Fenómeno de Raynaud en el pezón durante la lactancia: serie de casos / Fenómeno de Raynaud en el pezón durante la lactancia: serie de casos

El fenómeno de Raynaud consiste en la contracción excesiva de los vasos sanguíneos en respuesta a diversos estímulos y, si bien suele comprometer las extremidades, existen otras localizaciones menos frecuentemente afectadas. Este trabajo se enfoca en describir las características de una serie de mujeres con fenómeno de Raynaud en el pezón. Mediante revisión de historias clínicas y comunicación directa con las pacientes, se recopilaron y analizaron los datos de 12 mujeres con Raynaud del pezón entre 2016 y 2023. Se evaluaron variables como edad, síntomas, desencadenantes, tratamientos y duración de los síntomas. En esta serie de casos, el fenómeno de Raynaud del pezón en mujeres lactantes se manifestó con mayor frecuencia en primigestas alrededor del décimo día posparto; el dolor fue intenso, en la mayoría mejoró con tratamientos locales y/o farmacológicos, y no limitó la duración de la lactancia materna.

Raynaud's phenomenon consists of excessive contraction of the blood vessels in response to various stimuli; although it usually affects the extremities, other locations are less frequently involved. This study focused on describing the characteristics of a series of women with Raynaud's phenomenon of the nipple. Through medical record review and direct communication with patients, data from 12 women diagnosed with Raynaud's phenomenon of the nipple between 2016 and 2023 were collected and analyzed. The following variables were assessed: age, symptoms, triggering factors, treatment, and duration of symptoms. In this case series, Raynaud's phenomenon of the nipple in breastfeeding women was more common among primiparous women around 10 days after delivery; pain was severe and, in most cases, improved with local and/or drug treatment, and did not limit the duration of breastfeeding.

2023 Brazilian Society of Rheumatology guidelines for the treatment of systemic sclerosis.

BACKGROUND: Systemic sclerosis (SSc) is a rare chronic autoimmune disease with heterogeneous manifestations. In the last decade, several clinical trials have been conducted to evaluate new treatment options for SSc. The purpose of this work is to update the recommendations of the Brazilian Society of Rheumatology in light of the new evidence available for the pharmacological management of SSc. METHODS: A systematic review including randomized clinical trials (RCTs) for predefined questions that were elaborated according to the Patient/Population, Intervention, Comparison, and Outcomes (PICO) strategy was conducted. The rating of the available evidence was performed according to the Grading of Recommendations Assessment, Development and Evaluation (GRADE) methodology. To become a recommendation, at least 75% agreement of the voting panel was needed. RESULTS: Six recommendations were elaborated regarding the pharmacological treatment of Raynaud's phenomenon, the treatment (healing) and prevention of digital ulcers, skin involvement, interstitial lung disease (ILD) and gastrointestinal involvement in SSc patients based on results available from RCTs. New drugs, such as rituximab, were included as therapeutic options for skin involvement, and rituximab, tocilizumab and nintedanib were included as therapeutic options for ILD. Recommendations for the pharmacological treatment of scleroderma renal crisis and musculoskeletal involvement were elaborated based on the expert opinion of the voting panel, as no placebo-controlled RCTs were found. CONCLUSION: These guidelines updated and incorporated new treatment options for the management of SSc based on evidence from the literature and expert opinion regarding SSc, providing support for decision-making in clinical practice.

Raynaud's Phenomenon of the Nipple: Epidemiological, Clinical, Pathophysiological, and Therapeutic Characterization.

Raynaud's phenomenon of the nipple is a possible cause of pain and breastfeeding cessation in lactating women. However, there are still few studies on the characterization of this manifestation. Thus, we aim to develop a systematic review of the literature carried out between January 1992 and January 2024 in PubMed, Scopus, Web of Science, Virtual Health Library (VHL), and Portal de Periódicos da CAPES. Of the 438 articles, 19 met the eligibility criteria. The findings were divided by heuristic questions into two groups: "Epidemiological, pathophysiological, and clinical characterization of Raynaud's Phenomenon of the nipple" and "Treatment of Raynaud's Phenomenon of the nipple". Raynaud's phenomenon of the nipple is commonly primary, being more prevalent in the postpartum period, in women with a mean age of 32 years. The main triggers appear to be stress and temperature change. Generally, it is associated with a change in color and pain during breastfeeding. A calcium channel blocker was the most used medication with or without non-pharmacological measures.

Raynaud's phenomenon of the nipple during breastfeeding: A case series. / Fenómeno de Raynaud en el pezón durante la lactancia: serie de casos.

Raynaud's phenomenon consists of excessive contraction of the blood vessels in response to various stimuli; although it usually affects the extremities, other locations are less frequently involved. This study focused on describing the characteristics of a series of women with Raynaud's phenomenon of the nipple. Through medical record review and direct communication with patients, data from 12 women diagnosed with Raynaud's phenomenon of the nipple between 2016 and 2023 were collected and analyzed. The following variables were assessed: age, symptoms, triggering factors, treatment, and duration of symptoms. In this case series, Raynaud's phenomenon of the nipple in breastfeeding women was more common among primiparous women around 10 days after delivery; pain was severe and, in most cases, improved with local and/or drug treatment, and did not limit the duration of breastfeeding.

El fenómeno de Raynaud consiste en la contracción excesiva de los vasos sanguíneos en respuesta a diversos estímulos y, si bien suele comprometer las extremidades, existen otras localizaciones menos frecuentemente afectadas. Este trabajo se enfoca en describir las características de una serie de mujeres con fenómeno de Raynaud en el pezón. Mediante revisión de historias clínicas y comunicación directa con las pacientes, se recopilaron y analizaron los datos de 12 mujeres con Raynaud del pezón entre 2016 y 2023. Se evaluaron variables como edad, síntomas, desencadenantes, tratamientos y duración de los síntomas. En esta serie de casos, el fenómeno de Raynaud del pezón en mujeres lactantes se manifestó con mayor frecuencia en primigestas alrededor del décimo día posparto; el dolor fue intenso, en la mayoría mejoró con tratamientos locales y/o farmacológicos, y no limitó la duración de la lactancia materna.

Association of neutrophil extracellular trap levels with Raynaud's phenomenon, glomerulonephritis and disease index score in SLE patients from Brazil.

Neutrophil extracellular traps (NETs) are cell-extruded DNA strands coated with neutrophils' nuclear proteins and enzymes from cytotoxic granules, produced by NETosis, a cell death pathway. They perform an important defensive role in innate immunity, but their increased production and/or inefficient degradation expose new antigens, such as DNA or citrullinated histone peptides, triggering autoimmunity. This study aimed to access possible associations between serum NETs levels with epidemiological, clinical, and serological data from a well-characterized SLE Brazilian patients' cohort. NET levels were evaluated in one hundred seventy serum samples of patients with Systemic Lupus Erythematosus (SLE) using an Immunoassay. Univariate and multivariate binary logistic regression used clinical patients' data as independent variables. Parametric and non-parametric tests compared log10 base serum NET levels transformed between patients' groups. SLE patients were also dichotomized into "High serum NET levels" and "Low serum NET levels" groups. All analyses were performed in R language 4.1.2, and p < 0.05 were considered significant. Increased susceptibility for high serum NET levels was observed in SLE patients with Raynaud's phenomenon (OR = 2.30, 95 % CI = 1.06-5.21 and p = 0.039), independently of any other risk factor. Also, SLE patients with Raynaud's phenomenon presented higher mean NET serum levels (mean = -0.13 vs. -0.51, p = 0.01). In addition, higher mean NET serum levels were associated with glomerulonephritis (mean = -0.45 vs. -0.12, p = 0.03). Ultimately, the SLEDAI index scored higher in the high NETs serum levels group (median = 2.0 vs. 0.0, p = 6 × 10-3). The formation of NETs might be implicated in Raynaud's phenomenon, glomerulonephritis, and disease index score in SLE patients. Our results highlight the importance of serum NET levels as a possible therapeutical target to modulate the clinical course of SLE.

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)

Adipose derived stromal vascular fraction and fat graft for treating the hands of patients with systemic sclerosis. A randomized clinical trial.

BACKGROUND: Systemic Sclerosis in the hand is characteristically evidenced by Raynaud's phenomenon, fibrosis of the skin, tendons, ligaments, and joints as well as digital ulcers with prolonged healing. Current medical treatment does not always cure these complications. Local adipose-derived stromal vascular fraction administration into the hands has been proposed as an emerging treatment due to its regenerative properties. The objective of this randomized controlled clinical trial was to evaluate the safety and clinical effects of fat micrografts plus adipose derived-stromal vascular fraction administration into the hands of patients with systemic sclerosis. METHODS: This was an open-label, monocentric, randomized controlled study. Twenty patients diagnosed with systemic sclerosis were assigned to the experimental or control group. Fat micrografts plus the adipose derived-stromal vascular fraction were injected into the right hand of experimental group patients. The control group continued to receive only medical treatment. Demographic, serologic data and disease severity were recorded. Digital oximetry, pain, Raynaud phenomenon, digital ulcers number, mobility, thumb opposition, vascular density of the nail bed, skin affection of the hand, serologic antibodies, hand function, and quality of life scores were evaluated in both groups. RESULTS: The results of the intervention were analyzed with the Wilcoxon rank test, and the differences between the control and experimental groups at 0 days and 168 days were analyzed with the Mann-Whitney U test. Adverse events were not observed in both groups. At the end of the study, statistically significant improvements were observed in pain levels (p<0.05) and number of digital ulcers (p<0.01) in the experimental vs control group. CONCLUSION: The injection of adipose derived-stromal vascular fraction plus fat micrografts is a reproducible, and safe technique. Pain and digital ulcers in the hands of patients with systemic sclerosis can be treated with this technique plus conventional medical treatment.

Tratamiento del síndrome de Raynaud con toxina botulínica tipo A / Treatment of Raynaud's syndrome with botulinum toxin type A

El fenómeno de Raynaud es un trastorno vasoespástico de las arterias digitales por la exposición al frío o al estrés, el cual genera isquemia que se manifiesta con cianosis o palidez, dolor y úlceras crónicas; es una afección incapacitante para el paciente y de difícil manejo para el cirujano de mano. Se clasifica en primario y secundario. El primario es la forma de presentación más frecuente. Las modalidades de tratamiento están dirigidas a prevenir el vasoespasmo exagerado, fomentando la respuesta vasodilatadora y con ello disminuyendo la sintomatología, que es en la mayoría de los casos incapacitante. Se ha descrito el uso de la toxina botulínica A para aquellos casos severos y refractarios, con mala evolución, con el que se obtuvieron buenos resultados. Este sería un tratamiento de baja morbilidad, con escasos efectos adversos, que puede ofrecer un alivio del dolor rápido y duradero, contribuyendo además a la cicatrización de úlceras crónicas. De esta manera mejora la calidad de vida de los pacientes y evita tratamientos invasivos y la amputación. El propósito de este trabajo fue realizar una revisión bibliográfica incluyendo a los trabajos más importantes y relevantes hasta el momento en el tratamiento de la toxina botulínica A en el síndrome de Raynaud. Se presenta además un caso clínico refractario al tratamiento médico que fue tratado en la Cátedra de Cirugía Plástica, Reparadora y Estética del Hospital de Clínicas, Montevideo, Uruguay

Raynaud's phenomenon is a vasospastic disorder of the digital arteries due to exposure to cold or stress, which generates ischemia that manifests with cyanosis or paleness, pain, and chronic ulcers; it is a disabling condition for the patient and difficult to manage for the hand surgeon. It is classified into primary and secondary. Primary is the most common form of presentation. Treatment modalities are aimed at preventing exaggerated vasospasm, promoting the vasodilator response and thereby reducing symptoms, which are disabling in most cases. The use of botulinum toxin A has been described for those severe and refractory cases, with poor evolution, with which good results were obtained. This would be a low morbidity treatment, with few adverse effects, which can offer rapid and long-lasting pain relief, also contributing to the healing of chronic ulcers. In this way, it improves the quality of life of patients and avoids invasive treatments and amputation. The purpose of this work was to carry out a bibliographic review including the most important and relevant works to date on the treatment of botulinum toxin A in Raynaud's syndrome. In addition, a clinical case refractory to medical treatment that was treated in the Chair of Plastic, Reconstructive and Aesthetic Surgery of the Hospital de Clínicas, Montevideo, Uruguay, is also presented.

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

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

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

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

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

Inflammatory myopathies overlapping with systemic sclerosis: a systematic review.

We performed a systematic review of the clinical manifestations and complementary exams of patients with myopathies and systemic sclerosis overlap syndrome (MyoSScOS). Systematic review from January 1976 to November 2021 according PRISMA protocol on three electronic databases: PubMed, Web of Science, and Scopus. Studies were analyzed based on the following eligibility criteria: at least one combination of the terms described in the search strategy appears in the title; written in English, Portuguese, or Spanish; and addresses MyoSScOS. Brief communications, reviews, studies that addressed myopathies in children, congress proceedings, monographs, and dissertations were excluded. Thirty-five articles were selected. MyoSScOS seems to be more common in women. It also commonly affects the esophagus and joints with symmetrical and bilateral muscle involvement, Raynaud's phenomenon, and impairment of forced vital capacity. Concerning SSc, the most common subtype was the diffuse form. Cardiovascular and pulmonary complications are an important cause of death. Anti-centromere, anti-PM/Scl, anti-Scl70, anti-RNA polymerase III, anti-Ku, and anti-RNP were more correlated with this entity, and muscle biopsies may present a more aggressive pattern. Electroneuromyography patterns are quite similar to those found in inflammatory myopathies. The absence of studies with robust methodologies and the large number of case reports and series make more robust statistical analyses such as meta-analyses unfeasible. The characterization of MyoSScOS is important for the formulation of therapeutic measures and specific treatments aiming at better quality of life and prognosis. Greater and better theoretical contributions are necessary to better characterize it.

Predictors of progression to systemic sclerosis: analysis of very early diagnosis of systemic sclerosis in a large single-centre cohort.

OBJECTIVE: This study analysed the very early disease of SSc (VEDOSS) characteristics in a group of 217 patients with RP and at least one manifestation of SSc in search of predictors for the progression to SSc. METHODS: This was a cross-sectional single-centre analysis of patients presenting with RP with a specific SSc clinical manifestation or SSc autoantibody or SD pattern at nailfold capillaroscopy (SD-NFC), without skin involvement, who attended a scleroderma outpatient clinic between 2010 and 2019. The performance of VEDOSS and the importance of the combination of VEDOSS characteristics to predict the progression to SSc were evaluated. RESULTS: Among 217 patients, 153 (70.5%) were classified as SSc, including 65 (30%) in the first investigation; 69.3% of the SSc patients met VEDOSS criteria compared with 6.3% of patients who did not progress to SSc. The combinations most associated with progression to SSc were RP + puffy fingers (PF) + positive ANA + SD-NFC and/or SSc-specific antibody (VEDOSS level 2), with an odds ratio (OR) of 19.52 (95% CI 4.48, 85.06; P < 0.001) and RP + PF + positive ANA (VEDOSS level 1; 'red flags') (OR 15.45; P < 0.001), while combinations without non-RP clinical symptoms, as RP + SD-NFC (OR 0.03; P < 0.001) and RP + anticentromere + SD-NFC (OR 0.06; P = 0.006) were associated with non-progression to SSc. CONCLUSION: Among patients with RP with at least one manifestation of SSc, without skin involvement, combinations of VEDOSS characteristics were the strongest predictors of progression to SSc at a median follow-up of 4 years.

Characterization of Venezuelan patients with systemic sclerosis: a study in a tertiary hospital in Caracas.

OBJECTIVE: The aim of the study was to investigate the demographic and clinical characteristics of Venezuelan patients with systemic sclerosis (SSc) seen in a tertiary hospital. METHODS: Consecutive patients 18 years and older who fulfilled the 2013 ACR/EULAR classification criteria for SSc and who were followed up in the outpatient clinic of the Division of Rheumatology at the Hospital Universitario de Caracas were selected for the study. Demographic and clinical variables were registered at the time of inclusion using a standard protocol. RESULTS: Forty-eight SSc patients were included; 46 (95.8%) were female; the mean age was 55.1±13.7 (mean±SD) years and all were of Hispanic ethnicity. Thirty-one (64.6%) had limited SSc and 17 (35.4%) had diffuse SSc. The mean duration of disease was 13.4±11.7 (mean±SD) years, 16.74±12.99 years for limited SSc and 7.52±5.25 years for diffuse SSc (p=0.0077). Raynaud's phenomenon was the most frequent manifestation (100%), followed by arthritis (68.8%), telangiectasia (60.4%), dyspnea (60.4%), dysphagia (58.3%) and puffy hands (56.3%). The modified Rodnan Skin Score (mRSS) and the frequency of dyspnea were higher in those with diffuse as compared to limited SSc (p=0.0211 and p=0.0003, respectively). We performed high-resolution computed tomography (HRCT) of the lungs in 31 patients; 14 (45.2%) had evidence of interstitial lung disease (ILD), 11 (68.8%) with diffuse SSc (p=0.0052). The most frequent anti-nuclear antibody pattern was nucleolar, accounting for 18 (42.8%) of the cases. Anti-centromere antibodies were present in 16.7% of the cases and were associated with the limited SSc subset (p=0.0443) and with calcinosis (p=0.0020). Anti-topoisomerase antibodies were associated with ILD (p=0.0077). CONCLUSIONS: Typical clinical and serological manifestations were present in this sample of Venezuelan patients with SSc, with an expected distribution according to disease subtype. The autoantibody profile allows clinicians to identify those patients with limited forms of the disease and those without pulmonary involvement.

Systemic Sclerosis: Elevated Levels of Leukotrienes in Saliva and Plasma Are Associated with Vascular Manifestations and Nailfold Capillaroscopic Abnormalities.

The role of leukotrienes (LTs) in the pathogenesis of systemic sclerosis (SSc) needs clarification. We analyzed the association of salivary (sa) and plasma (p) levels (pg/mL) of cysteinyl-leukotrienes (CysLT) and LTB4 with SSc vascular manifestations and nailfold capillaroscopy (NFC) in a cross-sectional study. Patients and healthy controls were evaluated for vascular manifestations and NFC. LTs were compared between groups as follows: SSc with or SSc without vascular features and controls, and by NFC parameters. Twenty SSc patients and 16 volunteers were recruited; Raynaud's phenomenon (RP) history (SSc: saCysLT 99.4 ± 21.8 vs. controls: 23.05 ± 23.7, p = 0.01), RP at examination (SSc: saCysLT 129.3 ± 24.6 vs. controls: 23.05 ± 22.46, p = 0.01; pCysLT SSc: 87.5 ± 11.2 vs. controls: 32.37 ± 10.75, p = 0.002), capillary loss (saCysLT 138.6 ± 26.7 vs. 23.05 ± 21.6, p = 0.0007; saLTB4 3380.9 ± 426.6 vs. 1216.33 ± 346.1, p = 0.0005), "late" scleroderma pattern vs. controls (saCysLT 205.6 ± 32 vs. 23 ± 19.6, p = 0.0002; saLTB4 4564.9 ± 503.6 vs. 1216.3 ± 308.3; p < 0.0001) were all significant. Late patterns had higher levels (saCysLT, p = 0.002; LTB4 p = 0.0006) compared to active and early patterns (LTB4, p = 0.0006), and giant capillaries (p = 0.01) showed higher levels of LTs. Levels of pCysLT were higher in patients with RP at examination vs. patients without RP; saCysLT and LTB4 were higher in SSc group with vs. without capillary loss. LTs could be involved in the pathophysiology of vascular abnormalities. Further research is required to determine if blocking LTs could be a therapeutic target for SSc vascular manifestations.

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.

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.

Novel Combined Approach for Digital Necrosis Secondary to Raynaud's Phenomenon.

The presence of severe Raynaud's Phenomenon (RP), with permanent pain or digital necrosis is a rare condition. Cervical sympathectomy or distal sympathectomy or A botulinum toxin have demonstrated efficacy after medical treatment failure. We report the case of a 38-year-old female patient with an acute onset of severe RP in both hands secondary to systemic sclerosis. Medical treatment failed, so a novel approach by a combination of a modified distal sympathectomy and injection of A botulinum toxin on digital neuromuscular bundles was performed. Remission of the pain occurred immediately after the procedure and 45 days later she had complete healing of the digital wounds and recovered full mobilization of both hands. The patient remained asymptomatic 6 month after the procedure, and a Doppler ultrasound showed tri-phasic flows distal to the surgical site. This novel technique is described, and a brief review of the literature is performed.

Ultrasound characterization of the nail bed in patients with systemic lupus erythematosus.

OBJECTIVE: To characterize the ultrasound findings of the nail plate and nail bed in systemic lupus erythematosus (SLE) and its association with nail dystrophy. METHODS: Thirty-two SLE patients, 36 patients with osteoarthritis (OA) and 20 healthy individuals were studied. High-frequency linear ultrasound was performed in nails of the second to fifth fingers in all participants. Disease activity (SLEDAI-2K index), accrued organ damage (SLICC/ACR index), autoantibody profile, and Raynaud's phenomenon were also assessed in SLE patients. RESULTS: Nail bed thickness in SLE patients was higher than in healthy individuals (1.25 ± 0.31 mm vs 1.17 ± 0.29 mm; P = 0.01) but lower than in OA (1.39 ± 0.37 mm; P < 0.001), while nail plate thickness was similar among groups. Nail dystrophy was found more frequently in SLE and OA than in healthy individuals. SLE patients with nail dystrophy were older than their counterparts with no dystrophy (39.4 ± 10.4 years vs 27.8 ± 5.6 years; P = 0.004), although nail dystrophy showed no association with SLICC/ACR, SLEDAI-2K, nail bed vascularity, or autoantibodies. CONCLUSIONS: Nail bed in SLE patients is thicker than in healthy individuals but thinner than in OA patients. Nail dystrophy in SLE is associated with advanced age, but not with accrued organ damage, disease activity, Raynaud's phenomenon, or DIP synovitis assessed by ultrasound.