Uso de micofenolato mofetilo fuera de registro en enfermedades inmunomediadas / Off-label use of mycophenolate mofetil in immune-mediated diseases

BACKGROUND: Mycophenolate mofetil (MMF) is a largely used immunosuppressive agent in the prevention of transplant rejection and lupus nephritis. Its use has been extended to other immune-mediated diseases (ID). AIM: To assess the off-label use of MMF, its performance as a glucocorticoid sparing agent, the therapeutic response, and its adverse effects. MATERIAL AND METHODS: A retrospective study was performed. One hundred-seven patients aged 58 ± 16 years (83% females) who received MMF for ID in off label uses between 2016 and 2018 were included. The study variables were cause of MMF indication, sex, age, use as a first- or second-line treatment and maintenance dosing. The cumulative doses of glucocorticoids six months before and after MMF indication were compared. RESULTS: MMF was used as a second-line therapy in 66 patients (62%). The mean maintenance dose of MMF was 1,500 ± 540 mg/day. Prednisone cumulative doses were 3,908 ± 2,173 and 1,672 ± 1,083 milligrams six months before and six months after starting MMF, respectively (p < 0.01). Adverse effects were identified in 21 (20%) cases, none of them serious. CONCLUSIONS: Mycophenolate has a favorable response profile as a second line immunosuppressive agent. It is effective as a glucocorticoid sparing drug. The safety profile is also favorable as adverse effects were scanty and mild.

Prednisone and long-term damage in systemic lupus erythematosus: Which is the threshold dose? A pilot study.

OBJECTIVES: We aimed to study the daily dose of glucocorticoids in the first month associated with damage after 5 years of systemic lupus erythematosus (SLE) diagnosis; and to assess the daily dose of prednisone during the first year, over which damage after 5 years could be predicted. METHODOLOGY: A retrospective cohort study of SLE patients from the diagnosis and up to 5 years of follow-up was performed. Cumulative prednisone doses in the first month (T1m), at 1 year (T12m), and at 5 years (T5y) were calculated. Damage was estimated using the SLICC index and activity by the SLEDAI-2K. Damage at T5y was categorized as "present" or "absent". To determine the cutoff point of prednisone dose for the first month and for the first year of treatment, related to damage at 5 years, a ROC curve was done. A logistic regression was performed to control damage at 5 years by the activity levels, anti-DNA titers, and the corticosteroid burden between 1 and 5 years. RESULTS: Forty-eight patients were included with a mean age of 42.4 (12.6) years; 46 (95.8%) were female. The cumulative dose in T1m associated with greater sensitivity and specificity in the ROC curve (1.54; AUC 0.793; 95% CI: 0.66-0.92) to predict glucocorticoid-related damage at 5 years of follow-up was 980mg, which leads to a daily dose for the first month of 32.6 mg/day. The daily dose during the first year associated with damage at 5 years, allowed to calculate an optimal cutoff point of 7.38 mg/day, with a sensitivity of 92.9% (AUC 0.695, 95% CI: 0.52-0.86) to predict damage at 5 years. In the logistic regression, this estimate was maintained, controlling for SLEDAI-2K and for positivity or not of anti-DNA antibodies and for the cumulative dose of prednisone between T5y and T12m. CONCLUSION: This pilot study allows to estimate a threshold dose of 32.6 mg/day during the first month and 7.38 mg/day during the first year, over which the risk of permanent damage is high at 5 years, regardless of the activity levels and the glucocorticoid doses considered between the first and the fifth year of follow-up.

[Off-label use of mycophenolate mofetil in immune-mediated diseases]. / Uso de micofenolato mofetilo fuera de registro en enfermedades inmunomediadas.

BACKGROUND: Mycophenolate mofetil (MMF) is a largely used immunosuppressive agent in the prevention of transplant rejection and lupus nephritis. Its use has been extended to other immune-mediated diseases (ID). AIM: To assess the off-label use of MMF, its performance as a glucocorticoid sparing agent, the therapeutic response, and its adverse effects. MATERIAL AND METHODS: A retrospective study was performed. One hundred-seven patients aged 58 ± 16 years (83% females) who received MMF for ID in off label uses between 2016 and 2018 were included. The study variables were cause of MMF indication, sex, age, use as a first- or second-line treatment and maintenance dosing. The cumulative doses of glucocorticoids six months before and after MMF indication were compared. RESULTS: MMF was used as a second-line therapy in 66 patients (62%). The mean maintenance dose of MMF was 1,500 ± 540 mg/day. Prednisone cumulative doses were 3,908 ± 2,173 and 1,672 ± 1,083 milligrams six months before and six months after starting MMF, respectively (p < 0.01). Adverse effects were identified in 21 (20%) cases, none of them serious. CONCLUSIONS: Mycophenolate has a favorable response profile as a second line immunosuppressive agent. It is effective as a glucocorticoid sparing drug. The safety profile is also favorable as adverse effects were scanty and mild.

A longitudinal multiethnic study of biomarkers in systemic lupus erythematosus: Launching the GLADEL 2.0 Study Group.

Introduction: After more than 20 years of sustained work, the Latin American Group for the Study of Lupus (GLADEL) has made a significant number of contributions to the field of lupus, not only in the differential role that race/ethnicity plays in its course and outcome but also in several other studies including the beneficial effects of using antimalarials in lupus patients and the development of consensus guidelines for the treatment of lupus in our region. Methods: A new generation of "Lupus Investigators" in more than 40 centers throughout Latin America has been constituted in order to continue the legacy of the investigators of the original cohort and to launch a novel study of serum and urinary biomarkers in patients with systemic lupus erythematosus. Results: So far, we have recruited 807 patients and 631 controls from 42 Latin-American centers including 339 patients with SLE without renal involvement, 202 patients with SLE with prevalent but inactive renal disease, 176 patients with prevalent and active renal disease and 90 patients with incident lupus nephritis. Conclusions: The different methodological aspects of the GLADEL 2.0 cohort are discussed in this manuscript, including the challenges and difficulties of conducting such an ambitious project.

Inmunosupresores en la Esclerosis Sistémica. Uso y respuesta terapéutica

Resumen: Introducción: La esclerosis sistémica (ES) es una enfermedad compleja y heterogénea. Su abordaje terapéutico y oportunidad de inmunosupresión no está completamente sistematizada y constituye un reto. Objetivo: Describir los usos y respuesta terapéutica a los inmunosupresores (IS) en una cohorte de pacientes con ES. Metodología: Se identificaron los pacientes con Esclerosis sistémica asistidos en dos unidades de enfermedades autoinmunes sistémicas. Se incluyeron los que recibieron IS. Variables estudiadas: sexo, edad y motivo de IS. En los que presentaron compromiso respiratorio se analizaron los resultados de las pruebas de función respiratoria pre y postratamiento. Resultados: 20 pacientes, 17 de sexo femenino. Edad media 64 años. Principal indicación de IS: compromiso respiratorio seguido del cutáneo-articular. Se realizó inducción en 75% de los pacientes respiratorios vs. 12% con otro compromiso. Micofenolato mofetilo fue el inmunosupresor más utilizado. Conclusiones: El uso de IS predomina en el compromiso respiratorio de los pacientes con ES. Se observó un mayor uso de MMF respecto a otros IS así como una respuesta favorable en las pruebas de función respiratoria en los pacientes tratados con IS.

Abstract: Introduction: Systemic sclerosis (SS) is a complex and heterogeneous disease. Its therapeutic approach and immunosuppression opportunity is not completely defined, it remains a challenge. Objective: To describe the usage and therapeutic response to immunosuppressive agents (IS) in a cohort of patients with SS. Methodology: Patients with SS assisted in 2 units of systemic autoimmune diseases were selected. Those who received IS were included. Variables studied were sex, age and IS indication. Those with respiratory manifestations were assessed with functional lung tests previous and after treatment. Results: 20 patients, 17 females. The mean age was 64 years old. The main indication of IS was respiratory manifestations followed by skin and articular symptoms. Induction treatment was indicated in 75% of respiratory patients vs. 12% in those with other manifestations. Mycophenolate mofetil was the most widely immunosuppressive agent employed. Conclusions: The main prescription of IS in SS was for patients with respiratory manifestations. An increased use of MMF among other IS was observed as well as a favourable response in lung function tests in patients treated with IS.

Resumo: Introdução: A esclerose sistêmica (ES) é uma doença complexa e heterogênea. Sua abordagem terapêutica e oportunidade de imunossupressão não estão totalmente sistematizadas e constituem um desafio. Objetivo: Descreva os usos e resposta terapêutica a imunossupressores (SI) em uma coorte de pacientes com SS. Metodologia: Foram identificados pacientes com esclerose sistêmica atendidos em duas unidades de doenças autoimunes sistêmicas. Aqueles que receberam IS foram incluídos. Variáveis estudadas: sexo, idade e motivo do IS. Naqueles com comprometimento respiratório, foram analisados os resultados dos testes de função respiratória pré e pós-tratamento. Resultados: 20 pacientes, 17 mulheres. Idade média 64 anos. Principal indicação para IS: comprometimento respiratório seguido de cutâneo-articular. A indução foi realizada em 75% dos pacientes respiratórios vs. 12% com outro compromisso. O micofenolato de mofetil foi o imunossupressor mais amplamente utilizado. Conclusões: O uso de SI predomina no comprometimento respiratório de pacientes com SS. Um maior uso de MMF foi observado em comparação com outros ISs, bem como uma resposta favorável em testes de função respiratória em pacientes tratados com IS.

Recertificación médica en Uruguay: múltiples miradas de una obligación impostergable / Medical recertification in Uruguay: multiple perspectives of an imperative need / Recertificação médica no Uruguai: múltiplas visões de uma obrigação urgente

Resumen: La recertificación es el resultado de un acto por el que una entidad legalmente acreditada, asegura que el profesional médico (previamente certificado) mantiene actualizados sus conocimientos y destrezas, y que ha desarrollado su actividad dentro del marco ético y científico adecuado al progreso del "saber" y del "hacer" propio de su especialidad. A pesar de un largo camino recorrido, en Uruguay no se ha podido establecer un proceso de recertificación universal. Múltiples actores (usuarios del sistema, médicos, Facultad de Medicina, programas de Desarrollo Profesional Médico Continuo) consideran que es una necesidad, sin embargo es necesario vencer algunas barreras para que se establezca un programa de recertificación. Se recorren algunos de estos aspectos en este documento, desarrollados en el contexto de un grupo de trabajo para el Congreso por los 100 años del Sindicato Médico del Uruguay.

Abstract: Recertification is the result of an act by which a legally accredited entity ensures that medical professionals (previously certified) keep their knowledge and skills up to date, and that they have practiced their profession within the ethical and scientific framework that regulates the process that evolves from "knowing" to "knowing how" in their areas of specialization. Despite a long journey in Uruguay, it has not been possible to establish a universal recertification process. Multiple actors (system users, doctors, the School of Medicine, Continuing Medical Professional Development programs) regard it as a need, although some barriers must be overcome in order to define a recertification program. This document covers a few of these aspects and is the result of a working group created for the Congress held in commemoration of the 100 years of the Uruguayan Medical Association.

Resumo: A recertificação é o resultado de um ato pelo qual uma entidade legalmente credenciada garante que o profissional médico (previamente certificado) mantém os seus conhecimentos e competências atualizados, e que desenvolveu a sua atividade dentro do quadro ético e científico adequado ao progresso do "conhecimento" e o "fazer" da sua especialidade. Apesar do longo caminho percorrido no Uruguai, ainda não foi possível estabelecer um processo de recertificação universal. Múltiplos atores (usuários do sistema, médicos, Faculdade de Medicina, programas de Desenvolvimento Continuado do Profissional Médico) consideram isso uma necessidade, porém é necessário superar algumas barreiras para que um programa de recertificação seja estabelecido. Alguns desses aspectos são abordados neste documento, desenvolvido no contexto de um grupo de trabalho para o Congresso pelos 100 anos do Sindicato Médico del Uruguay.

Nefritis lúpica: experiencia con dosis reducidas de glucocorticoides en una unidad de enfermedades autoinmunes sistémicas / Lupus nephritis: low-dose glucocorticoids in a systemic autoimmune diseases unit / Nefritis lúpica: experiência con doses reduzidas de glicocorticoides em uma unidade de doenças autoinmunes sistémicas

Resumen: Introducción: las recomendaciones actuales del tratamiento de la nefritis lúpica (NL) apuntan a dosis de glucocorticoides más bajas para lograr el control de la enfermedad y evitar el daño acumulado. Objetivo: conocer y comparar la respuesta al tratamiento de pacientes con NL proliferativa en su etapa de inducción con dos pautas de tratamiento con prednisona (PDN): dosis iniciales reducidas 30 mg/d. Método: se compararon variables clínicas, analíticas y terapéuticas de pacientes con NL proliferativa categorizados en dos grupos según la dosis inicial de prednisona (PDNi) estándar o reducida. Resultados: se estudiaron 21 pacientes con NL proliferativa (n=12 PDNi reducida vs. n=9 PDNi estándar). No hubo diferencias significativas en las variables clínicas y analíticas. Se observó una diferencia estadísticamente significativa en el número de pulsos de metilprednisolona (5 ± 2,95 PDNi 30 mg/d, p = 0,041) y en la dosis de prednisona acumulada a 6 meses (12,8 mg ± 4,9 PDNi 30 mg/d, p =0,008). No hubo diferencias significativas en la proporción de pacientes que alcanzaron la respuesta completa, en el tiempo hasta alcanzarla ni en los efectos adversos entre ambos grupos. Conclusiones: el esquema terapéutico del grupo PDNi <30 mg/d se asoció a una menor dosis acumulada de prednisona y una respuesta al tratamiento comparable, lo que hace presumir menor daño acumulado relacionado al uso de glucocorticoides.

Abstract: Introduction: current recommendations to treat lupus nephritis (LN) point to low-dose glucocorticoids to control the disease and avoid cumulativedamage. Objective: to learn about and compare the response of patients with proliferative LN who are treated following two prednisone therapy guidelines: reduced initial doses 30 mg/d during the induction stage. Method: clinical, analytical and therapeutic guidelines of patients with proliferative LN were compared and classified into two groups according to the standard or low-dose initial prednisone dose. Results: 21 patients with proliferative LN were studied (n=12 low-dose initial prednisonevs. n=9 standard initial prednisone). No significant differences were found between clinical and analytical variables, although a significantly different statistic difference was observed in the number of methylprednisone pulses (5 ± 2.95 initial prednisone 30 mg/d, p = 0.041) and in the prednisone dose accumulated in 6 months (12.8 mg ± 4.9 initial prednisone 30 mg/d, p =0.008). No significant differences were seen between both groups in the proportion of patients who achieved complete response, neither in terms of the time it took to achieve it or in the side effects. Conclusions: the treatment plan for the initial prednisone <30 mg/d was associated to a lower cumulative dose of response prednisone considering the comparable treatment, what suggests there being smaller cumulative harm as a consequence of the use of glucocorticoids.

Resumo: Introdução: as recomendações atuais para o tratamento da nefrite lúpica (NL) estão orientadas a doses de glicocorticoides mais baixas para controlar a enfermidade e evitar o dano acumulado. Objetivo: conhecer e comparar a resposta ao tratamento de pacientes com NL proliferativa na etapa de indução com duas pautas de tratamento com prednisona (PDN): doses iniciais reduzidas 30 mg/d. Método: foram comparadas variáveis clínicas, analíticas e terapêuticas de pacientes com NL proliferativa divididos em dois grupos segundo a dose inicial de prednisona (PDNi) padrão ou reduzida. Resultados: 21 pacientes com NL proliferativa (n=12 PDNi reduzida vs. n=9 PDNi estândar) foram estudados. Não foram observadas diferenças significativas nas variáveis clínicas e analíticas. Observou-se uma diferença estatisticamente significativa no número de pulsos de metilprednisolona (5 ± 2,95 PDNi 30 mg/d, p = 0,041) e nas doses de prednisona acumulada aos 6 meses (12,8 mg ± 4,9 PDNi 30 mg/d, p =0,008). Não foram observadas diferenças significativas na proporção de pacientes que alcançaram a resposta completa, no tempo até alcançá-la nem nos efeitos adversos entre ambos grupos. Conclusões: o esquema terapêutico do grupo PDNi <30 mg/d foi associado a uma menor dose acumulada de prednisona em resposta ao tratamento comparável, o que sugere menos dano cumulativo relacionado ao uso de glicocorticoides.

Calidad en la asistencia en neumonía aguda comunitaria en una clínica médica del Hospital de Clínicas en el período de 2010 a 2014 / Quality of health care for patients with acute pneumonia in a medical clinic of the university hospital between 2010 and 2014 / Qualidade da assistência em pneumonia aguda comunitária em uma clínica médica do Hospital de Clínicas no período 2010-2014

Resumen: Introducción: el cumplimiento de las guías de práctica clínica en el manejo de la neumonía aguda comunitaria se desconoce en Uruguay. Objetivo: evaluar el cumplimiento de las guías de práctica clínica en pacientes inmunocompetentes hospitalizados con diagnóstico de neumonía aguda comunitaria en una Clínica Médica del Hospital de Clínicas. Metodología: estudio observacional retrospectivo. Se estudiaron variables patronímicas, clínicas e indicadores de calidad asistencial de acuerdo con estándares internacionales. El nivel de cumplimiento de los indicadores se clasificó como óptimo cuando el cumplimiento fue > 90%, intermedio entre 60% y 90% y bajo cuando fue < 60%. Se utilizaron como guías de referencia ConsenSur II e IDSA. Resultados: se incluyeron 143 pacientes, 62,9% de sexo femenino (88/140), con una mediana de edad de 57 (38-73) años. La población estudiada se caracterizó por una mediana de Charlson de 1 (0-3), mediana de internación de 10 (7-14) días y 26,6% (38/143) de pacientes con criterios de neumonía severa. En cuanto al cumplimento de indicadores de calidad: ninguno se cumple en nivel óptimo, tres en nivel intermedio (hemocultivos en pacientes con neumonía severa, hemocultivos previo a antibióticos y administración de antibióticos dentro de las primeras 8 horas), mientras que cuatro variables que evalúan el uso de antibiótico apropiado, según las guías y la prevención de la neumonía, tienen un cumplimiento en nivel bajo. Conclusiones: ninguno de los indicadores evaluados alcanzó un nivel óptimo. Se deben realizar intervenciones para mejorar la calidad en asistencia en la neumonía.

Summary: Introduction: the extent to which clinical practice guidelines in the handling of community acquired acute pneumonia are observed is not known in Uruguay. Objective: to assess the degree of compliance with the clinical guidelines in immunocompetent patients hospitalized with a diagnosis of community-acquired pneumonia in a medical clinic of the University Hospital. Method: observational, retrospective study. Patronymic and clinical variables, and quality of health care indicators were analysed as per international standards. The degree of compliance with indicators was classified as ideal when compliance was > 90%, intermediate when it was between 60% and 90% and low when it was < 60%. ConsenSur II and IDSA were the reference guidelines used. Results: 143 patients were included in the study, 6.9 % were female (88/140), with a median age of 57 years old (38-73). The population studied was characterized by Charlson median of 1 (0-3), hospitalization median of 10 (7-14) days and 26.6% (38/143) of patients with acute pneumonia criteria. As to compliance with quality of health care indicators: none of them is ideal, 3 in intermediate level (hemoculture in patients with acute pneumonia, hemoculture prior to antibiotic therapy and administration of antibiotics within the first 8 hours), while 4 variables that assess the use of the appropriate antibiotic according to the guidelines and the prevention of pneumonia evidence a low level of compliance. Conclusions: none of the assessed indicators evidenced an ideal level of compliance. Interventions are required to improve quality in health care for patients with pneumonia.

Resumo: Introdução: o cumprimento das guias de prática clínica no manejo da pneumonia aguda comunitária não é conhecido no Uruguai. Objetivo: avaliar o cumprimento das guias de prática clínica em pacientes imunocompetentes hospitalizados com diagnóstico de pneumonia aguda comunitária em uma Clínica Médica do Hospital de Clínicas. Metodologia: estudo observacional retrospectivo. Foram estudadas variáveis patronímicas e clínicas, e indicadores de qualidade assistencial de acordo com padrões internacionais. O nível de cumprimento dos indicadores foi classificado como ótimo quando o cumprimento foi > 90%, intermédio entre 60 e 90% e baixo quando foi < 60%. As guias ConsenSur II e IDSA foram utilizadas como guias de referência. Resultados: foram incluídos 143 pacientes, sendo 62,9 % de sexo feminino (88/140), com una mediana de idade de 57 (38-73) anos. A população estudada caracterizou-se por uma mediana de Charlson de 1 (0-3), mediana de internação de 10 (7-14) dias e 26,6% (38/143) dos pacientes com critérios de pneumonia severa. Com relação ao cumprimento de indicadores de qualidade: nenhum em nível óptimo, 3 em nível intermédio (hemoculturas em pacientes com pneumonia severa, hemoculturas prévia a antibióticos e administração de antibióticos nas primeiras 8 horas), enquanto 4 variáveis que avaliam o uso de antibiótico apropriado segundo as guias e a prevenção da pneumonia tem um cumprimento em nível baixo. Conclusões: nenhum dos indicadores avaliados alcançou um nível ótimo. É necessário realizar intervenções para melhorar a qualidade da assistência na pneumonia.

Hepatitis in patients with syphilis: an overlooked association.

We present the case of a patient who sought treatment for fever and a maculopapular rash involving the trunk, limbs, palms and soles. The patient also presented with hepatomegaly and elevated levels of liver enzymes (with a higher increase of alkaline phosphatase). With the proposal of early syphilitic hepatitis, during the stage of secondary syphilis, a venereal disease research laboratory and Treponema pallidum haemagglutination tests were requested, which confirmed the diagnosis. All altered parameters improved with antibiotic treatment for secondary syphilis. Syphilitic hepatitis is an often overlooked presentation of syphilis and should be considered as a differential diagnosis in patients with elevated levels of liver enzymes and risk factors for syphilis.

Hypoxic hepatitis and furosemide.

Hypoxic hepatitis is a rather common complication of heart, circulatory or respiratory failure. We present the case of a patient with hypoxic hepatitis in the setting of heart failure and dehydration from furosemide as a reminder of an important clinical lesson. The pathogenesis of hypoxia (especially in the case of heart failure) is explained by a two-hit mechanism in which the liver at risk of hypoxic injury by passive hepatic congestion (right heart failure) is subsequently exposed to systemic hypoperfusion, which leads to a marked and transient elevation of aminotransferases. In the case presented, the use of furosemide (at least partially) promoted the second hit because it helped to generate hypotension and splanchnic hypovolaemia and favoured hepatic hypoxia.

The Clinician Faced With Anticochlear Antibodies: What to Do With Them?

BACKGROUND: Autoimmune inner ear disease as a cause of sensorineural hearing loss is a poorly understood entity. Thus, the role of anticochlear antibodies (ACLAs) in clinical management is still not well established. OBJECTIVE: The aims of this study were to describe the use of ACLAs in our clinical setting and to understand the clinicians' therapeutic approach in these cases. We also analyzed the usefulness of these autoantibodies in clinical practice. METHODS: A retrospective study with nonprobabilistic convenience sampling of patients who were tested for ACLAs in the period from January 1, 2013, to December 31, 2015, was performed. The study was carried out in 2 stages: (1) medical records of all patients who were investigated for ACLAs were reviewed. The following variables were analyzed: age, sex, reason for requesting ACLAs, concomitant autoimmune disease, audiogram, immunosuppressive treatment, duration of treatment, and clinical response; (2) patients who received immunosuppressive therapy were contacted by telephone. A visual analog scale (VAS) (0-10) was applied to evaluate the therapeutic response. RESULTS: Thirty-nine patients who were investigated for ACLAs were identified. The mean age was 41 (SD, 16) years; there were 33 female patients. Of the 34 patients with ACLA-positive antibodies, 16 patients received immunosuppressive agents, of for management of their sensorineural hearing loss, corticosteroids was the most commonly used treatment. No clinical improvement was reported by patients after immunosuppressive treatment in this subgroup. CONCLUSIONS: The role of ACLAs in the diagnosis and management of sensorineural hearing loss remains unclear. In this small study at a single institution, ACLA testing may not have improved the outcome of sensorineural hearing loss.

Capilaroscopía en el diagnóstico de las enfermedades autoinmunes sistémicas / Capillaroscopy in the diagnosis of systemic autoimmune disease

Introducción: la capilaroscopía del pliegue ungueal (CPU) consiste en la observación in vivo de la microcirculación capilar, donde habitualmente pueden describirse tres patrones (tortuoso, esclerodermiforme y normal). Objetivo: describir las alteraciones capilares en pacientes queconsultaron en la Unidad de Enfermedades Autoinmunes Sistémicasdel Hospital de Clínicas entre agosto de 2009 y octubre de 2010. Pacientes, materiales y métodos: realizamos un estudio descriptivo, retrospectivo, cualitativo de los patrones capilaroscópicos. Resultados: se revisaron historias clínicas y CPU de 110 pacientes (102 mujeres), con una media de edad 46,6 ± 17,5 años siendo el grupo mayoritario representado por 34 (31%) pacientes con esclerosis sistémica. Los patrones en la CPUhallados fueron normales en 38% y patológicos en 62% de los pacientes. El 88% de los pacientes con esclerosis sistémica presentaron una CPU patológica, de estos, 74% correspondióa un patrón esclerodermiforme. En pacientes con enfermedades autoinmunes (exceptuando la esclerosis sistémica) encontramos un patrón patológico en 66% (27% correspondieron a un patrón esclerodermiforme). Conclusiones: la CPU contribuyó en distintos aspectos en el estudio del fenómeno de Raynaud y de enfermedades autoinmunes. La detección de un patrón esclerodermiforme fue altamentesugestiva de la presencia de una enfermedad autoinmune sistémica. La CPU, junto a los hallazgos clínicos y marcadores biológicos adecuados, adquiere valor y especificidad en el diagnóstico, debiendo formar parte de la valoración clínica de pacientes con fenómeno de Raynaud y sospecha clínicao analítica de enfermedades autoinmunes sistémicas.

Introduction: nailfold capillaroscopy (NC) consists of the in vivo observation of capillary microcirculation, which usually presents three patterns (tortuos, sclerodermiform and normal).Objective: to describe capillary alterations in patients who consulted at the Systemic Autoimmune DiseasesUnit of the Clínicas Hospital, between August 2009 and October 2010.Patients, material and methods: we conducted a descriptive, retrospective and qualitative study of capillaroscopypatterns. Results: the medical records and NC of 110 patients were reviewed (102 women), average age was 46.6 ±17.5 years old, being the largest group represented by 34 (31%) patients with systemic sclerosis. Patterns foundin the NC were normal in 38% of cases and pathological in 62% of them. Eighty eight per cent of patients withsystemic sclerosis presented a pathological NC, and 74% of the latter corresponded to a sclerodermiformpattern. We found a pathololgical pattern in 66% of patients with autoimmune diseases (except for systemic sclerosis), where 27% corresponded to a sclerodermiformpattern. Conclusions: NC contributed to the study of the Raynaud phenomenon and autoinmune diseases indifferent ways. Identifying a sclerodermiform pattern highly suggested the presence of a systemic autoimmune disease. The NC, together with clinical findings and the appropriate biological markers gains value and specificity in the diagnosis, and it thus should be a part of the clinical assessment of patients with the RaynaudÆs disease and a clinical or analytical suspicion of systemicautoimmune disease.

Introdução: a capilaroscopia periungueal (CPU) consiste na observaçao in vivo da rede microvascular da região periungueal, onde habitualmente se podem descrevertrês padrões: tortuoso, esclerodermiforme e normal.Objetivo: descrever as alterações capilares em pacientes que consultaram na Unidade de Doenças Auto-imunes Sistêmicas do Hospital de Clínicas entreagosto de 2009 e outubro de 2010.Pacientes, materiais e métodos: realizamos um estudio descritivo, retrospectivo, qualitativo dos padrões capilaroscópicos. Resultados: revisamos o prontuário médico e CPU de 110 pacientes (102 mulheres), com idade média de 46.6 ± 17.5 anos; 34 (31%) eram pacientes com esclerose sistêmica. Os padrões encontrados na CPU foramnormais em 38% e patológicos em 62% dos pacientes. 88% dos pacientes com esclerose sistêmica apresentaramCPU patológica; destes, 74% correspondeu a um padrão esclerodermiforme. En pacientes com doenças auto-imunes (excluindo a esclerose sistêmica) encontramos um padrão patológico em 66% (27% correspondeu a um padrão esclerodermiforme). Conclusões: a CPU contribuiu em diferentes aspectosao estudo do fenômeno de Raynaud e de doenças auto-imunes.Adetecção de umpadrão esclerodermiforme foi um forte indicio da presença de uma enfermedad auto-imune sistêmica. A CPU, juntamente com outros aspectosclínicos e marcadores biológicos adequados, tem valor e especificidade no diagnóstico, devendo formar parte da avaliação clínica de pacientes com fenômeno de Raynaud e suspeita clínica ou analítica de doenças auto-imunes sistêmicas.

Manifestaciones clínicas en pacientes con esclerosis sistémica / Clinical presentation in patients with systemic sclerosis

Introducción: la esclerosis sistémica es una enfermedad autoinmune sistémica caracterizada por daño endotelial, inflamación y fibrosis de piel, vasos y órganos internos. Existen diferencias en frecuencia, gravedad y pronóstico en diferentes grupos étnicos, lo que remarca la importancia del estudio en cada región geográfica, para poder diagnosticar tempranamente sus manifestaciones incipientes. Material y método: realizamos un estudio descriptivo y retrospectivo entre marzo de 2006 y marzo de 2008, incluyendo pacientes con diagnóstico definitivo de esclerosis sistémica, asistidos en la Unidad de Enfermedades Autoinmunes Sistémicas del Hospital de Clínicas.Resultados: fueron incluidas 31 mujeres, con 39,2 meses de seguimiento promedio, edad mediaal diagnóstico de 47,6 años. Once pacientes (35,5) presentaron enfermedad difusa y 20 (64,5) enfermedad limitada. Treinta presentaron fenómeno de Raynaud. En 92 la capilaroscopia mostró un patrón esclerodermiforme. A nivel respiratorio encontramos intersticiopatía en 25, hipertensión arterial pulmonar en 22,2 y un patrón restrictivo enestudios de función respiratoria en 35,5. El 67,7 presentó manifestaciones digestivas y 9,6 desarrolló una crisis renal esclerodérmica. Hallamos anticuerpos antinucleares (ANA) en 29/31 (93,5) pacientes; 16 presentaron anticuerpos anticentromero y cinco antitopoisomerasa-I. Las cuatro (12,9) pacientes fallecidas durante el seguimiento presentaron elementos comunes como esclerosis sistémica difusa, úlceras digitales y compromisorespiratorio severo. Conclusiones: las características clínicas e inmunológicas encontradas fueron similares a lasdescriptas en otras series. En ausencia de tratamiento específico, debe insistirse en la valoración periódica de las repercusiones viscerales para controlar y retrasar la instalaciónde complicaciones con elevada morbimortalidad.

Introduction: systemic sclerosis is an autoimmune disease characterized by endothelial damage, and skin, vesseland internal organ fibrosis and inflammation. There are differences in terms of frequency, severity and prognosis for the different ethnic groups, what reinforces the importanceof the study in each geographical region with the purpose of enabling early diagnosis of its incipient symptoms.Methods: we conducted a descriptive and retrospective study form March 2006 through March 2008, includingpatients with a final diagnosis of systemic sclerosis, who are treated at the Systemic Autoimmune DiseasesUnit at the Clínicas Hospital. Results: 31 women were included in the study, average follow-up of patients was 39.2 months, and average age at the time of diagnosis was 47.6 years. Eleven patients (35,5) presented diffuse disease and 20 (64.5) of them evidenced limited disease. Thirty patients presented RaynaudÆs phenomenon. In 92 of cases capilaroscopy showed a sclerodermiform pattern. In terms of the respiratory system, we found interstilial pathology in 25 of cases, pulmonary arterial hypertension in 22.2 and arestrictive pattern in respiratory function studies in 35.5. Also, 67.7 presented digestive manifestations and 9.6 developed sclerodermic renal crisis. We found anti-nuclear antibodies (ANA) in 29 out of 31 patients (93,5) patients; 16 presented anticentromere antibodies and five anti-topoisomerasa-I antibodies. The four patients (12.9)who died during follow-up presented common elements such as diffuse sclerosis, digital ulcers and severe respiratory compromise. Conclusions: the clinical and immune characteristicsfound in our study were similar to those described in other series. Should there be no specific treatment, it is essentialto perform regular assessment of visceral impact in order to control and delay complications which result in high morbimortality rates.

Introdução: a esclerose sistêmica é uma doença autoimune sistêmica caracterizada por dano endotelial, inflamação e fibrose de pele, vasos e órgãos internos. Existem diferenças na freqüência, severidade e prognóstico nosdiferentes grupos étnicos, por isso é importante estudá-la em diferentes regiões geográficas para o diagnóstico precoce de suas manifestações. Material e método: realizamos um estudo descritivo e retrospectivo entre março de 2006 e março de 2008, incluindo pacientes com diagnóstico definitivo deesclerose sistêmica, atendidos na Unidade de Doenças Auto-imunes Sistêmicas do Hospital das Clínicas.Resultados: foram incluídas 31 mulheres, com um seguimento médio de 39,2 meses, idade média no diagnósticode 47,6 anos. Onze pacientes (35,5) apresentaram doença difusa e 20 (64,5) doença limitada. Trintaapresentaram fenômeno de Raynaud. Em 92 a capilaroscopia mostrou um padrão esclerodermiforme. No aparelho respiratório encontramos intersticiopatia em 25, hipertensão arterial pulmonar em 22,2 e um padrão restritivo nos estudos de função respiratória em 35,5. 67,7 apresentaram manifestações digestivas e 9,6 crise renal esclerodérmica. Em 29/31 (93,5) pacientes encontramosanticorpos antinucleares (ANA); 16 apresentaram anticorpos anticentrômero e cinco anti-topoisomerase-I. As quatro (12,9) pacientes que faleceram durante oseguimento apresentaram elementos comuns como esclerose sistêmica difusa, úlceras digitais e compromissorespiratório severo. Conclusões: as características clínicas e imunológicasencontradas foram similares às descritas em outras séries. Na ausência de tratamento específico, deve-se insistir na avaliação periódica das repercussões viscerais para controlare retardar a instalação de complicações com morbimortalidade elevada.