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.

Incidence, Characteristics, Laboratory Findings and Outcomes in Acro-Ischemia in COVID-19 Patients.

AIM: In addition to its respiratory impact of SARS-CoV2, skin lesions of probable vascular origin have been described. This study intends to quantify the incidence of acro-ischemic lesions in COVID-19 infected adult subjects in our population, describing clinical patterns and associated findings. METHODS: All adult confirmed cases of COVID-19 infection who presented with acro-ischemic lesions and received care in our institution were prospectively enrolled up to May 15th, 2020. The variables included demographics, comorbidities, analytical parameters, clinical presentations and COVID-19 treatment. RESULTS: We enrolled 24 patients. The overall rate of acro-ischemic findings in COVID-19 patients was 1.2% [0.6% for outpatients and 2.9% for hospitalized (ICU and non-ICU patients)], but the observed incidence for acro-ischemia in ICU patients was remarkably higher (23.0%, p<0.001). We have described four different clinical patterns of acroischemia: atypical Raynaud´s phenomenon (ARP), (4); pseudo-pernio (PP), (5); severe microcirculatory ischemia with preserved pulse (SMI), (6); and dry gangrene with arteriosclerosis obliterans (AO), (9). Kendall´s τ correlation with lung disease severity was 0.877 (95% CI, 0.756 to 0.968); p<0.01). ARP individuals were predominantly female, while SMI appeared lately in elderly hospitalized subjects with better prognosis. AO occurred in patients with more comorbidity and younger than those with SMI. We observed other associated lesions of suggestive ischemic nature in other organs in all groups (15 patients of total sample). Plasma procalcitonin was significantly higher in patients who developed SMI (median and interquartile range: 9.99 (4.2, 12.3) mg/mL vs 0.26 (0.11, 0.89) mg/mL; p<0.001), and D-dimer level at hospital admission was significantly higher in AO patients (median and interquartile range: 1166 (1050, 2111) mg/L vs 502 (448, 777) mg/L; p<0.001). CONCLUSION: The observed risk for acroischemia in COVID-19 is high in ICU patients (23%). We have described four different clinical patterns of acroischemia (ARP, PP, SMI and AO) associated with lung disease severity. Authors have communicated various lesions of suggestive ischemic nature in other organs. Raynaud-like pattern is reported as a "novelty".

Primary Raynaud's phenomenon in premature infant: a case report / 中华围产医学杂志

This is a case report of preterm female neonate with Raynaud's phenomenon, who was born at 30+3 weeks of gestation. Her right upper limb completely turned pale from wrist to fingers with undetermined cause five days after birth, with weakened radial artery pulsation and lower skin temperature than the other side. After several treatment steps including right upper limb massage, keeping warm, intravenous infusion of Alprostadil and application of tropical mucopolysaccharide polysulfate cream, the affected limb gradually turned to purple and then ruddy. No abnormality was detected in the examinations for secondary Raynaud's phenomenon, therefore a primary Raynaud's phenomenon was considered. No similar symptoms recurred during hospitalization or follow-ups. Though rare, Raynaud's phenomenon should be considered if patients' hands suddenly turn to pale and then purple. The main treatments involve removing the causative factors, keeping warm and massage of the affected limb. Medications can be used to improve peripheral circulation if necessary. Long-term follow-up is needed for neonates suffered from Raynaud's phenomenon.

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.

Managing Neuropathic Pain in Multiple Sclerosis: Pharmacological Interventions.

BACKGROUND: Multiple sclerosis (MS) is a chronic inflammatory demyelinating disease of the central nervous system (CNS). Of the plethora of motor and sensory disturbances experienced by sufferers, neuropathic pain is a highly prevalent and debilitating symptom, and at present remains extremely difficult to treat. Common forms of neuropathic pain seen in MS patients include central neuropathic pain, Lhermitte's phenomenon and trigeminal neuralgia, which are all speculated to arise from specific patterns of lesion formation. OBJECTIVE: Efficacious pharmacological interventions for the treatment of neuropathic pain associated with MS are lacking, and have been largely informed by drug trials in peripheral neuropathies and spinal cord injury. METHOD/RESULTS: Neuropathic pain in MS is inadequately relieved by conventional analgesics, and first-line therapies are generally comprised of anti-depressive and anti-convulsive drugs. A range of alternatives have been proposed and tested with variable success, including cannabinoids and certain opioid analgesics. Animals with experimental autoimmune encephalomyelitis (EAE), an autoimmune model of MS, also exhibit neuropathic pain symptoms. CONCLUSION: Studies aimed at understanding the mechanisms underlying EAE-induced neuropathic pain and investigating the efficacy of novel pharmacological interventions at the animal level offer an exciting area of future research, and may inform future therapeutic options for MS-associated neuropathic pain.

Reacciones leprosas / Leprosy reactions

Resumen Entre 30-50 % de pacientes con lepra pueden llegar a presentar reacciones inmunológicas, que son eventos agudos que interrumpen el curso crónico de la lepra y son importantes debido a que pueden causar a largo plazo secuelas graves, como el daño permanente de nervios, lo que conlleva a discapacidad y deformidad. Se revisan los diferentes aspectos clónicos y terapéuticos de las reacciones lepromatosas con el fin de profundizar en el conocimiento actual de estas complicaciones de difícil diagnóstico y que son prioritarias para prevenir discapacidades.

Abstract Between 30-50% of patients with leprosy could have immune reactions, which are acute events that disrupt the chronic course of leprosy and are important because they can cause serious long-term consequences such as permanent damage nerve, leading to deformity and disability. We review the different clinical and therapeutic aspects of lepromatous reactions in order to deepen the current knowledge of these complications of a difficult diagnosis and are the priority to prevent disabilities.

Nailfold capillaroscopy microscopy - an interdisciplinary appraisal.

Nailfold capillaroscopy is a method of great diagnostic value in the differential diagnosis of primary versus secondary Raynaud´s phenomenon, of systemic sclerosis versus other so called connective tissue diseases and of additional diagnostic value in other entities. Rheumatologists, dermatologists, and angiologists in Germany have convened in an interdisciplinary working group in which they synergistically combined their expertise to develop a common nomenclature and standards for the technical performance of nailfold capillary microscopy. The article gives an overview of historical and technical aspects of capillaroscopy, morphologic findings, and disease-specific patterns. It also provides a critical appraisal of its significance in the diagnosis and sequelae of these interdisciplinarily-managed diseases including its performance in children and gives an excursion in the potential perspectives of capillaroscopy in less common indications.

Thrombotic complications after radial arterial line placement in systemic sclerosis: A case series.

OBJECTIVE: To demonstrate potential thrombotic complications after radial arterial line placement in patients with scleroderma. METHODS: This is a retrospective case series of 4 patients with scleroderma who were hospitalized in the intensive care unit (ICU) requiring invasive hemodynamic monitoring and developed severe complications after radial arterial line placement. We reviewed their medical records to assess their laboratory findings and clinical presentations. RESULTS: All 4 patients met the 2013 ACR/EULAR criteria for systemic sclerosis and had a radial arterial line placement in the setting of invasive hemodynamic monitoring. Overall, 2 of 4 patients had arterial line placement during surgery; while 1 patient had it placed for invasive blood pressure monitoring during an ICU admission for renal crisis; and 1 patient had arterial line placement during cardiac resuscitation, but before administration of vasopressor support. In all, 3 of 4 patients had major ischemic events including digital gangrene, hand auto-amputation, and below-elbow amputation. Among all, 1 patient had temporary hand ischemia with recovery of perfusion with immediate arterial line removal within 24 hours. CONCLUSIONS: Radial arterial line placement may trigger critical ischemic events in scleroderma patients. This experience suggests that placement of radial lines needs to be thoughtfully weighed prior to insertion in patients with scleroderma, and alternative options should be carefully considered.

Raynaud’s phenomenon - assessment and differential diagnoses.

Raynaud’s phenomenon (RP) is characterised by paroxysmal reversible episodes of vasospasm, usually involving peripheral small vessels of the fingers or toes and resulting in a triple-colour change starting with pallor and followed by cyanosis and erythema. Attacks are typically triggered by cold or emotional stress. The diagnosis of RP can be made on the basis of the patient’s clinical symptoms. Primary RP occurs without underlying disease and is considered a benign condition. A normal erythrocyte sedimentation rate, negative testing for antinuclear antibodies, normal nailfold capillaries and the absence of structural micro- or macrovascular damage and other diseases lead to the diagnosis of primary RP. Digital photoplethysmography and pulse contour analysis can be used as an additional tool to exclude structural macro- or microvascular disease. In contrast, secondary RP is associated with other diseases, mainly connective tissue diseases such as systemic sclerosis. If there is a suspicion of secondary RP, a thorough laboratory and vascular assessment is required to make the diagnosis of underlying disease. Acrocyanosis and erythromelalgia are additional functional vascular disorders that can be easily distinguished when patients are carefully assessed for their history and clinical symptoms.

Contribution of Nail Fold Videocapillaroscopy in Patients with Early Inflammatory Arthritis.

BACKGROUND: Early inflammatory arthritis (EIA) a condition defined by joint swelling, with or without morning stiffness, and/or swelling of metacarpophalangeal and/or metatarsophalangeal joints. Nail fold video-capillaroscopy (NVC) is important for the evaluation of microcirculation in vivo. There are limited data about the role of NVC in EIA. OBJECTIVES: To study the capillaroscopic pattern in patients with EIA. PATIENTS AND METHODS: 27 patients with EIA - 21 women, 6 men, mean age of 41.6±4.2 yrs, mean disease duration of 6.9±3.1 months. Anamnesis and clinical examination were perform to identify clinical signs associated with connective tissue diseases. All the patients were nonsmokers and had no personal medical history of diabetes or non-immune mediated occlusive vascular disease. Blood and urine samples were collected for bio-chemical and immunological evaluation. All the patients were interrogated by NVC. RESULTS: Raynaud's phenomenon was found in 9 patients, puffy fingers in 2 pts, telangiectasia in 2 pts, Sicca symptoms in 6 pts, malar rash in 2 pts, photosensitivity and psoriasis plaque in one patient. Combined information from clinical exam, NVC and immunological assessment allowed a specific diagnosis in 5 patients. CONCLUSIONS: Nail fold capillaroscopy assessment can provide further information and has diagnostic value in some cases of early arthritis. Nail fold capillaroscopy assessment contribution to the differential diagnosis in patients with early arthritis is sometimes significant, especially in poorly clinical and immunological defined cases.

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...

Bosentan in the treatment of refractory extremities ulcers in systemic sclerosis / Bosentana no tratamento de úlceras de extremidades refratárias na esclerose sistêmica

INTRODUÇÃO: Acometimento vascular é uma manifestação central da esclerose sistêmica (ES) e pode levar a complicações como úlceras, gangrena ou amputação de extremidades. Bosentana é um medicamento antagonista dos receptores da endotelina utilizado na prevenção de úlceras digitais na ES. OBJETIVO: Avaliar a eficácia de bosentana em úlceras de extremidades recorrentes e refratárias em pacientes com ES. PACIENTES E MÉTODOS: Realizamos estudo aberto e observacional em três pacientes com diagnóstico de ES provenientes do Ambulatório de ES da UNIFESP com idades de 31, 58 e 61 anos. Todas apresentavam uma ou mais úlceras de extremidades ativas que não haviam respondido ao tratamento convencional: paciente P1 com uma úlcera digital; P2 com três úlceras em membro inferior direito; e P3 com úlcera em dígito, perna, e calcanhar direitos e maléolo esquerdo. Bosentana foi administrado na dose de 62,5 mg VO duas vezes ao dia por quatro semanas, seguido por 125 mg duas vezes ao dia por mais quatro ou oito semanas. As pacientes foram avaliadas quanto ao número e ao diâmetro das úlceras em três momentos: no início deste estudo, após quatro semanas e após oito semanas. A paciente mais grave foi também avaliada após 12 semanas. RESULTADOS: Após tratamento com bosentana, todas apresentaram cicatrização ou diminuição no diâmetro das úlceras. Nenhuma paciente apresentou surgimento de novas úlceras. CONCLUSÃO: O tratamento com bosentana se mostrou eficaz na prevenção do surgimento de novas úlceras em curto prazo e na cicatrização de úlceras de extremidades em três pacientes com ES. Sugere-se assim, que a droga possa ser uma opção terapêutica nos pacientes com acometimento vascular grave.

INTRODUCTION: Vasculopathy is a hallmark of systemic sclerosis (SSc) and may lead to complications such as ischemic ulcers, necrosis or amputation of fingers or lower limbs. Bosentan is a dual endothelin receptor antagonist currently used for prevention of digital ulcers in SSc. OBJECTIVE: To evaluate the efficacy of bosentan in the treatment of recurrent and refractory extremity ulcers in patients with SSc. PATIENTS AND METHODS: An open and observational study was performed with three patients from the Rheumatology Division of UNIFESP aged 31, 58 and 61 years with diagnosis of SSc. All patients presented one or more active extremity ulcer refractory to conventional treatment. The first one (P1) presented one digital ulcer; P2 presented three ulcers on the right lower limb; and P3 presented an ulcer on the right digit, leg and heel, and on left maleolar region. Bosentan was prescribed in a dose regimen of 62.5 mg twice a day for 4 weeks, followed by 125 mg twice a day for additional 4 or 8 weeks. All patients were evaluated regarding the number and diameter of the ulcers in weeks 0, 4, and 8, and one of them in week 12 as well. RESULTS: After the treatment with bosentan all patients presented complete resolution or reduction in the diameter of the ulcers. None of the patients presented a new ulcer. CONCLUSION: Bosentan was an effective treatment in refractory extremities ulcers and in the prevention of new ulcers in three SSc patients suggesting that this medication could be an option for patients with severe vascular involvement.

Coexistencia de psoriasis y vitiligo / Coexistence of psoriasis and vitiligo

Se presenta el caso de un paciente de 53 años de edad, con historia de 10 años de evolución de vitiligo y quien posteriormente, sobre estas lesiones, desarrollo psoriasis en placas, manifestandose como un fenómeno isomórfico de Koebner. En la actual recibe tratamiento con fototerapia, luz ultravioleta B de banda estrecha, con resultados satisfactorios especialmente en la psoriasis.

We present a 53-years old male with a 10 years history of vitiligo who further developed psoriatic lesions clearly representing a Koebner´s phenomenon. He is currently being treated with phototherapy and narrowband UVB with an interin satisfactory outcome especially in the psoriasis.

El papel de la capilaroscopia del lecho ungueal en reumatología / No presenta

La capilaroscopia del lecho ungueal (CLU) es un método simple, inocuo y económico para el estudio detallado de la microcirculación en una amplia gama de enfermedades o síndromes de interés reumatológico. En el fenómeno de Raynaud (FR) no solo permite un preciso estudio de la circulación capilar sino que facilita la distinción entre FR primario y secundario. Posee un excepcional valor predictivo en la individuación de anormalidades morfoestructurales en fases tempranas de la esclerosis sistémica (ES), lo cual representa una de las principales ventajas de este método de imagen. El daño microvascular es una típica característica de la esclerosis sistémica que viene representada por desorganización arquitectónica, presencia de tortuosidades, aumento del diámetro de los capilares, neoangiogénesis, hemorragias/trombosis y reducción de la densidad de los capilares. Tales cambios capilaroscópicos delinean el “patrón esclerodérmico” que a su vez es la máxima expresión de la microangiopatía esclerodérmica propiamente dicha. Anormalidades capilaroscópicas están también presentes en otras enfermedades reumáticas sistémicas como: dermatomiositis, síndrome de Sjögren, lupus eritematoso sistémico, enfermedad indiferenciada del tejido conectivo y síndrome antifosfolípido. Esta revisión tiene como objetivo remarcar las aplicaciones y los límites de la CLU y sus respectivas aplicaciones en la práctica cotidiana reumatológica.

Nailfold capillaroscopy is a simple, non-invasive, inexpensive and useful method for the analysis of microvascular abnormalities that can be found in rheumatic disorders. The well-known Raynaud’s phenomenon represents a clinical condition that should promptly lead to a microvascular analysis, in order to distinguish its primary form from the secondary. Capillaroscopy has an exceptional predictive value for the diagnosis of early systemic sclerosis and this may be the best advantage this technique can offer. Microvascular damage is a typical feature of systemic sclerosis and a great number of this patients present architectural disorganization, tortuositis, giant capillaries, neovascularization haemorrhages, loss of capIllaries and avascular areas. These capillaroscopic changes characterize the “scleroderma pattern” and reflect the scleroderma microangiopathy. Microvascular abnormalities have also been found in other systemic rheumatic diseases such as dermatomyositis, Sjögren’s syndrome, systemic lupus erythematosus, undifferentiated connective tissue disease and antiphospholipid syndrome. The aim of this review is to describe the applications and limits of naifold capillaroscopy in the rheumatological clinical practice.