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)

Atención de la úlcera de pie como manifestación clínica de la esclerosis sistémica / Managing foot ulcers as a clinical manifestation of systemic sclerosis

Las úlceras pueden aparecer sobre protuberancias óseas, como las falanges proximales o los codos, pero es más probable que sean secundarias a la piel tensa y al trauma y no respondan a la terapia vasodilatadora. Todas las úlceras son muy dolorosas. El propósito de este trabajo es presentar aspectos relacionados con la atención de las úlceras digitales en la esclerodermia, según la experiencia de diferentes autores. En reumatología, se recomienda, en todo paciente con dolor reumático crónico, calcular la intensidad del dolor, tanto para la elección primera del tratamiento analgésico como para la medición de la respuesta. Y, para ello, se han propuesto varios métodos de cuantificación. El manejo de las úlceras digitales en la esclerodermia incluye la intervención no farmacológica, farmacológica y quirúrgica. El uso de nitratos, infusiones de acetilcisteína, el gel de vitamina E, entre otros métodos mejoran el tiempo de curación en pacientes con esclerodermia. La falta de terapias aprobadas por la Administración de Alimentos y Medicamentos de los Estados Unidos para las ulceraciones digitales garantiza un enfoque y una atención renovados para encontrar opciones de tratamiento significativas. Los médicos deberán emplear un enfoque terapéutico multifacético para optimizar el tratamiento de úlceras digitales existente y evitar la formación de nuevas lesiones para proporcionar a los pacientes la mejor calidad de vida(AU)

Ulcers can occur on bony bumps, such as the proximal phalanges or elbows, but are more likely to be secondary to tight skin and trauma and not responsive to vasodilator therapy. All ulcers are characteristically very painful. To present aspects related to the management of digital ulcers in scleroderma based on the experience of different authors. The management of digital ulcers in scleroderma includes non-pharmacological, pharmacological and surgical intervention. The use of nitrates, infusions of acetylcysteine, Vitamin E Gel, among other methods, improve the healing time in patients with scleroderma. The lack of US Food and Drug Administration approved therapies for digital ulcerations ensures a renewed focus and attention on finding meaningful treatment options. Clinicians should employ a multi-faceted therapeutic approach to optimize existing digital ulcers treatment and avoid new lesion formation to provide patients with the best quality of life(AU)

Esclerosis sistémica y neoplasias, a propósito de un estudio en la provincia de Holguín / Systemic Sclerosis and neoplastic purpose of a study at Holguin province

La esclerosis sistémica (ES) es una enfermedad de causa desconocida, que se caracteriza por una producción exagerada de moléculas que componen la matriz extracelular. La disminución en la producción de óxido nítrico por las células endoteliales de la microvasculatura parece desempeñar un papel central en la patogenia de la enfermedad. Los resultados alcanzados en un estudio de serie de casos de un universo de 44 pacientes y muestra de 31, con baja incidencia de las causas neoplásicas en la muerte y como reacciones secundarias a tratamiento inmunosupresor con ciclofosfamida, según la conducta terapéutica aplicada, fue el motivo para la presentación de este trabajo, que reflejó la posible relación entre la esclerosis sistémica y las neoplasias. Se concluyó que la relación entre autoinmunidad y cáncer puede ser el resultado de un origen etiológico común (genético, hormonal, metabólico o factores ambientales) o un mecanismo de síndrome paraneoplásico. La enfermedad es terreno de riesgo para la ocurrencia de neoplasias, así como las neoplasias pueden inducir ES(AU)

The systemic sclerosis (SS) is a disease of unknown cause, that the fact that they fix the extra-cell womb characterizes itself for a production exaggerated of molecules. The decrease in the production of nitric oxide for the microvasculature's endothelial cells seems to play a central role in the pathogenesis of the disease. The results attained in 44 patients' study of series of cases of universe and inmunosupresor with cyclophosphamide according to therapeutic applied conduct shows of 31, with low incidence of the causes neoplastic in the death like secondary reactions and to treatment you went from motivation for the presentation of this work, that you reflected the possible relation between the systemic sclerosis and the neoplastic. It was concluded that the relation between auto-immunity and cancer can stem from an etiological common origin (genetic, hormonal, metabolic or environmental factors) or a mechanism of syndrome paraneoplastic. The disease is earthly of risk for neoplastic funny remark, the same way that the neoplastic can induce SS(AU)

Síndrome de CREST asociado a shock séptico por osteomielitis hematógena aguda / CREST syndrome associated with septic shock due to acute osteomyelitis hematologic

El síndrome de CREST (calcinosis, fenómeno de Raynaud, dismotilidad esofágica, esclerodactilia, telangiectasias) forma parte del espectro clínico de la esclerosis sistémica, enfermedad del colágeno, denominada en la clasificación clínica como esclerodermia cutánea limitada. Se presenta un paciente masculino de 53 años, raza blanca y procedencia rural, que desde hace 10 años presenta poliartritis, cambios en zonas distales de la piel que subyace adherida al hueso, falanges de manos y pies, fenómeno de Raynaud, aparición de nódulos subcutáneos de pequeño tamaño en varias localizaciones, que luego se tornan calcificaciones. Llega con una úlcera en el quinto metatarsiano del pie izquierdo, de 14 meses de evolución con varios ingresos para tratamiento de la lesión con resolución aparente y luego recidiva. Desarrolla un cuadro de osteomielitis hematógena aguda con shock séptico. El tratamiento multidisciplinario y oportuno permitió la sobrevida del paciente(AU)

CREST's Syndrome (calcinosis, Raynaud's phenomenon, dysmotilitic esofhagical, sclerodactilitys, telanghiectasis), the form departs from the clinical spectrum of the Systemic Sclerosis, disease of collagen, named in the clinical classification like cutaneous limited scleroderma. Patient, masculine of 53 years, white race, peasant procedence. 10 years ago with changes at zones level distally of the skin that underlies once the bone was adhered, phalanges of hands and feet, Raynaud's phenomenon so big a child's appearing of subcutaneous nodules at several locations, that next calcifications appear. Ulcer in metatarsal foot left-hand fifth, of 14 months of evolution with multiple entrances for treatment of the lesion with apparent resolution and next relapse. Develop acute osteomyelitis hematologic with septic shock. The multi-disciplinary and opportune treatment enabled the patient's over-life(AU)

Esophageal manometry in systemic sclerosis: findings and association with clinical manifestations

SUMMARY INTRODUCTION Systemic sclerosis (SSC) is an autoimmune disorder that affects several organs of unknown etiology, characterized by vascular damage and fibrosis of the skin and organs. Among the organs involved are the esophagus and the lung. OBJECTIVES To relate the profile of changes in esophageal electromanometry (EM), the profile of skin involvement, interstitial pneumopathy (ILD), and esophageal symptoms in SSC patients. METHODS This is an observational, cross-sectional study carried out at the SSC outpatient clinic of the Hospital de Clínicas of the Federal University of Uberlândia. After approval by the Ethics Committee and signed the terms of consent, 50 patients were initially enrolled, from 04/12/2014 to 06/25/2015. They were submitted to the usual investigations according to the clinical picture. The statistical analysis was descriptive in percentage, means, and standard deviation. The Chi-square test was used to evaluate the relationship between EM, high-resolution tomography, and esophageal symptoms. RESULTS 91.9% of the patients had some manometric alterations. 37.8% had involvement of the esophageal body and lower esophageal sphincter. 37.8% had ILD. 24.3% presented the diffuse form of SSC. No association was found between manometric changes and clinical manifestations (cutaneous, pulmonary, and gastrointestinal symptoms). CONCLUSION The present study confirms that esophageal motility alterations detected by EM are frequent in SSC patients, but may not be related to cutaneous extension involvement, the presence of ILD, or the gastrointestinal complaints of patients.

RESUMO INTRODUÇÃO A esclerose sistêmica (ES) é uma doença autoimune que afeta vários órgãos de etiologia desconhecida, caracterizada por dano vascular e fibrose da pele e órgãos. Entre os órgãos envolvidos estão o esôfago e o pulmão. OBJETIVOS Relacionar o perfil das alterações na eletromanometria (ME), o perfil de acometimento da pele, a pneumopatia intersticial (PI) e os sintomas esofágicos em pacientes com ES. MÉTODO Trata-se de um estudo observacional, transversal, realizado no ambulatório de SSC do Hospital das Clínicas da Universidade Federal de Uberlândia. Após aprovação pelo Comitê de Ética e assinatura dos termos de consentimento, 50 pacientes foram inicialmente convidados, de 04/12/2014 a 25/06/2015. Eles foram submetidos às investigações usuais de acordo com o quadro clínico. A análise estatística foi descritiva em porcentagem, média e desvio padrão. O teste Qui-quadrado foi utilizado para avaliar a relação entre ME, tomografia de alta resolução e sintomas esofágicos. RESULTADOS 91,9% dos pacientes apresentaram alterações manométricas. 37,8% tinham envolvimento do corpo esofágico e do esfíncter esofágico inferior. 37,8% tinham IP. 24,3% apresentaram a forma difusa da ES. Não há associação entre alterações manométricas e manifestações clínicas (sintomas cutâneos, pulmonares e gastrointestinais). CONCLUSÃO O presente estudo confirma que as alterações da motilidade esofágica detectadas pela EM são frequentes em pacientes com SSC, mas podem não estar relacionadas ao envolvimento cutâneo, à de DPI ou às queixas gastrointestinais dos pacientes.

Síndrome de solapamiento: polimiositis-esclerodermia. Presentación de un caso / Polymyosis-Sclerosis Overlap Syndrome. Case report and review of literature

Introducción: en el síndrome de solapamiento o superposición existen simultáneamente manifestaciones clínicas o serológicas de dos o más enfermedades autoinmunes sistémicas. Afectan al 5 por ciento de la población con predominio en mujeres. Presentación del Caso: hombre de 48 años, sin antecedentes patológicos personales. Acude con dolor en ambas manos, inflamación en las articulaciones metacarpo e interfalángicas proximales, bilateral y simétrica, acompañada de rigidez matinal que duraba casi todo el día desde hace 4 meses. Se le diagnostica artritis reumatoidea y se inicia tratamiento que abandonó. Luego aparece un cuadro que es interpretado como un solapamiento por lo que es ingresado con un cuadro clínico florido. En los exámenes de laboratorio: las transaminasas, la creatín fosfoquinasa, el lactato deshidrogenasa, se encontraban elevadas. Por la clínica y los complementarios se diagnosticó Síndrome de Solapamiento de polimiosistis-esclerodermia. Se comenzó tratamiento con inmunosupesores. El paciente evolucionó desfavorablemente y falleció. Discusión: el diagnóstico de este caso se realizó por las manifestaciones clínicas como fascie esclerodérmica, signo de sal y pimienta, fibrosis de predominio distal en ambas manos, telangiectasias peribucales. Los diagnósticos diferenciales son las enfermedades autoinmunes sistémicas cuando se presentan como cuadros únicos. El tratamiento de primera línea y el pronóstico dependen de las enfermedades que se solapen(AU)

Introduction: In the syndrome of overlap or overlap there are simultaneously clinical or serological manifestations of two or more systemic autoimmune diseases. They affect 5 percent of the population with predominance in women. Case Presentation: A 48-year-old man with no personal pathological history. He came with pain in both hands, inflammation in the metacarpal and interphalangeal proximal joints, bilateral and symmetrical, accompanied by morning stiffness that lasted almost all day for 4 months. He was diagnosed with rheumatoid arthritis and started treatment that he abandoned. Then a picture appears that is interpreted as an overlap so it is entered with a florid clinical picture. In laboratory tests: transaminases, creatine phosphokinase, lactate dehydrogenase, were elevated. Clinical and complementary diagnosis of Polymyosis-Sclerosis Overlap Syndrome was diagnosed. Treatment with immunosupers was started. The patient evolved unfavorably and died. Discussion: The diagnosis of this case was made by clinical manifestations such as sclerodermic fascia, salt and pepper sign, predominantly distal fibrosis in both hands, perioral telangiectasias. Differential diagnoses are systemic autoimmune diseases when presented as single frames. First-line treatment and prognosis depend on overlapping diseases(AU)

Ventilation distribution as a contributor to the functional exercise capacity in patients with systemic sclerosis-associated interstitial lung disease without pulmonary hypertension

Phenotypic differences have been described between patients with systemic sclerosis (SSc)-associated interstitial lung disease (ILD) and SSc-associated pulmonary hypertension, including performance differences in the 6-min walk test (6MWT). Moreover, the correlations between the 6MWT and traditional pulmonary function tests (PFTs) are weak, indicating the need to search for new parameters that explain exercise performance. Thus, our objective was to evaluate the impact of ventilation distribution heterogeneity assessed by the nitrogen single-breath washout (N2SBW) test and peripheral muscle dysfunction on the exercise capacity in patients with SSc-ILD and limited involvement of the pulmonary parenchyma. In this cross-sectional study, 20 women with SSc-ILD and 20 matched controls underwent PFTs (including spirometry, diffusing capacity for carbon monoxide (DLco), and the N2SBW test) and performed the 6MWT and knee isometric dynamometry. The 6-min walking distance (6MWD, % predicted) was strongly correlated with the phase III slope of the single-breath nitrogen washout (phase III slopeN2SBW) (r=−0.753, P<0.0001) and reasonably correlated with the forced vital capacity (FVC) (r=0.466, P=0.008) and DLco (r=0.398, P=0.011). The peripheral oxygen saturation (SpO2) during exercise was not significantly correlated with any of the pulmonary or muscle function parameters. The phase III slopeN2SBW was the only predictive variable for the 6MWD, whereas quadriceps strength and FVC/DLco were predictive variables for SpO2. Ventilation distribution heterogeneity is one factor that contributes to a lower 6MWD in SSc-ILD patients. In addition, muscle dysfunction and abnormal lung diffusion at least partly explain the decreased SpO2 of these patients.

The Brazillian version of the hand mobility in scleroderma (HAMIS) test: translation and validation

Abstract Background: The Hand Mobility in Scleroderma (HAMIS) test was created to measure the degree of dysfunction of hand movements imposed by systemic sclerosis (SSc). The modified version (mHAMIS), with 4 of the 9 original items, was developed later. The goal of the present study was to translate and validate HAMIS and mHAMIS into Brazilian Portuguese and culture. Methods: After direct and reverse translation and comprehension test in 10 SSc patients, HAMIS-Br was applied to another 32 patients with SSc. To evaluate internal consistency, intraobserver and interobserver agreement, and intraobserver and interobserver reliability, we used respectively the Cronbach's α coefficient, kappa concordance and intraclass correlation (ICC). The correlation between HAMIS-Br and mHAMIS-Br was evaluated and a factorial analysis was performed. Results: HAMIS-Br showed excellent internal consistency (Cronbach's α = 0.997), good intraobserver agreement (kappa between 0.78 [95%CI =0.57-0.99] and 1) and intraobserver and interobserver reliability (ICC = 0.993, 95% CI = 0.973-0.993 and ICC = 0.994, 95% CI = 0.987-0.997, respectively). The mHAMIS-Br presented similar results and excellent correlation with HAMIS-Br (r = 0.923). The factorial analysis extracted three groups of questions that explain 84.4% of the total variance, and that can be understood through the influence of certain movements in the interpretation of others: [1] questions whose interpretation is influenced by the extension of the fingers, [2] questions whose interpretation is influenced by flexion of the fingers, [3] volar flexion of the fingers, with similar correlation with both other factors. Conclusions: HAMIS-Br and mHAMIS-Br showed good agreement, intraobserver and interobserver reliability, and internal validity. It is necessary to be attentive to the influence of certain limitations of movements in the interpretation of others.

Alterações auditivas na esclerose sistêmica / Hearing alterations in systemic sclerosis

RESUMO Objetivo Descrever as queixas e alterações auditivas em indivíduos com esclerose sistêmica (ES), bem como verificar a evolução do quadro audiológico. Método Trata-se de estudo seccional, com uma fase prospectiva, realizado no período de 2012 e 2015, com pacientes com diagnóstico médico de ES. Foram coletados dados sociodemográficos, ano de início da doença, ano de diagnóstico e subtipo da enfermidade. Posteriormente, foram realizadas a anamnese audiológica, para identificação de queixas e sintomas e para a investigação de realização de audiometria pregressa ao estudo, e, em seguida, a avaliação audiológica básica. Resultados Participaram do estudo 50 indivíduos. Tontura e zumbido foram os sintomas mais frequentes. A perda auditiva foi identificada em 23 (46%) indivíduos, sendo a maioria do tipo sensorioneural, de grau e configurações variáveis. A análise dos limares auditivos obtidos na avaliação audiológica realizada em 2012 e, posteriormente, em 2015 indicou desencadeamento ou progressão da perda auditiva, com piora de 10dB na maioria das frequências avaliadas, sendo mais expressiva nas frequências agudas. Conclusão Elevada frequência de queixas e alterações auditivas em indivíduos com ES e desencadeamento e/ou progressão da perda auditiva naqueles que realizaram avaliação audiológica sequencial.

ABSTRACT Purpose Describe hearing complaints and alterations in individuals with systemic sclerosis (SS) and to verify the development of audiological manifestations. Methods This is a cross-sectional study with a prospective phase, conducted in the period from 2012 to 2015, with patients with medical diagnosis of SS. Sociodemographic data, year of disease onset, year of diagnosis and disease subtype were collected. Later, audiological anamnesis was performed to identify complaints and symptoms and to investigate the performance of audiometry before the study and, after that, a basic audiological evaluation was conducted. Results Fifty individuals participated in the study. Dizziness and tinnitus were the most frequent symptoms. Hearing loss was identified in 23 (46%) individuals; most of them were of sensorineural type, of variable degrees and configurations. The analysis of hearing thresholds obtained in the audiological evaluation performed in 2012 and, later, in 2015, indicated onset or progression of hearing loss, with aggravation of 10dB in most frequencies evaluated, being more expressive in acute frequencies. Conclusion High rate of hearing complaints and alterations in individuals with SS and onset and/or progression of hearing loss in those who underwent serial audiological evaluation were observed.

Estenosis de las arterias cubital y radial en pacientes con esclerosis sistémica, comparado con un grupo control / Ulnar and radial stenosis in systemic sclerosis

Objetivos: describir la frecuencia de estenosis arterial (cubital y radial) en pa-cientes con esclerosis sistémica (ES); analizar la relación entre estenosis macro-vascular y úlceras digitales. Método: se incluyeron 57 pacientes con ES, según la clasificación del Colegio Americano de Reumatología de 1980 y 21 pacientes sin ES. Se realizó ecografía doppler arterial de miembros superiores. Resultados: la estenosis en al menos una arteria cubital se objetivó en 31% de pacientes con ES (18/57) (p=0.003). Se objetivó estenosis radial en 9 de 57 pacientes con ES (15%) y en uno de los 21 controles (p=0.19). En el modelo multivariado, los predicto-res de úlceras digitales fueron inicio de Raynaud antes de los 40 años (OR 5.3 IC95% 1.54-18.22, p=0.008) y patrón tardío en la capilaroscopia (OR 4.4 IC95% 1.29-15.63, p=0.018). Conclusiones: un tercio de los pacientes ES presentó este-nosis cubital. El compromiso de los grandes vasos no se asoció a úlceras digitales.

Objectives: to describe the frequency of ulnar and radial stenosis in SSc patients. Analyze the correlation between arterial stenosis and digital ulcers. Methods: we included 57 SSc consecutive patients who fulfilled ACR 1980 classification criteria, and 21 healthy controls. An arterial ecodoppler was performed to all participants. Results: the presence of stenosis in at least one ulnar artery was observed in 18 of 57 patients with SSc (31%) and in none of the 21 controls (p=0.003). Stenosis was present in at least one radial artery in 9 of 57 SSc patients (15%) (p=0.19). In multivariate model, the best predictors of digital ulcers were age at onset of Ray-naud phenomenon before 40 years (OR 5.3 95%CI 1.54-18.22, p=0.008) and late SD pattern (OR 4.4 95%CI 1.29-15.63, p=0.018). Conclusion: in the present series, ulnar stenosis was observed frequently in SSc patients. Stenosis of large vessels was not associated with digital ulcers.

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.

Correlation between nailfold capillaroscopic findings and presence of interstitial lung disease in systemic sclerosis patients / 北京大学学报(医学版)

OBJECTIVE@#To evaluate the correlation between nailfold capillaroscopic (NC) findings and the presence of interstitial lung disease (ILD) in systemic sclerosis (SSc) patients.@*METHODS@#We retrospectively involved 71 SSc patients, 45 patients with ILD. NC was performed in all the patients according to the standard method. The NC findings were semi-quantitatively scored, including enlarged and giant capillaries, hemorrhages, loss of capillaries, avascular areas, ramified/bushy capillaries and disorganization of the vascular array. The demographic and clinical data collected were gender, age, presence/absence of Raynaud phenomenon (RP), duration of RP, serological acute phase reactants and antibodies, presence of ILD (also evaluated the CT score for ILD) and pulmonary function parameters.@*RESULTS@#Among the 71 patients, the frequency of the women was 91.5%, the mean age was (52.59±12.77) years, and disease duration was (3.00±6.00) years. NC changes of the scleroderma pattern were observed in 90.1% patients. There were 45 patients with ILD and 26 patients without ILD. The patients with ILD had significantly higher loss of capillaries score [0.50 (1.03) vs. 0.00 (0.43), P=0.003], avascular area score [0.75 (1.24) vs. 0.25 (0.83), P=0.006] and ramified/bushy capillaries score [0.33 (0.88) vs. 0.13 (0.25), P=0.006] compared with those without ILD. Moreover, ramified/bushy capillaries score together with diffused SSc were independent risk factors for the presence of ILD. And the score of giant capillaries were significantly lower in the patients with more severe ILD group [0.25 (0.94) vs. 0.00 (0.28), for the mild and severe ILD groups respectively, P=0.019]. There was no statistically significant difference between the two groups with respect to the scores of enlarged capillaries, hemorrhages, or disorganization of the vascular array.@*CONCLUSION@#Capillary deletion and severe deformity in NC were associated with the presence of ILD in SSc patients. And patients with less giant capillaries had more severe ILD involvement. These indicated that NC maybe a useful tool to evaluate ILD in SSc.

Evaluation of Left Ventricular Diastolic Function by Echocardiography with Tissue Doppler in Systemic Sclerosis / Avaliação da Função Diastólica do Ventrículo Esquerdo com Ecocardiografia Associada a Doppler Tecidual na Esclerose Sistêmica

Abstract Background: Systemic sclerosis (SS) is a connective tissue abnormality characterized by fibrosis of the skin and internal organs. Cardiac involvement with consequent myocardial dysfunction in SS is associated with increased morbidity and mortality. Objective: To investigate the left ventricular (LV) diastolic function in patients with SS and preserved systolic function. Methods: Patients with SS were evaluated with two-dimensional echocardiography with tissue Doppler for analysis of chamber diameters, LV mass index (LVMI), indexed left atrial volume (iLAV), systolic function of both ventricles, and presence and degree of diastolic dysfunction (DD). Results: We evaluated 50 patients, divided according to the presence of DD into Group 1 (n = 25; normal diastolic function, E/A ratio ≥ 0.8, deceleration time [DT] > 150 ms and < 200 ms, and septal e' > 8 cm/s) and Group 2 (n = 25; with DD, subdivided into type I DD [E/A < 0.8, DT > 200 ms], type II [E/A ≥ 0.8, septal e' < 8 cm/s, iLAV > 34 mL/m2], and type III [E/A > 2, DT < 150 ms, septal e' < 8 cm/s]). Type I DD was the most frequent (34%), followed by type II DD (16%). LVMI and iLAV were similar in both groups, but septal and lateral e' were reduced only in Group 2. In Group 2, we observed that patients with moderate DD had longer disease duration (p = 0.02). Conclusion: The prevalence of type I DD was elevated in SS and associated with aging. Disease duration emerged as an important factor in moderate DD.

Resumo Fundamento: A esclerose sistêmica (ES) é uma anomalia do tecido conjuntivo caracterizada por fibrose da pele e dos órgãos internos. O envolvimento cardíaco com consequente disfunção miocárdica na ES está associado a uma maior morbimortalidade. Objetivo: Investigar a função diastólica do ventrículo esquerdo (VE) em pacientes com ES e função sistólica preservada. Método: Pacientes com ES foram estudados com ecocardiografia bidimensional com Doppler tecidual para análise dos diâmetros cavitários, índice de massa do VE (IMVE), volume indexado do átrio esquerdo (VAEi), função sistólica biventricular e presença, além de grau de disfunção diastólica (DD) pelo Doppler convencional e tecidual. Resultados: Estudamos 50 pacientes, divididos conforme presença de DD em Grupo 1 (n = 25; função diastólica normal, razão E/A ≥ 0,8, tempo de desaceleração [TD] > 150 ms e < 200 ms e e' septal > 8 cm/s) e Grupo 2 (n = 25; com presença de DD, subdividido em DD tipo I [E/A < 0,8, TD > 200 ms], tipo II [E/A ≥ 0,8, e' septal < 8 cm/s, VAEi > 34 mL/m2] e tipo III [E/A > 2, TD < 150 ms, e' septal < 8 cm/s]. A DD tipo I foi a mais frequente (34%), seguida de DD tipo II (16%). O IMVE e o VAEi foram similares entre os grupos, porém a e' septal e lateral estiveram reduzidas apenas no Grupo 2. No Grupo 2, observamos que os pacientes com DD moderada apresentavam maior duração da doença (p = 0,02). Conclusão: A prevalência de DD tipo I foi elevada na ES e esteve associada ao envelhecimento. No caso da DD moderada, a duração da doença despontou como um fator importante.

Treatment of digital ulcers in systemic sclerosis: Case series study of thirteen patients and discussion on outcome / Tratamento de úlceras digitais na esclerose sistêmica: série de casos de 13 doentes e análise crítica dos resultados

Summary Introduction: In systemic sclerosis (SSc), digital ulcers (DU) are debilitating and recurrent. They are markers of prognosis and are associated with disability and mortality. Treatment strategies have been developed to block the proposed mechanisms of this complication. Objective: Clinical description of a population of SSc patients with DU, treatment, complications and outcome. Method: Analysis of 48 SSc patients meeting 2013 ACR-EULAR criteria, followed between 1999-2015; 13 patients had DU. Treatment protocol applied included cycles of 21 days of alprostadil, which can be repeated in the absence of DU healing. After DU healing, bosentan was initiated. Results: DU healing was achieved with intravenous prostanoid in 12 patients; seven patients required repeated treatment for DU healing. Twelve patients were later treated with bosentan; three of them experienced recurrence of DU, while one was anti-B2-GPI positive. Four patients had soft tissue loss and three other suffered digital amputation, these being late diagnosis. Conclusion: Younger patients and early referrals had better outcomes. Endothelin receptor antagonist toxicity should be monitored, particularly in patients previously exposed to hepatotoxic drugs.

Resumo Introdução: As úlceras digitais (UD) são complicações incapacitantes e recorrentes, associadas a menor qualidade de vida e maior mortalidade na esclerose sistêmica (ES). O tratamento baseia-se em antagonizar os mecanismos fisiopatológicos em causa. Objetivo: Descrever uma amostra de doentes com diagnóstico de ES e UD, o tratamento, as complicações e os resultados clínicos. Método: Série de 48 casos diagnosticados com ES, critérios de classificação ACR-EULAR 2013, seguidos entre 1999 e 2015, dos quais 13 apresentavam UD. O protocolo aplicado incluía ciclos de 21 dias de alprostadil podendo ser repetidos no caso de não existir cicatrização. Nos casos em que houve cicatrização foi iniciado bosentano. Resultados: No tratamento das UD, 12 doentes realizaram prostaciclina endovenosa, com necessidade de tratamentos repetidos em sete doentes. Doze doentes foram posteriormente tratados com bosentano, com recorrência de UD em três doentes, um deles com presença de anti-B2-GPI. Quatro doentes ficaram com cicatrizes e em três houve amputação digital, sendo casos de diagnóstico tardio. Conclusão: Os doentes mais jovens tiveram melhores resultados, possivelmente em razão de melhorias globais nos cuidados de saúde prestados e de referenciação precoce. A toxicidade dos antagonistas dos receptores da endotelina deve ser monitorizada, sobretudo em doentes com exposição prévia a drogas hepatotóxicas.

Alterações retrococleares na esclerose sistêmica: relato de casos / Retrocochlear impairments in systemic sclerosis: a case report study

RESUMO Objetivo Relatar três casos de pacientes portadores de Esclerose Sistêmica e que apresentaram alterações retrococleares. Método Trata-se de um estudo de relato de casos de três indivíduos com esclerose sistêmica e alteração retrococlear, acompanhados em um serviço de reumatologia. Todos os pacientes realizaram o Potencial Evocado Auditivo de Tronco Encefálico e, quando necessário, nova audiometria. Resultados Todos os indivíduos apresentaram perda auditiva do tipo sensorioneural. Não foi identificado na avaliação audiológica básica qualquer resultado que sugerisse alteração retrococlear, porém o PEATE apresentou-se alterado. Conclusão Pode-se concluir que o estudo revela alterações retrococleares nesta população, ocorrendo tanto nas latências absolutas quanto no intervalo interpico. E, neste contexto, reumatologistas e fonoaudiólogos, ao acompanharem pacientes com esclerose sistêmica, devem estar atentos para a possibilidade da ocorrência dessa alteração nessa população. Revela também, a necessidade de estudos epidemiológicos sobre o tema.

ABSTRACT Purpose To report three cases of patients with Systemic Sclerosis (SSc) and retrocochlear impairments. Methods This is a case report of three individuals with SSc and retrocochlear impairments assisted at a rheumatology outpatient clinic. All individuals underwent Brainstem Auditory Evoked Potential (BAEP) and, when necessary, audiometry. Results All three individuals presented sensorineural hearing loss. Although no retrocochlear impairment was identified in the basic audiologic evaluation, the BAEP results were altered. Conclusion Retrocochlear impairments were present in the individuals under study, both in the absolute latencies and interpeak interval, thereby demanding the attention of rheumatologists and speech-language pathologists to such changes during the monitoring of SSc patients. The results also show a need for epidemiological studies on the theme.

Overlap between systemic sclerosis and rheumatoid arthritis: a distinct clinical entity? / Sobreposição de esclerose sistêmica e artrite reumatoide: uma entidade clínica distinta?

ABSTRACT Introduction: Systemic sclerosis (SSc) is an autoimmune disease of the connective tissue characterized by the triad of vascular injury, autoimmunity (cellular and humoral) and tissue fibrosis. It is estimated that musculoskeletal pain is a common complaint of patients with SSc, ranging from 40 to 80%, and mainly in patients with early diffuse disease. Arthritis, clinically observed, may be a feature seen in the presentation of SSc, often leading to early diagnostic errors with rheumatoid arthritis (RA). In the course of the disease, arthritis is observed in 24–97% of patients with SSc. Objectives: To correlate the occurrence or nonoccurrence of arthritis in patients with SSc of the Midwest region of Brazil with possible distinct clinical and laboratory manifestations observed in three groups of patients. To report the frequency of true association between systemic sclerosis and rheumatoid arthritis in patients with clinically and radiologically observed synovitis. Methods: Sixty-one SSc patients were subsequently assessed every 3 months within 1 year, in order to clinically observe the occurrence of synovitis and its patterns of progression. Patients were divided into 3 groups: 41 patients with SSc without arthritis, 16 SSc patients with arthritis and 4 patients with overlap of SSc and RA. All patients underwent a radiological examination of the hands at the end of the study. Results: Among all patients evaluated, we found a female predominance (98.7%), mean age of 50.94 years, white color (49.2%), limited form of the disease (47.6%), time of diagnosis between 5 and 10 years (47.6%) and duration of the disease of 8.30 years. Among all patients, 14 (22.9%) had positive rheumatoid factor (RF), while among those with positive RF, only 10 patients had arthritis during one-year follow-up. The antibody anticitrulline (anti-CCP) test was performed in 24 patients, being positive in 4 of them (16.7%), with positivity being observed only in patients with SSc/RA overlap. Comparing the clinical manifestations among the groups of patients, there was a higher incidence of gastritis and cardiac valvulopathy in patients with SSc and arthritis, but not in the others. In the group of patients with SSc/RA overlap and in patients with SSc and arthritis a significant reduction in quality of life was observed, measured by HAQ index, especially in patients with arthritis present during clinical evaluation. We found radiographic changes in 42.6% of patients with SSc. However, in patients with synovitis, radiological changes consistent with rheumatoid arthritis were found in 50% of patients. Conclusions: While the frequency of clinical arthritis observed in patients with systemic sclerosis was 32.8%, the true overlap between of SSc and RA was 6.6% in this study. We also observed the frequency of positive anti-CCP in 20% of patients with arthritis versus no patients with SSc without arthritis.

RESUMO Introdução: A esclerose sistêmica (ES) é uma enfermidade do tecido conjuntivo de caráter autoimune caracterizada pela tríade de injúria vascular, autoimunidade (celular e humoral) e fibrose tecidual. Estima-se que a dor musculoesquelética seja uma queixa frequente dos pacientes com ES, que oscila entre 40% e 80%, e principalmente em pacientes com doença difusa precoce. A artrite, clinicamente observada, pode ser uma característica observada na apresentação da ES, frequentemente leva a erros diagnósticos iniciais com artrite reumatoide (AR). No curso da enfermidade, a artrite é observada em 24% a 97% dos pacientes com ES. Objetivos: Correlacionar a ocorrência ou não de artrite em pacientes com ES da região Centro-Oeste do Brasil com possíveis manifestações clínicas e laboratoriais distintas observadas em três grupos de pacientes. Relatar a frequência de verdadeira associação entre esclerose sistêmica e artrite reumatoide em pacientes com sinovite clínica e radiologicamente observada. Métodos: Foram avaliados 61 pacientes portadores de ES subsequentemente a cada três meses durante um ano, para fins de se constatar clinicamente a ocorrência de sinovite e padrões de evolução. Os pacientes foram divididos em três grupos: 41 com ES sem artrite, 16 com ES com artrite e quatro com sobreposição entre ES e AR. Todos os pacientes foram submetidos a exame radiológicos das mãos no fim do estudo. Resultados: Dentre todos os pacientes avaliados, encontrou-se predomínio feminino (98,7%), idade média de 50,94 anos, cor branca (49,2%), forma limitada da doença (47,6%), tempo de diagnóstico entre cinco e 10 anos (47,6%) e tempo de evolução da doença de 8,30 anos. Entre todos os pacientes, 14 (22,9%) apresentavam fator reumatoide (FR) positivo, embora entre aqueles com FR positivo apenas 10 apresentaram artrite durante o seguimento de um ano. O anticorpo anticitrulina (anti- CCP) foi feito em 24 pacientes, com positividade em quatro deles (16,7%), observada somente nos pacientes com sobreposição ES/AR. Na comparação das manifestações clínicas entre os grupos de pacientes, observou-se a maior ocorrência de gastrite e valvulopatia cardíaca em pacientes com ES e artrite, mas não nos demais grupos. No grupo de pacientes com overlap ES/AR e nos pacientes com ES e artrite observou-se redução importante de qualidade de vida, medida pelo índice HAQ, sobretudo nos pacientes com artrite presente no momento da avaliação clínica. Encontramos alterações radiográficas em 42,6% dos pacientes com ES. Contudo, nos pacientes com sinovite, encontraram-se alterações radiológicas compatíveis com artrite reumatoide em 50%. Conclusões: Enquanto a frequência de artrite clínica observada em pacientes com esclerose sistêmica foi de 32,8%, a verdadeira sobreposição entre ES e AR foi de 6,6% neste estudo. Observou-se ainda a frequência de anti-CCP positivo em 20% dos pacientes com artrite contra nenhum paciente com ES sem artrite.

Familial autoimmunity and polyautoimmunity in 60 Brazilian Midwest patients with systemic sclerosis / Autoimunidade familial e poliautoimunidade em 60 pacientes portadores de esclerose sistêmica da região Centro-Oeste do Brasil

ABSTRACT Introduction: Systemic sclerosis (SSc) is a connective tissue disease of unknown etiology, characterized by a triad of vascular injury, autoimmunity and tissue fibrosis. It is known that a positive family history is the greatest risk factor already identified for the development of SSc in a given individual. Preliminary observation of a high prevalence of polyautoimmunity and of familial autoimmunity in SSc patients support the idea that different autoimmune phenotypes may share common susceptibility variants. Objectives: To describe the frequency of familial autoimmunity and polyautoimmunity in 60 SSc patients in the Midwest region of Brazil, as well as to report the main autoimmune diseases observed in this association of comorbidities. Methods: A cross-sectional study with recruitment of 60 consecutive patients selected at the Rheumatology Department, University Hospital, Medicine School, Federal University of Mato Grosso do Sul (FMUFMS), as well as interviews of their relatives during the period from February 2013 to March 2014. Results: A frequency of 43.3% of polyautoimmunity and of 51.7% of familial autoimmunity in SSc patients was found. Patients with the presence of polyautoimmunity and familial autoimmunity presented primarily the diffuse form of SSc, but this indicator did not reach statistical significance. The autoimmune diseases most frequently observed in polyautoimmunity patients were: Hashimoto's thyroiditis (53.8%), Sjögren's syndrome (38.5%), and inflammatory myopathy (11.5%). The main autoimmune diseases observed in SSc patients' relatives were: Hashimoto's thyroiditis (32.3%), rheumatoid arthritis (22.6%), and SLE (22.6%). The presence of more than one autoimmune disease in SSc patients did not correlate with disease severity or activity. Conclusions: From the high prevalence of coexisting autoimmune diseases found in SSc patients, we stress the importance of the concept of shared autoimmunity, in order to promote a continued vigilance and promptly diagnose other possible autoimmune disease in patients, or in their kin.

RESUMO Introdução: A esclerose sistêmica (ES) é uma enfermidade do tecido conjuntivo de etiologia desconhecida, caracterizada pela tríade de injúria vascular, autoimunidade e fibrose tecidual. Sabe-se que uma história familiar positiva representa o maior fator de risco já identificado para o desenvolvimento da ES em um determinado indivíduo. Observação prévia de alta prevalência de poliautoimunidade e de autoimunidade familiar em pacientes com ES reforça a ideia de que fenótipos autoimunes distintos podem dividir variantes comuns de susceptibilidade. Objetivos: Descrever a frequência de autoimunidade familiar e de poliautoimunidade em 60 pacientes com ES da região Centro-Oeste do Brasil, bem como relatar as principais doenças autoimunes observadas nessa associação de comorbidades. Métodos: Estudo transversal com recrutamento de 60 pacientes consecutivos, selecionados no Serviço de Reumatologia do Hospital Universitário da Faculdade de Medicina da Universidade Federal de Mato Grosso do Sul (FMUFMS), bem como entrevista de seus parentes, de fevereiro de 2013 a março de 2014. Resultados: Foi encontrada uma frequência de 43,3% de poliautoimunidade e de 51,7% de autoimunidade familiar nos pacientes com ES. Os pacientes com presença de poliautoimunidade e de autoimunidade familiar eram principalmente da forma difusa de ES, porém esse índice não atingiu significância estatística. As doenças autoimunes mais comumente observadas nos pacientes com poliautoimunidade foram: tireoidite de Hashimoto (53,8%), síndrome de Sjögren (38,5%) e miopatia inflamatória (11,5%). As principais doenças autoimunes observadas nos parentes dos pacientes com ES foram: tireoidite de Hashimoto (32,3%), artrite reumatoide (22,6%) e LES (22,6%). A presença de mais de uma enfermidade autoimune em pacientes com ES não se correlacionou com maior gravidade ou atividade da doença. Conclusões: A partir da alta prevalência encontrada de doenças autoimunes coexistentes em pacientes com ES, salientamos a importância do conceito de autoimunidade compartilhada, de forma a promover uma vigilância constante e diagnosticar prontamente uma possível outra doença autoimune nos pacientes ou em seus parentes.

Calcinosis pseudotumoral en esclerodermia / Pseudotumoral calcinosis in systemic sclerosis

La calcinosis es una manifestación frecuente en la esclerodermia sistémica. Sin embargo, la calcinosis pseudotumoral es de presentación excepcional. Las radiografías o tomografía axial computada son de utilidad para el diagnóstico y el seguimiento. Aunque no existe un tratamiento estandarizado efectivo, la remoción quirúrgica de las lesiones sintomáticas y algunos fármacos son útiles en algunos enfermos. Se presenta el caso de una paciente de 23 años con esclerodermia sistémica variedad limitada que desarrolló múltiples lesiones pseudotumorales.

Soft tissue calcinosis is a common cutaneous manifestation in systemic sclerosis. However, pseudotumoral calcinosis is rare. Radiographs or computed tomography are useful tools to diagnose and assess this condition. Although no treatment was uniformly effective, surgical excision of symptomatic lesions and medical treatment provided benefit for some patients. We report here a case of a 23 year old woman, suffering from limited cutaneous systemic sclerosis, who developed many pseudo tumoural calcinosis lesions.

An update of neurological manifestations of vasculitides and connective tissue diseases: a literature review / Atualização das manifestações neurológicas das vasculitides e das doenças do tecido conjuntivo: revisão de literatura

Vasculitides comprise a heterogeneous group of autoimmune disorders, occurring as primary or secondary to a broad variety of systemic infectious, malignant or connective tissue diseases. The latter occur more often but their pathogenic mechanisms have not been fully established. Frequent and varied central and peripheral nervous system complications occur in vasculitides and connective tissue diseases. In many cases, the neurological disorders have an atypical clinical course or even an early onset, and the healthcare professionals should be aware of them. The purpose of this brief review was to give an update of the main neurological disorders of common vasculitis and connective tissue diseases, aiming at accurate diagnosis and management, with an emphasis on pathophysiologic mechanisms.

As vasculites são um grupo heterogêneo de doenças autoimunes primárias ou secundárias a uma grande variedade de doenças infecciosas sistêmicas, malignas ou do tecido conjuntivo. Estas últimas são as que ocorrem com mais frequência, porém os mecanismos patogênicos ainda não foram plenamente determinados. Diversas e frequentes complicações do sistema nervoso central e periférico ocorrem nas vasculites e doenças do tecido conjuntivo. Em muitos casos, os distúrbios neurológicos têm evolução clínica atípica ou mesmo início precoce, ao que todos os profissionais de saúde devem estar cientes. O objetivo desta breve revisão foi atualizar os principais distúrbios neurológicos da vasculite comum e das doenças do tecido conjuntivo, visando ao diagnóstico e ao tratamento corretos, com ênfase nos mecanismos fisiopatológicos.