival

BACKGROUND/AIMS: We investigated whether transthoracic echocardiography-suspected pulmonary hypertension (PH) affects survival in systemic lupus erythematosus (SLE) patients and examined factors associated with PH occurrence and survival. METHODS: This retrospective single-center study included 154 Korean SLE patients fulfilling the American College of Rheumatology criteria (January 1995 to June 2013). Student t test, Mann-Whitney U test, Kaplan-Meier curves, and log-rank tests were used for comparisons. RESULTS: A total of 35 SLE patients with PH (SLE/PH+) and 119 without PH (SLE/PH-) were analyzed. Higher percentages of interstitial lung disease, Raynaud's phenomenon (RP), World Health Organization functional classification III/IV, and cardiomegaly were found in SLE/PH+ compared to SLE/PH-. Furthermore, the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index was significantly higher in SLE/PH+ (2.46 +/- 1.245 vs. 1.00 +/- 1.235), whereas survival rates were significantly higher in SLE/PH- in log-rank tests (p = 0.001). In multivariate analysis, the adjusted mortality hazard ratio (HR) for SLE/PH+ patients was 3.10. Subgroup analysis demonstrated a higher percentage of lupus nephritis in the SLE/PH+ patients who died (p = 0.039) and low complement-3 levels (p = 0.007). In univariate analysis, the mortality HR for SLE/PH+ patients with lupus nephritis was 4.62, whereas the presence of RP decreased the mortality risk in multivariate analysis; adjusted HR, 0.10. CONCLUSIONS: PH is an independent factor predicting survival in SLE patients. The presence of lupus nephritis resulted in an increased trend for mortality, whereas coexistence of RP was associated with a better survival prognosis in SLE/PH+ patients.

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.

Comparison of the Thickness of the Lamina Cribrosa and Vascular Factors in Early Normal-tension Glaucoma with Low and High Intraocular Pressures

PURPOSE: To compare the thickness of the lamina cribrosa (LC) and vascular factors of early normal-tension glaucoma (NTG) patients with high and low intraocular pressure (IOP) that are expected to be associated with the development of glaucoma. METHODS: Seventy-one Korean NTG patients with low IOP (the highest IOP 15 mmHg, 31 patients) were included in this study. The thickness of LC and vascular factors were compared. The thickness of the LC was measured using the enhanced depth imaging method with spectral domain optical coherence tomography (Heidelberg Spectralis). RESULTS: The mean thickness of the central LC was 190.0 +/- 19.2 microm in the low IOP group and 197.8 +/- 23.6 microm in the high IOP group, but there was no statistical significant difference between the two groups (p > 0.05). The prevalence of self-reported Raynaud phenomenon was significantly higher in the low IOP group (33.0%) than the high IOP group (10.3%, p = 0.04). CONCLUSIONS: The laminar thickness did not significantly differ between the high and low IOP groups. However, the prevalence of Raynaud phenomenon was higher in the low IOP groups. These results suggest that the development of glaucoma with low IOP patients may be more influenced by peripheral vasospasm, such as Raynaud phenomenon, rather than laminar thickness in NTG.

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.

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.

Liver Cirrhosis Due to Autoimmune Hepatitis Combined with Systemic Sclerosis / 대한소화기학회지

Systemic sclerosis (SSc) is a chronic systemic disease that affects the skin, lungs, heart, gastrointestinal tract, kidneys, and musculoskeletal system. Although up to 90% of patients with scleroderma have been estimated to have gastrointestinal involvement, liver disease has been reported only rarely. A 51-year-old woman was hospitalized due to esophageal variceal bleeding. Her serum was positive for anti-nuclear antibody and anti-centromere antibody. Sclerodactyly was noted on both hands, and she had recently developed Raynaud's syndrome. Punch biopsy of the hand showed hyperkeratosis, regular acanthosis, and increased basal pigmentation in the epidermis, and thick pale collagenous bundles in the dermis. Liver biopsy showed chronic active hepatitis with bridging fibrosis. Consequently, she was diagnosed with liver cirrhosis due to autoimmune hepatitis (AIH) combined with SSc. AIH had subsided after administration of prednisolone at 40 mg per day. She received 5-10 mg/day of prednisolone as an outpatient, and her condition has remained stable. Patients with either AIH or SSc should be monitored for further development of concurrent autoimmune diseases. The early diagnosis of AIH combined with SSc will be helpful in achieving optimal management.

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.

Enfoque del paciente con fenómeno de Raynaud / Approach to patients with Raynaud's phenomenon

Raynaud's phenomenon is a common disorder with vasospasm of the digital arteries causing pallor with cyanosis and/or rubor. It can be primary (idiopathic), where it is not associated with other diseases, or secondary to several diseases or conditions, including connective tissue diseases, such as scleroderma and systemic lupus erythematosus. Capillaroscopy is the most reliable way to distinguish between primary and secondary Raynaud's phenomenon through identification of an early pattern of systemic sclerosis. Treatment is often non-pharmacological, including avoiding cold and smoking cessation. Calcium channel antagonists are often considered when treatment is needed.

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.

Usefulness of videocapillaroscopy in clinical practice: systomatic review of indications and results in rheumatology and in non rheumatic disorders

Nailfold videocapillaroscopy [VCP] is nowadays worldwide considered as one of the best diagnostic noninvasive imaging technique able to study microcirculation in vivo. To review the applications of VCP in the clinical practice and its results in rheumatic and non-rheumatic diseases. Review of literature. The possibility of managing the imaging, by means of dedicated software able to characterize quantitative and qualitative data, represents another relevant property of VCP. This technique is very useful at the identification of microvascular involvement in many rheumatic diseases, particularly in systemic sclerosis and related disorders. At the same time, VCP has been showed valuable in many other extra-rheumatic diseases. The authors review the applications of VCP in the clinical practice and its results in rheumatic and non-rheumatic diseases

O que o cirurgião-dentista deve sobre o síndrome de Raynaud / What the dentist must knows concerning Raynaud's syndrome

A síndrome, fenômeno ou doença de Raynaud, embora de patogênese desconhecida, caracteriza-se por ser uma desordem vaso espástica definida por quadro isquêmico transitório causado, principalmente,pela vaso constrição das artérias digitais, pré-capilares e plexo artériovenoso subcutâneo. As manifestações clínicas ocorrem principalmente nas mãos (dedos), porém, pode ocorrer em pés, ponta do nariz, língua e orelha, sendo que o órgão afetado pode apresentar como características, a pele branca e isquêmica, com duração variável, seguida de fase cianótica e, posteriormente, hiperêmica. O fenômeno é particularmente importante porque pode representar uma indicação de desenvolvimento futuro (média de 10,4 anos a partir do aparecimento da doença de Raynaud) de doenças secundárias como esclerose sistêmica, artrite reumatóide, dermatopolimiosite, lúpuseritematoso sistêmico, síndrome de Sjögren, entre outras. O caso relatado é de paciente do sexo feminino, 32 anos, leucoderma, com a classificação mais alta na escala de Taylor-Pelmear (estágio: 4; graduação: muito severa). A anamnese e exame físico revelaram a condição com evolução de 3 anos, com sinais e sintomas clássicos. A paciente apresentava importantes cicatrizes nas extremidades digitais de ambas as mãos, referente a quadros severos de isquemia. Exames hematológicos e sorológicos mostraram-se dentro dos limites normais e a paciente não apresentava indícios de doença secundária, entretanto, a observação em longo prazo é imperativa

Fenomeno de Raynaud / Raynaud's phenomenon

Raynaud's phenomenos is a common paroxysmal vasomotor disorder. It must be distinguised between those with a functional character- primary phenomenon or Raynaud's disease and secondary Raynaud's phenomenon or syndrome. In our study the highest incidence (81 por ciento) corresponds to patients with secondary Raynaud's syndrome; 58 por ciento of these patients showed capillary microscopy alterations and 64 por ciento were ANA test positive, showing those numbers their sensibility and specificity since primary Raynaudïs and healthy control patients did not show these figures. The clear prevalencxe of specific capillary microscopy alterations in secondary Raynaud's patients schow that this technique is very useful in the differential diagnosis between primary and secondary Raynaud's phenomenon. Serial capillaroscopy studies must then be performed to diagnose connective tissue diseases. Nailfold capillary microscopy is a very usefuld and easy complementary technique; it offers great help and also is of prognostic value in patient with Raynaud's phenomenon or connwctive tissue diseases, together with the clinical and laboratory studies contributes for the early diagnosis of these disorders

Crioglobulinemia: comunicación de un caso / Crioglobulinemia: communication of a case

Se presenta el caso clínico de una mujer de 50 años, con necrosis en sus dedos, crioglobulinemia y Síndrome de Raynaud. Se discuten los diferentes aspectos relacionados con la identificación, clasificación, y patologías asociadas a la crioglobulinemia, así como los mecanismos clínicos y patofisiológicos involucrados en esta enfermedad

Raynaud primario y secundario a síndromes esclerodermiformes: comunicación de 97 pacientes estudiados por capilaroscopía periungueal / Primary Raynaud and secondary to sclerodermoform syndromes: a communication of 97 patients studies with periungueal capilaroscopy

La detección temprana de síndromes esclerodermoformes es de gran relevancia para el pronóstico del paciente. Nuestro objetivo es determinar la utilidad de la capilaroscopía periungueal en el diagnóstico diferencial entre Raynaud primario y secundario a síndromes esclerodermiformes. Se estudian pacientes con Raynaud primario (21), Raynaud secundario a síndromes esclerodermia sistémica (57) y Raynaud secundario a síndromes de superposición con esclerodermia (19). El grupo control de sujetos sin Raynaud estudiados fue de 40 personas. Todos los grupos fueron estudiados por capilaroscopía periungueal de dedos de ambas manos por medio de microscopio esteroscópico de 40 aumentos. La especificidad del método para detección de patrón SD (esclerodermiforme) fue de 100 por ciento y la sensibilidad asciende 82,5 por ciento para esclerodermia sistémica y 57 por ciento para síndromes de superposición