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)

Una paciente con fenómeno de Raynaud del pezón e hipertiroidismo / A patient with Raynaud's phenomenon of the nipple and hyperthyroidism

Introducción: el fenómeno de Raynaud del pezón es una patología poco frecuente. Puede presentarse asociada a hipertiroidismo o enfermedades autoinmunes del tejido conectivo. Presentamos un caso asociado a hipertiroidismo.Caso clínico: mujer, de 39 años, que consulta por dolor en pezón que se agrava con la lactancia 1 mes después del parto. Se diagnosticó fenómeno de Raynaud del pezón, que mejoró con la toma de nifedipino. Tres meses después, la paciente presentó fiebre. El análisis de sangre mostró hormona estimulante del tiroides (TSH) 0,0008 mU/L (normal: 0,55-4,75 mU/L) y T4 libre 48 pg/mL (normal: 2,30-4,20 pg/mL). Los anticuerpos antitiroglobulina fueron > 500 UI/mL. La T3, los anticuerpos antiperoxidasa (TPO) y la inmunoglobulina estimulante del tiroides fueron normales. Se diagnosticó tiroiditis posparto (TPP). Dos meses después, los niveles de TSH y T4 libre volvieron a la normalidad.Conclusión: nuestra paciente presenta una TPP asociada a un fenómeno de Raynaud.(AU)

Introduction: Raynaud's phenomenon of the nipple is a rare pathology. It can occur associated with hyperthyroidism or autoimmune connective tissue diseases.We report a case associated with hyperthyroidism.Case study: a 39-year-old woman consulted for nipple pain, which worsened with breastfeeding, one month after childbirth. Raynaud's phenomenon of the nipple was diagnosed, which improved with nifedipine. Three months later the patient developed fever. Blood test revealed thyroid stimulating hormone (TSH) 0.0008 mU/L (normal 0.55-4.75 mU/L) and free T4 48 pg/mL (normal 2.30-4.20 pg/mL). Antithyroglobulin antibodies were >500 IU/mL. T3, antiperoxidase antibodies (TPO), and thyroid-stimulating immunoglobulin were normal. Postpartum thyroiditis (PPT) was diagnosed. Two months later, TSH and free T4 levels returned to normal.Conclusion: our patient presented PPT associated with Raynaud's phenomenon.(AU)

Tratamiento con oxígeno hiperbárico en el fenómeno de Raynaud y las úlceras digitales asociadas a esclerosis sistémicas / Treatment of Raynaud Phenomenon and Ischemic Ulcers Associated to Systemic Sclerosis with Hyperbaric Oxygen

Presentamos 4pacientes con fenómeno de Raynaud asociado a esclerosis sistémica, 3de ellos con úlceras isquémicas, con intolerancia o falta de respuesta a tratamiento convencional, que presentaron mejoría tras tratamiento con oxígeno hiperbárico. Esta terapia ha sido utilizada para el tratamiento de úlceras de diversa etiología debido a su efecto cicatrizante, angiogénico, antiinflamatorio y antimicrobiano. El oxígeno hiperbárico podría constituir una alternativa terapéutica en pacientes con fenómeno de Raynaud o úlceras isquémicas de difícil manejo.(AU)

We describe 4patients with Raynaud's phenomenon associated with systemic sclerosis, 3with ischaemic ulcers, successfully treated with hyperbaric oxygen. This therapy has been useful in the treatment of chronic wounds due to its anti-inflammatory, antimicrobial and angiogenic effects. Hyperbaric oxygen treatment could be a therapeutic option in patients with Raynaud's phenomenon refractory to conventional treatment.(AU)

Treatment of Raynaud phenomenon and ischemic ulcers associated to systemic sclerosis with hyperbaric oxygen.

We describe 4 patients with Raynaud's phenomenon associated with systemic sclerosis, 3 with ischaemic ulcers, successfully treated with hyperbaric oxygen. This therapy has been useful in the treatment of chronic wounds due to its anti-inflammatory, antimicrobial and angiogenic effects. Hyperbaric oxygen treatment could be a therapeutic option in patients with Raynaud's phenomenon refractory to conventional treatment.

Cáncer de mama triple negativo asociado a dermatomiositis paraneoplásica: Reporte de caso y revisión de la literatura / Triple-negative breast cancer associated with paraneoplastic dermatomyositis: Case report and literature review

Resumen La dermatomiositis (DM) es un tipo de miopatía inflamatoria bien definida, inmunomediada, con afectación específica del músculo esquelético y con compromiso variable de piel y otros órganos. Se caracteriza por debilidad muscular proximal, lesiones cutáneas patognomónicas de dermatomiositis como el signo de Gottron, eritema violáceo o heliotropo, y evidencia de inflamación muscular por enzimas elevadas, cambios miopáticos en electromiografía y biopsia muscular anormal. Tiene una asociación bien establecida con diferentes tipos de cáncer pero es rara su asociación con cáncer de mama. Cuando se presentan de manera concomitante, su diagnóstico requiere un estudio multidisciplinario para orientar el origen paraneoplásico frente a una etiología propiamente autoinmune que requiera terapia inmunosupresora dirigida. Describimos el caso de una paciente con diagnóstico simultáneo de carcinoma infiltrante de mama triple negativo y criterios de dermatomiositis como manifestación paraneoplásica.

Abstract Dermatomyositis (DM) is a well-defined immune-mediated inflammatory myopathy, with specific involvement of skeletal muscle and variable involvement of skin and other organs. It is characterized by proximal muscle weakness, pathognomonic skin lesions of dermatomyositis such as Gottron's sign, violaceous or heliotrope rash, and evidence of muscle inflammation due to elevated enzymes, myopathic changes on electromyography, and abnormal muscle biopsy. It has a well-established association with different types of cancer, but its association with breast cancer is rare. When they occur concomitantly, their diagnosis requires a multidisciplinary study to confirm the paraneoplastic origin versus a primarily autoimmune etiology that may require targeted immunosuppressive therapy. We describe the case of a patient with a simultaneous diagnosis of triple-negative infiltrating breast carcinoma and criteria for dermatomyositis as a paraneoplastic manifestation.

Hallazgos capilaroscópicos y características clínicas en pacientes con fenómeno de Raynaud primario y secundario / Capillaroscopic findings and clinical characteristics in patients with primary and secondary Raynaud's phenomenon

Objetivos: describir hallazgos de videocapilaroscopía (VCP) en pacientes con fenómeno de Raynaud primario (FRP) y secundario (FRS); comparar características demográficas y clínicas entre ambos. Materiales y métodos: estudio observacional, analítico, transversal. Se documentaron edad, ocupación, tiempo de evolución del FR, enfermedad del tejido conectivo (ETC) y características capilaroscópicas. Las VCP se informaron como patrón normal, inespecífico o SD temprano, activo y tardío. Se realizó estadística descriptiva. Para variables categóricas se empleó Chi² o test exacto de Fisher; para variables continuas, t test o Man Whitney, considerando estadísticamente significativa p<0,05. Resultados: se realizaron 290 VCP. En pacientes con FRP (n:122), 18% (n:23) fue normal y 81% (n:99) con patrón inespecífico. En pacientes con FRS (n:168), 8% fue normal, 42% con patrón inespecífico y 51% con patrón SD (25% temprano, 44% activo, 31% tardío). Se hallaron diferencias estadísticamente significativas: tiempo de evolución de FR en meses (12 vs 36, p<0,01), VCP normal (18,85% vs 7,4%, p<0,01), patrón inespecífico (81,14% vs 41%, p<0,01) en pacientes con FRP vs. FRS. Conclusiones: en pacientes con FRS predominó el patrón SD, mientras que en aquellos con FRP fue superior el patrón normal e inespecífico. El FRS se asoció a mayor tiempo de evolución.

Objectives: to describe videocapillaroscopy (VCP) findings in patients with primary Raynaud's phenomenon (PRP) and secondary (SRP); compare demographic and clinical characteristics between both. Materials and methods: observational, analytical, cross-sectional study. Age, occupation, evolution time of RP, connective tissue disease (CTD) and capillaroscopic characteristics were documented. The VCP were reported as normal, nonspecific or early, active, and late SD pattern. Descriptive statistics were performed. Chi² or Fisher's exact test were used for categorical variables; for continuous variables t test or Man Whitney, considering statistically significant p<0.05. Results: 290 VCP were performed. In patients with PRP (n:122), 18% (n:23) were normal and 81% (n:99) non-specific. In patients with SRP (n:168), 8% were normal, 42% non-specific and 51% with SD pattern (25% early, 44% active, 31% late). We found statistically significant differences: time of evolution of RP in months (12 vs. 36, p<0.01), normal VCP (18.85% vs 7.4%, p<0.01), non-specific pattern (81.14% vs 41%, p<0.01) in patients with PRP vs SRP. Conclusions: in patients with FRS predominated the SD pattern, while in those with FRP the normal and nonspecific pattern was superior. FRS was associated with a longer evolution time.

Treatment of Raynaud Phenomenon and Ischemic Ulcers Associated to Systemic Sclerosis with Hyperbaric Oxygen. / Tratamiento con oxígeno hiperbárico en el fenómeno de Raynaud y las úlceras digitales asociadas a esclerosis sistémicas.

We describe 4patients with Raynaud's phenomenon associated with systemic sclerosis, 3with ischaemic ulcers, successfully treated with hyperbaric oxygen. This therapy has been useful in the treatment of chronic wounds due to its anti-inflammatory, antimicrobial and angiogenic effects. Hyperbaric oxygen treatment could be a therapeutic option in patients with Raynaud's phenomenon refractory to conventional treatment.

Validación del Score de Condición de Raynaud en Argentina / Validation of Raynaud's Condition Score in Argentina

Objetivo: El objetivo de nuestro estudio fue adaptar y validar el Score de Condición de Raynaud (SCR) en pacientes con Esclerosis Sistémica (SSc) que concurren a un hospital público de Argentina. Materiales y Métodos: Para la adaptación, reumatólogos tradujeron al español la versión original en inglés. Para evaluar la validez de constructo se utilizó: Cuestionario de Capacidad Funcional HAQ (HAQ), Índice Duruöz (ID), validados al español para Argentina, Escala Visual Análoga (EVA) de Raynaud por un experto y Score de Rodnan modificado (mRSS). Para evaluar reproducibilidad, se evaluó de forma aleatoria un subgrupo de pacientes sin mediar cambios en el tratamiento ni en la condición clínica 10 días después de la evaluación basal. Resultados: Se incluyeron 35 pacientes con diagnóstico de SSc. La correlación entre SCR y EVA del médico fue de 0.89; SCR y HAQ 0.58; SCR y mRSS 0.61; SCR e ID 0.57 indicando una muy buena correlación principalmente con el EVA del médico y siendo todos estadísticamente significativos. La reproducibilidad fue de 0.998. Conclusiones: Los resultados muestran que el SCR es una herramienta confiable y válida para esta población argentina con SSc.

Objetive: The aim of our study was to adapt and validate the Raynaud's Condition Score (RCS) in patients with Systemic Sclerosis (SSc) who attend a public hospital in Argentina. Materials and Methods: For adaptation, rheumatologists translated to Spanish the original version in English. To assess the construct validity we used: Health Assesment Questionnaire (HAQ), Duruöz´s Hand Index (DHI), spanish validation for Argentina, Raynaud Visual Analogue Scale (VAS) by an expert and Modified Rodnan skin score (mRSS). To assess reproducibility, a subgroup of patients was randomly evaluated with no changes in treatment or clinical condition ten days after the baseline evaluation. Results: A total of 35 patients with SSc were included. The correlation between RCS and Raynaud VAS by an expert was 0.89; RCS and HAQ 0.58; RCS and mRSS 0.61; RCS and DHI 0.57 indicating a very good correlation mainly between the studied Score and the Raynaud VAS and being all statistically significant. The reproducibility was 0.998. Conclusion: The results show that the RCS is a reliable and valid tool for this argentinian population with SSc.

Is Botulinum Toxin Useful in Systemic Sclerosis Related Peripheral Vasculopathy? A Literature Review / ¿Es la toxina botulínica útil en la vasculopatía periférica de la esclerosis sistémica? Una revisión sistemática

Introduction: In systemic sclerosis (SSc), peripheral vasculopathy presents typically as Raynaud Phenomenon (RP) and Digital Ulceration (DU). Over the last decade, botulinum toxin (BT) has been reported effective in this scenario. Our goal was to review existing literature evaluating the efficacy of BT on RP/DU in SSc. Materials and methods: We performed a search in Pubmed with the MeSH terms “systemic sclerosis” and “botulinum toxin”. Original studies evaluating BT in the treatment of SSc-associated RP/DU were considered for inclusion. Results were screened by title, abstract and full-text. Results: We identified 30 results, of which 5 original papers were included: 2 randomized controlled trials (RCT), 2 case series and 1 case control study, from a total of 133 patients. Only one RCT showed negative results, with worse blood flow in treated arm, but with lower dose of BT. Despite this, all 5 included studies reported improvement of at least 1 RP/hand function outcome measure. Concerning DU healing, resolution of baseline DU at the end of follow-up was reported in 75–100% of the patients, with 1 RCT showing superiority over placebo. The only reported adverse effect was transient hand weakness, affecting only 0–16.7% of patients. BT injection protocols were highly heterogeneous. Conclusion: Despite conflicting results in 1 RCT, evidence points BT as an option in the treatment of SSc-related peripheral vasculopathy. However, future larger prospective trials are necessary to corroborate this hypothesis.(AU)

Introducción: En la esclerosis sistémica (ES), la vasculopatía periférica se presenta normalmente como fenómeno de Raynaud (FR) y ulceración digital (UD). En el último decenio se ha reportado la efectividad de la toxina botulínica (TB) en este escenario. Nuestro objetivo fue revisar la literatura existente que evalúa la eficacia de la TB en el FR/UD en la ES. Materiales y métodos: Realizamos una búsqueda en Pubmed con los términos MeSH «esclerosis sistémica» y «toxina botulínica». Se consideraron para inclusión los estudios originales que evaluaban la TB en el tratamiento del FR/UD asociados a ES. Se cribaron los resultados por título, resumen y texto completo. Resultados: Identificamos 30 resultados, de los cuales se incluyeron 5 documentos originales: 2 ensayos controlados aleatorizados (ECA), 2 series de casos y un estudio de control de caso, de un total de 133 pacientes. Únicamente un ECA reflejó resultados negativos con peor flujo sanguíneo en el brazo tratado, aunque con menor dosis de TB. A pesar de ello, los 5 estudios incluidos reportaron una mejora de al menos una medida del resultado FR/función de la mano. En cuanto a la sanación de la UD, la resolución de la UD basal al final del seguimiento se reportó en el 75-100% de los pacientes, y un ECA reflejó superioridad con respecto al placebo. El único efecto adverso reportado fue debilidad transitoria en la mano, que afectó únicamente al 0-16,7% de los pacientes. Los protocolos de inyección de la TB fueron altamente homogéneos. Conclusión: A pesar de los resultados conflictivos en un ECA, la evidencia apunta a la TB como opción para el tratamiento de la vasculopatía periférica asociada a la ES. Sin embargo, son necesarios ensayos prospectivos futuros más amplios para corroborar esta hipótesis.(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.

Is Botulinum Toxin Useful in Systemic Sclerosis Related Peripheral Vasculopathy? A Literature Review.

INTRODUCTION: In systemic sclerosis (SSc), peripheral vasculopathy presents typically as Raynaud Phenomenon (RP) and Digital Ulceration (DU). Over the last decade, botulinum toxin (BT) has been reported effective in this scenario. Our goal was to review existing literature evaluating the efficacy of BT on RP/DU in SSc. MATERIALS AND METHODS: We performed a search in Pubmed with the MeSH terms "systemic sclerosis" and "botulinum toxin". Original studies evaluating BT in the treatment of SSc-associated RP/DU were considered for inclusion. Results were screened by title, abstract and full-text. RESULTS: We identified 30 results, of which 5 original papers were included: 2 randomized controlled trials (RCT), 2 case series and 1 case control study, from a total of 133 patients. Only one RCT showed negative results, with worse blood flow in treated arm, but with lower dose of BT. Despite this, all 5 included studies reported improvement of at least 1 RP/hand function outcome measure. Concerning DU healing, resolution of baseline DU at the end of follow-up was reported in 75-100% of the patients, with 1 RCT showing superiority over placebo. The only reported adverse effect was transient hand weakness, affecting only 0-16.7% of patients. BT injection protocols were highly heterogeneous. CONCLUSION: Despite conflicting results in 1 RCT, evidence points BT as an option in the treatment of SSc-related peripheral vasculopathy. However, future larger prospective trials are necessary to corroborate this hypothesis.

Capillaroscopy: A Valuable Diagnostic Tool. / Capilaroscopia: una herramienta diagnóstica valiosa.

Capillaroscopy produces in vivo images of skin microcirculation. It is a simple, noninvasive tool for analyzing microvascular abnormalities and, as such, can be used in both adults and children. Capillaroscopy has proven useful in many diseases, but it is of particular value in Raynaud phenomenon and other autoimmune diseases. The test is used to analyze capillaroscopic patterns in the nailfold bed of the second to fifth fingers of each hand. A normal capillaroscopic pattern is characterized by the presence of 7 to 11 capillaries in a hairpin shape. Pathologic patterns are characterized by morphologic and structural alterations, such as ectasias, giant capillaries, pathologic hemorrhages, avascular areas, and neoangiogenesis. In this article, we review the fundamentals of capillaroscopy, with an emphasis on the technique and its indications, diagnostic value, and use as a prognostic tool for rheumatologic disorders.

Retrospective view of primary Raynaud's phenomenon in childhood.

OBJECTIVES: Primary Raynaud's phenomenon (PRP) manifests as episodes of transient spasms of peripheral blood vessels. To elucidate the clinical clues and laboratory characteristics will facilitate the identification of PRP. METHODS: A retrospective data collection of clinical and laboratory characteristics of 58 children with PRP was performed between January 2007 and December 2016. RESULTS: A positive ANA test at lower titers <1:100 was detected in 24.1% of the patients. There was a significant relationship between presence of ANA positivity and migraine in female patients with PRP (p=0.01; p=0.020 respectively). The most common accompanying disorder was migraine which was detected in 37.9% of all patients with PRP. Hemoglobin and serum ferritin levels were significantly lower in PRP patients with migraine (p=0.045; p<0.05, respectively). Additionally, the mean platelet volume (MPV) measurements were significantly higher in patients with migraine compared to those without migraine (p=0.045; p<0.05 respectively). DISCUSSION: There is limited data concerning childhood PRP. For the first time we showed a high frequency of migraine in childhood PRP. Anemia and high MPV could be the underlying triggering factors of these two episodic diseases.

Autoimmune associations in a Mexican cohort with primary biliary cholangitis. / Asociaciones autoinmunes en una cohorte mexicana con colangitis biliar primaria.

INTRODUCTION: Several groups have reported associations of primary biliary cholangitis with other autoimmune entities, particularly Sjögren's syndrome and hypothyroidism. Its prevalence and characteristics in Mexican patients is unknown. AIM: To determine the frequency and characteristics of autoimmune diseases in a Mexican cohort of patients with primary biliary cholangitis. MATERIALS AND METHODS: The medical records of patients that presented with primary biliary cholangitis within the time frame of 2005 and 2012 were reviewed and assessed for other autoimmune diseases. RESULTS: Seventy-eight patients, 75 women and 3 men, were included. Their mean age was 55.8 years. Seventy-three cases had positive antimitochondrial antibodies (94.8%) and disease was confirmed in 5 through liver biopsy. Five patients (8%) had anti-smooth muscle antibodies and 55/78 (70.5%) had antinuclear antibodies by indirect immunofluorescence. Forty-nine patients (62.8%) were positive for an autoimmune disease other than primary biliary cholangitis. Among those, 20 patients had one associated disease, 14 had 2, and 15 patients had 3 concomitant diseases. They included: Sjögren's syndrome in 23/78 patients (29.5%), dysthyroidism in 21/78 cases (26.9%), Raynaud syndrome in 11/78 (14.1%), CREST syndrome in 9/78 patients (11.4%), rheumatoid arthritis in 6/78 patients (7.7%), vitiligo in 5/78 (6.4%), scleroderma in 4/78 patients (5.1%), and other diseases in 8 patients. In 12/78 patients (15.4%), there was a documented family background of autoimmune disease. CONCLUSIONS: The presence of autoimmune associations in our cohort was frequent, and similar in characteristics to the information reported by other groups. The clinical implications of those findings remain to be determined.

Síndrome vibratorio mano-brazo: revisión literaria / Hand-arm vibratory syndrome: literary review

Resumen El síndrome vibratorio mano-brazo forma parte de la categoría de enfermedades ocupacionales o asociadas al trabajo, específicamente aquellos trabajos manuales en los que se utilicen herramientas vibratorias, como taladros, moledoras, martillos neumáticos, sierras y cualquier otra que transmita energía vibratoria directamente a la mano y brazo del trabajador. La descripción de los primeros casos de este síndrome tuvo lugar hace ya más de un siglo, y con el desarrollo industrial se volvió progresivamente, en una entidad más importante en cuanto a la discapacidad y la pérdida de calidad de vida que genera en los pacientes, así como las pérdidas económicas y de horas laborales que produce a nivel mundial, en especial en los países más desarrollados en donde su prevalencia es notablemente mayor. En el presente artículo se ha realizado una revisión literaria acerca de los principales signos y síntomas de esta enfermedad, su clasificación, fisiopatología y métodos de diagnóstico. También se abordarán los mecanismos de prevención así como el pronóstico y evolución de los pacientes una vez han sido diagnosticados.

Abstract Hand-arm vibration syndrome is part of the occupational or work-related illness category, specifically those manual workers using vibratory tools such as drills, grinders, pneumatic hammers, saws and any other tool that transmits vibratory energy directly to the Hand and arm of the worker. The description of the first cases of this syndrome took place more than a century ago, and with the industrial development it became progressively, in a more important entity as far as the disability and the loss of quality of life that generates in the patients, As well as the economic losses and hours of work that it produces worldwide, especially in the more developed countries where its prevalence is significantly higher. In this article a literary review has been carried out on the main signs and symptoms of this disease, its classification, pathophysiology and diagnostic methods. The mechanisms of prevention as well as the prognosis and evolution of the patients once they have been diagnosed will also be addressed.

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.

Undifferentiated connective tissue disease and interstitial lung disease: Trying to define patterns. / Enfermedad indiferenciada del tejido conectivo y enfermedad pulmonar intersticial: intentando definir patrones.

OBJECTIVES: To identify clinical or immunological features in patients with undifferentiated connective tissue disease (UCTD) associated interstitial lung disease (ILD), in order to group them and recognize different functional and high resolution computed tomography (HRCT) behavior. METHODS: Retrospective cohort study. Patients meeting Kinder criteria for UCTD were included. We defined the following predictive variables: 'highly specific' connective tissue disease (CTD) manifestations (Raynaud's phenomenon, dry eyes or arthritis), high antinuclear antibody (ANA) titer (above 1: 320), and 'specific' ANA staining patterns (centromere, cytoplasmic and nucleolar patterns). We evaluated the following outcomes: change in the percentage of the predicted forced vital capacity (FVC%) during the follow-up period, and HRCT pattern. RESULTS: Sixty-six patients were included. Twenty-nine (43.94%) showed at least one 'highly specific' CTD manifestation, 16 (28.57%) had a 'specific' ANA staining pattern and 29 (43.94%) high ANA titer. Patients with 'highly specific' CTD manifestations were younger (mean [SD] 52 years [14.58] vs 62.08 years [9.46], P<.001), were more likely men (10.34% vs 48.65%, P<.001) and showed a smaller decline of the FVC% (median [interquartile range] 1% [-1 to 10] vs -6% [-16 to -4], P<.006). In the multivariate analysis, the presence of highly specific manifestations was associated with improvement in the FVC% (B coefficient of 13.25 [95% confidence interval, 2.41 to 24.09]). No association was observed in relation to the HRCT pattern. CONCLUSION: The presence of 'highly specific' CTD manifestations was associated with female sex, younger age and better functional behavior. These findings highlight the impact of the clinical features in the outcome of patients with UCTD ILD.

Esclerosis sistémica cutánea difusa con fibrosis pulmonar

La Esclerosis Sistémica Cutánea Difusa (ESCD) es una enfermedad del tejido conectivo multisistémica que afecta la piel y órganos internos. La Enfermedad Pulmonar Intersticial (EIP) se presenta en un 70 a 80% de los casos y aproximadamente un cuarto presentan Fibrosis Pulmonar, lo que es muy raro en la edad pediátrica. La enfermedad pulmonar es una de las principales causas de muerte en los pacientes con Esclerosis Sistémica. Se presenta el caso de una paciente con Esclerosis Sistémica Cutánea Difusa asociada a Fibrosis Pulmonar. El diagnóstico y tratamiento temprano de esta entidad permite prolongar y mejorar la calidad de vida de estos pacientes

Diffuse cutaneous systemic sclerosis is a connective tissue disorder that involve skin and internal organs. Interstitial lung disease is present in 70 ­ 80% of all cases and approximately one quarter with lung fibrosis, that is no common in pediatric patients. Lung disease is one of the most important cause of death in patient with systemic sclerosis. A case of diffuse cutaneous systemic sclerosis with lung fibrosis is present.