Controversias en neuroinmunología: esclerosis múltiple, vacunación, SARS-CoV-2 y otros dilemas / Controversies in neuroimmunology: multiple sclerosis, vaccination, SARS-CoV-2 and other dilemas

La neuroinmunología es una disciplina que cada vez amplía más sus horizontes en la comprensión de las enfermedades neurológicas. Contemporáneamente, y a la luz de los nexos fisiopatológicos de las enfermedades neurológicas y la inmunología, se han planteado enfoques diagnósticos y terapéuticos específicos. A pesar de los importantes avances de esta disciplina, existen múltiples dilemas que le conciernen y se filtran en la práctica clínica. En esta revisión, se presentan y discuten 15 controversias, las cuales se construyen con la información clínica disponible más actualizada. Los temas incluidos son: disminución de esteroides en recaídas de esclerosis múltiple; recomendaciones terapéuticas en esclerosis múltiple a la luz de la pandemia por el SARS-CoV-2; evidencia de vacunación en esclerosis múltiple y en otras enfermedades desmielinizantes; panorama actual del síndrome clínico y radiológico aislado; y fallas terapéuticas en esclerosis múltiple; además, criterios para suspender las terapias modificadoras de la enfermedad; evidencia del manejo en recaídas leves; recomendaciones para la profilaxis contra Strongyloides stercolaris; utilidad de un segundo ciclo de inmunoglobulina en el síndrome de Guillain-Barré; criterios para diferenciar una polineuropatía crónica desmielinizante inflamatoria de inicio agudo de un síndrome de Guillain-Barré y, utilidad de la enzima convertidora de angiotensina en neurosarcoidosis. En cada una de las controversias, se presenta la problemática general y se ofrecen recomendaciones específicas que pueden adoptarse en la práctica clínica diaria.

Neuroimmunology is a discipline that increasingly broadens its horizons in the understanding of neurological diseases. At the same time, and in front of the pathophysiological links of neurological diseases and immunology, specific diagnostic and therapeutic approaches have been proposed. Despite the important advances in this discipline, there are multiple dilemmas that concern and filter into clinical practice. This article presents 15 controversies and a discussion about them, which are built with the most up-to-date evidence available. The topics included in this review are: steroid decline in relapses of multiple sclerosis; therapeutic recommendations in MS in light of the SARS-CoV-2 pandemic; evidence of vaccination in multiple sclerosis and other demyelinating diseases; overview current situation of isolated clinical and radiological syndrome; therapeutic failure in multiple sclerosis, as well as criteria for suspension of disease-modifying therapies; evidence of the management of mild relapses in multiple sclerosis; recommendations for prophylaxis against Strongyloides stercolaris; usefulness of a second course of immunoglobulin in the Guillain-Barré syndrome; criteria to differentiate an acute-onset inflammatory demyelinating chronic polyneuropathy versus Guillain-Barré syndrome; and, the utility of angiotensin-converting enzyme in neurosarcoidosis. In each of the controversies, the general problem is presented, and specific recommendations are offered that can be adopted in daily clinical practice.

Epidemiological characterization of patients with sarcoidosis in a high complexity hospital in southwestern Colombia

Introduction: Epidemiological studies on sarcoidosis in Colombia are scarce, and although recent reports from the north of the country have been published, clinical-epidemiological associations are not clear. Our aim was to characterize patients with sarcoidosis diagnosed at Fundación Valle del Lili in Cali, Colombia. Methods: A retrospective study of a series of sarcoidosis cases was conducted between 2011 and 2019. Demographic, clinical, laboratory, imaging, histopathological, and treatment variables were analyzed. Results: A total of 34 patients with a diagnosis of sarcoidosis were found. The majority were women (n = 25; 73%), and the mean age was 50 years. The main symptoms of onset were erythema nodosum (n = 11; 33%), arthritis (n = 10; 30%), and cough (n = 9; 27%). In 64% of the cases, there was pulmonary involvement, with pulmonary nodules, mediastinal adenopathy, and interstitial lung disease found in 54%, 50%, and 36% of cases, respectively. In 85% of cases, there were extrapulmonary manifestations, mainly cutaneous (50%). Angiotensin-converting enzyme (ACE) was elevated in 62% of the cases in which it was measured (n = 16; 47%). During the diagnostic process, 23 biopsies were performed, of which 95% showed granulomas with noncaseating necrosis. Most of the patients (76%) were controlled with prednisolone, at an average dose of 20 mg (7.5-50 mg) per day. Conclusions: Sarcoidosis was more frequent in women and mestizos, and it presented earlier in men. Elevated ACE was not associated with extrapulmonary involvement. Calcium-phosphorus profile and antinuclear antibodies were not useful to establish the diagnosis.

Introducción: En Colombia son escasos los estudios epidemiológicos sobre la sarcoidosis; aunque recientemente se han publicado reportes del norte del país, son grupos muestrales pequeños, por lo que no quedan claras las asociaciones clínico-epidemiológicas. Nuestro objetivo fue caracterizar pacientes con sarcoidosis en la Fundación Valle del Lili, en Cali, Colombia. Métodos: Se realizó un estudio retrospectivo de una serie de casos de sarcoidosis entre el 2011 y el 2019. Se analizaron variables demográficas, clínicas, de laboratorio, imagenológicas, histopatológicas y de tratamiento. Resultados: Se encontraron 34 pacientes con diagnóstico de sarcoidosis, la mayoría fueron mujeres (n = 25; 73%), y la edad promedio fue 50 anos. Los principales síntomas de inicio fueron eritema nudoso (n = 11; 33%), artritis (n = 10; 30%) y tos (n = 9; 27%). En el 64% de los casos hubo compromiso pulmonar, y se encontraron nódulos pulmonares, adenopatías mediastinales y enfermedad pulmonar intersticial en un 54, 50 y 36% de los casos, respectivamente. En el 85% de los casos hubo manifestaciones extrapulmonares, principalmente cutáneas (50%). Los niveles de enzima conversora de angiotensina estuvieron elevados en el 62% de los casos en los que fue medida (n = 16; 47%). Durante el proceso diagnóstico se realizaron 23 biopsias, de las cuales el 95% evidenció granulomas con necrosis no caseificante. La mayoría de los pacientes (76%) fueron controlados con prednisolona, a una dosis promedio de 20 mg (7,5-50 mg) por día. Conclusiones: La sarcoidosis fue más frecuente en mujeres y mestizos. La presentación fue más temprana en hombres. La enzima conversora de angiotensina no se relacionó con compromiso extrapulmonar. Ni el perfil fósforo-calcio ni los anticuerpos antinucleares fueron útiles para establecer el diagnóstico.

Prognostic features of sarcoidosis course in a Brazilian cohort / Características prognósticas da evolução da sarcoidose em uma coorte brasileira

ABSTRACT Objective: To identify predictive features associated with the course of sarcoidosis at initial evaluation and to develop a predictive score. Methods: This was a retrospective study involving pulmonary sarcoidosis patients, classified as having a self-limited or persistent course of disease, comparing data between the outcomes by univariate analysis. Features related to persistent disease were selected by multivariate analysis and a prognostic score was designed. Results: The sample comprised 200 patients (mean age = 49 years). The median duration of symptoms to diagnosis was 12 months, and delayed diagnosis (> 12 months) was found in 43% of the cases. The most common radiological stage was II; 37% had reduced FVC. Relevant systemic involvement was detected in 37% of the patients. Treatment for tuberculosis was prescribed in 44 patients prior to sarcoidosis diagnosis. Treatment for sarcoidosis was required in 77% of the sample, and the disease course was persistent in 115 cases. Excluding 40 patients with fibrotic disease, prognostic factors to persistent disease were parenchymal involvement, delayed diagnosis, dyspnea, relevant systemic involvement, and reduced FVC. On the basis of the analysis, a 3-letter scoring system (A, B and C) was developed according to the selected factors. The positive predictive values for persistent course for A (≤ 1 point) and C scores (≥ 4 points) were 12.5% and 81.8%, respectively. Conclusions: A score can be derived by selected features at initial evaluation, allowing the prediction of outcomes in a significant number of sarcoidosis patients.

RESUMO Objetivo: Identificar características preditivas associadas à evolução da sarcoidose na avaliação inicial e desenvolver um escore preditivo. Métodos: Estudo retrospectivo com pacientes com sarcoidose pulmonar, classificados como tendo evolução autolimitada ou persistente da doença, comparando dados entre os desfechos por meio de análise univariada. Características relacionadas à doença persistente foram selecionadas por meio de análise multivariada, e foi desenvolvido um escore prognóstico. Resultados: A amostra foi composta por 200 pacientes (média de idade = 49 anos). A mediana da duração dos sintomas até o diagnóstico foi de 12 meses, e houve diagnóstico tardio (> 12 meses) em 43% dos casos. O estádio radiológico mais frequente foi o II; 37% apresentavam redução da CVF. Envolvimento sistêmico relevante foi detectado em 37% dos pacientes. Tratamento para tuberculose foi prescrito em 44 pacientes antes do diagnóstico de sarcoidose. Tratamento para sarcoidose foi necessário em 77% da amostra, e a evolução da doença foi persistente em 115 casos. Com a exclusão de 40 pacientes com doença fibrótica, os fatores prognósticos para doença persistente foram comprometimento parenquimatoso, diagnóstico tardio, dispneia, comprometimento sistêmico relevante e redução da CVF. Com base na análise, foi desenvolvido um sistema de pontuação por letras (A, B e C) de acordo com os fatores selecionados. Os valores preditivos positivos para evolução persistente para as pontuações A (≤ 1 ponto) e C (≥ 4 pontos) foram de 12,5% e 81,8%, respectivamente. Conclusões: É possível derivar um escore por meio de características selecionadas na avaliação inicial, permitindo a predição de desfechos em um significativo número de pacientes com sarcoidose.

Sarcoidosis con compromiso cutáneo casuística del Servicio de Dermatología del Hospital Nacional- período 2010-2020 / Sarcoidosis with skin engagement casuistics of the National Hospital Dermatology Service - period 2010-2020

Introducción: La sarcoidosis es una enfermedad inflamatoria, granulomatosa de etiología desconocida. Puede afectar cualquier órgano siendo la afectación pulmonar la más frecuente. La piel se compromete en aproximadamente 30% de los casos, pudiendo ser inicial o único. La sarcoidosis es muy rara en el Paraguay. Objetivos: Realizar una revisión de los casos de sarcoidosis observados en el Servicio de Dermatología del Hospital Nacional en el periodo 2010-2020; determinando sus características demográficas, clínicas, laboratoriales y evolutivas. Materiales y métodos: Estudio retrospectivo, descriptivo y observacional. Resultados: Se diagnosticaron 5 casos, todas mujeres de raza blanca, con edades que oscilaban de 41 a 63 años. Las formas clínicas fueron papulosa en 3 casos, en placa 1 y mixta 1. Las lesiones cutáneas eran asintomáticas y estaban ubicadas preferentemente en rostro (4 casos). El compromiso sistémico se objetivó en las primeras consultas en 2 casos (pulmonar, ocular y ganglionar en 1 y del aparato lagrimal en otro). En todos los casos el diagnóstico se fundamentó en la correlación clínico-histológica, con el hallazgo de los granulomas sarcoides. Todos recibieron clobetasol tópico, 3 pacientes ciclos de prednisona y 2 hidroxicloroquina. La evolución fue favorable en 3 casos y 2 no volvieron a sus controles. Conclusión: La sarcoidosis con manifestaciones cutáneas es rara en nuestro Servicio, pero representa un desafío diagnóstico, que debe ser oportuno para diferenciarla de otras patologías más prevalentes en nuestro medio

Introduction: Sarcoidosis is an inflammatory, granulomatous disease of unknown etiology. It can affect any organ, lung involvement being the most frequent. The skin is compromised in approximately 30% of the cases, and can be initial or unique. Sarcoidosis is very rare in Paraguay. Objectives: To carry out a review of the sarcoidosis cases observed in the Dermatology Service of the National Hospital in the period 2010-2020; determining their demographic, clinical, laboratory and evolutionary characteristics. Materials and methods: Retrospective, descriptive and observational study. Results: 5 cases were diagnosed; all white women; with ages ranging from 41 to 63 years. The clinical forms were papular in 3 cases, in plate 1 and mixed 1. The skin lesions were asymptomatic and were preferably located on the face (4 cases). Systemic involvement was observed in the first consultations in 2 cases (pulmonary, ocular and lymph node in 1 and the lacrimal apparatus in another). In all cases the diagnosis was based on the clinical-histological correlation, with the finding of sarcoid granulomas. All received topical clobetasol, 3 patients received prednisone cycles and 2 hydroxychloroquine. The evolution was favorable in 3 cases and 2 did not return to their controls. Conclusion: Sarcoidosis with skin manifestations is rare in our Service, but it represents a diagnostic challenge that must be timely to differentiate it from other more prevalent pathologies in our setting

Diagnóstico diferencial en pacientes con granulomas en biopsias de pulmón, pleura y/o ganglios mediastínicos del Instituto Nacional del Tórax / Differential diagnosis in patients with granulomas in the biopsy of the lung, pleura and/or mediastinal nodes

Las enfermedades granulomatosas incluyen una amplia gama de enfermedades. Sin embargo, en la práctica clínica, muchos casos de enfermedad granulomatosa permanecen sin etiología después del examen histológico. Nuestro objetivo fue determinar, a partir de las biopsias de pulmón, pleura y ganglios linfáticos mediastínicos, en los que se informaron granulomas, las características clínicas y los diagnósticos de estos pacientes. Así también la mortalidad a un año de seguimiento. Metodología: Analizamos retrospectivamente biopsias de pulmón, pleura y/o ganglios linfáticos mediastínicos con granulomas de 75 pacientes del Instituto Nacional del Tórax (2012-2016), sus características clínicas y de laboratorio. La información se obtuvo de los registros médicos. Los datos de mortalidad se obtuvieron del registro civil. Resultados: Se determinó una etiología en todos los casos, excepto en 3 (4%). Los diagnósticos más frecuentes fueron tuberculosis (n = 37; 49%) y sarcoidosis (n = 18; 24%). Otras causas fueron silicosis (5,3%), vasculitis (4%) y neumonitis por hipersensibilidad (2,7%). Los pacientes con tuberculosis (TB) tenían parámetros inflamatorios más altos, como velocidad de eritrosedimentación y proteína C reactiva. Además, sólo se encontraron granulomas con necrosis caseosa en pacientes con tuberculosis. En cambio, los pacientes con sarcoidosis tenían lesiones cutáneas y una mayor frecuencia de linfadenopatías. Cuatro (5.3%) pacientes fallecieron a un año de seguimiento: dos debido a neumonía, uno por hipersensibilidad crónica y uno por TB. Conclusión: La tuberculosis y la sarcoidosis fueron las causas más frecuentes de granulomas respiratorios en este estudio retrospectivo. Se logró determinar una etiología en el 96% de los casos, considerando variables clínicas, de laboratorio e histopatológicas para un diagnóstico diferencial correcto.

Granulomatous diseases comprise a wide range of pathologies. However, in clinical practice, many pulmonary granulomas remain without etiology after the histologic examination. Our aim was to determine from the biopsies of the lung, pleura and mediastinal lymph nodes in which granulomas were reported, the clinical characteristics and diagnoses of the patients. Methodology: We analyzed retrospectively biopsies of the lung, pleura and mediastinal lymph nodes with granulomas from 75 patients handled at our institution (2012-2016), as well as their clinical and laboratory data. The information was obtained from medical records. A one-year mortality date was obtained from the civil registry. Main results: A cause was determined in all the cases, except in three of them (4%). The most frequent diagnoses were tuberculosis (n =37; 49%) and sarcoidosis (n =18; 24%). Other causes were silicosis (5.3%), vasculitis (4%) and hypersensitivity pneumonitis (2.7%). Patients with tuberculosis (TB) had higher inflammatory parameters such as erythrocyte sedimentation rate and C-reactive protein. Besides granulomas with caseous necrosis were only found in TB patients. Instead, patients with sarcoidosis had skin lesions and a higher frequency of lymphadenopathy. Four patients (5.3%) died in a one-year of follow-up: two of them because of pneumonia and the other two patients because of chronic hypersensitivity and TB respectively. Conclusion: Tuberculosis and sarcoidosis were the most common causes of respiratory granulomas in this retrospective study. A specific cause was determined in 96% of cases, considering clinical, laboratory and histopathological variables to do a right differential diagnosis.

Renal manifestations of sarcoidosis: from accurate diagnosis to specific treatment

ABSTRACT Sarcoidosis is a multisystem granulomatous disease characterized by epithelioid noncaseating granulomas associated with clinical and radiologic findings. The cause of this disease is still uncertain. Sarcoidosis affects mostly lungs and lymph nodes and is not usually considered a urological disease, therefore, this etiology may be overlooked in several urological disorders, such as hypercalcemia, hypercalciuria and nephrolithiasis. It affects all races and genders. This review aims to describe the urological manifestations of sarcoidosis and to elucidate how the disease may affect the management of numerous urological conditions.

Taquicardia ventricular como manifestación de sarcoidosis cardíaca. Reporte de un caso / Ventricular tachycardia as a manifestation of cardiac sarcoidosis. Case report

La sarcoidosis es una enfermedad inflamatoria crónica que posee una amplia variabilidad de presentaciones clínicas. El compromiso cardíaco de esta patología oscila en el orden del 5%, pudiendo variar desde anormalidades en la conducción hasta la falla cardíaca. Existe poco conocimiento sobre los beneficios de su tratamiento y no hay datos que evalúen la reducción de la mortalidad a largo plazo, por lo que genera este tipo de pacientes un gran desafío para el profesional. A continuación presentamos el caso de un masculino de 37 años con taquicardia ventricular como debut cardiológico de una sarcoidosis.

Sarcoidosis of the breast: an unusual clinical presentation / Sarcoidosis da mama: uma apresentação clinica não usual

Introduction: sarcoidosis is a chronic systemic granulomatous disease of unknown etiology. The breast may be involved in less than 1% of cases and often mimics carcinomas at clinical examination. Case report: herein, the authors report a 54-year-old woman who had history of erythema nodosum, had clinical presentation suggestive of mastalgia whose mammography and ultrasound showed bilateral unspecific heterogeneous parenchymal densities. Histopathological study revealed non-caseous giant cell granulomas. Specifically, serum levels of angiotensin-converting enzyme (ACE) were elevated and a CT scan showed small bilateral pulmonary nodules distributed along the pleura and bronchovascular bundles (perilymphatic pattern), however without enlarged bilateral hilar and mediastinal lymph nodes. The patient was treated with prednisolone and methotrexate, and is currently asymptomatic. Conclusion: breast involvement by sarcoidosis, although rare, should be considered when dealing with granulomatous lesions of the breast.

Introdução: a sarcoidose é uma doença granulomatosa sistêmica crônica de etiologia desconhecida. A mama pode ser envolvida em menos de 1% dos casos e, muitas vezes, imita os carcinomas ao exame clínico. Relato de caso: aqui, os autores relatam uma mulher de 54 anos que teve história de eritema nodoso, apresentou quadro clinico sugestivo de mastalgia, cuja mamografia e ultra-som apresentaram densidades parenquimatosas heterogêneas inespecíficas bilaterais. O estudo histopatológico revelou granulomas de células gigantes não-caseosos. Especificamente, os níveis séricos de enzima conversora de angiotensina (ECA) estavam elevados e uma TC apresentou pequenos nódulos pulmonares bilaterais distribuídos ao longo da pleura e feixes broncovasculares (padrão perilinfático), porém sem gânglios bilaterais hilar e mediastinal. A paciente foi tratado com prednisolona e metotrexato, e atualmente está assintomática. Conclusão: o envolvimento mamário por sarcoidose, embora raro, deve ser considerado ao lidar com lesões granulomatosas da mama.

El síndrome de Löfgren como presentación aguda de la sarcoidosis / Löfgren syndrome as an acute presentation of sarcoidoisis

RESUMEN La sarcoidosis es una enfermedad granulomatosa crónica, que se ha relacionado con la exposición a antígenos ambientales entre los que se incluye el contacto previo con agentes infecciosos y variedad de partículas orgánicas e inorgánicas. Existe una forma de presentación aguda de esta enfermedad que cursa con hallazgos clínicos estereotípicos denominada síndrome de Löfgren. Nosotros presentamos un caso de una paciente con todos los hallazgos característicos de este síndrome.

ABSTRACT Sarcoidosis is a chronic granulomatous disease that has been linked to exposure to certain environmental antigens, including previous contact with infectious agents, and a variety of other organic and inorganic particles. There is an acute form of presentation of this disease that courses with stereotypical clinical findings known as Löfgren's syndrome. A case is presented of a female patient with all the characteristic features of this syndrome.

Sarcoid-like cutaneous lesions in chronic adult paracoccidioidomycosis: report of two cases

ABSTRACT This study reports two cases of chronic paracoccidioidomycosis with sarcoid-like cutaneous lesions. The patients began the treatment in 2013 at Hospital Universitário Clementino Fraga Filho (HUCFF) of the Universidade Federal do Rio de Janeiro (UFRJ). The first case (mild form) was treated with trimethoprim-sulfamethoxazole (8 mg /kg per day, orally) for three months and, then, with half the dose for nine months; the second (moderate form), with itraconazole (200 mg per day, orally) for 12 months. We point out the rareness of the sarcoid-like cutaneous lesions and the differential diagnoses for other granulomatous diseases.

Development of exclusively cutaneous sarcoidosis in patient with rheumatoid arthritis during treatment with etanercept / Desenvolvimento de sarcoidose exclusivamente cutânea em paciente com artrite reumatoide, durante tratamento com etanercepte

Summary We report the case of a patient with rheumatoid arthritis who, after 2 months of treatment with etanercept, showed disseminated asymptomatic violaceous papules. Biopsy of the skin lesion showed chronic granulomatous dermatitis with negative staining for fungi and acid-fast bacilli (AFB). After discontinuation of etanercept, the patient's condition improved. Although apparently paradoxical, cases of cutaneous and systemic sarcoidosis after anti-TNF medications have been reported in the literature, with very few cases presenting exclusive cutaneous involvement.

Resumo Relata-se caso de uma paciente com artrite reumatoide que, após 2 meses de tratamento com o medicamento imunobiológico anti-TNF-α etanercepte, apresentou quadro cutâneo compatível com sarcoidose. Notavam-se pápulas violáceas disseminadas e assintomáticas, cuja histopatologia revelou dermatite crônica granulomatosa, com pesquisa de fungos e bacilos álcool-ácido resistentes negativa. Após suspensão do etanercepte, houve regressão do quadro cutâneo. Apesar de paradoxal, têm sido relatados na literatura casos de sarcoidose cutânea e sistêmica após uso de medicações anti-TNF, sendo raríssimos os casos com acometimento cutâneo exclusivo.

Sarcoidosis sistémica: un reto diagnóstico en pediatría. Reporte de un caso / Systemic sarcoidosis: a diagnostic challenge in pediatrics. Case report

La sarcoidosis es una enfermedad inflamatoria granulomatosa de etiología desconocida, evidenciada con mayor frecuencia en adultos jóvenes, que puede comprometer diversos órganos, principalmente, el pulmón, los ganglios linfáticos, los ojos y la piel. La presencia de dicha enfermedad enla población pediátrica es poco frecuente y su denominación en pediatría se realiza basada en la edad y las manifestaciones clínicas del paciente. El objetivo del siguiente reporte es presentar un caso de sarcoidosis sistémica en un paciente adolescente sin compromiso pulmonar, con manifestaciones extrapulmonares graves. Se trata de un paciente de 15 años, quien debutó con síntomas inespecíficos (emesis, pérdida de peso). Estudios radiológicos, sin compromiso pulmonar aparente; sin embargo, con presencia de hallazgos sugestivos de histiocitosis de células de Langerhans. Se realizó un estudio histopatológico de componente óseo y linfático, cuyo resultado evidenció la presencia de una enfermedad granulomatosa crónica no necrotizante tipo sarcoidosis. La sarcoidosis infantil es una entidad rara y difícil de diagnosticar, dada, principalmente, la ausencia de biomarcadores específicos para su diagnóstico.

Sarcoidosis is a granulomatous inflammatory disease of unknown etiology, evidenced most often in young adults, which can compromise several organs, mainly lung, lymph nodes, eyes and skin. The presence of the disease in pediatric population is rare. Denomination in pediatrics is made based on age and clinical manifestations of the patient. The aim of this report is to present a case of systemic sarcoidosis in a pediatric patient without lung involvement with severe extrapulmonary manifestations. Fifteen year old patient who debuted with nonspecific symptoms (emesis, weight loss). Radiographic studies without lung involvement but with findings suggestive of Langerhans cell histiocytosis; however, histopathological report (bone and lymph) showed the presence of chronic granulomatous nonnecrotizing disease, sarcoidosis type. Childhood sarcoidosis is a rare and difficult entity to diagnose, mainly by the lack of specific biomarkers for diagnosis.

Administering the Sarcoidosis Health Questionnaire to sarcoidosis patients in Serbia / Aplicação do Sarcoidosis Health Questionnaire em pacientes com sarcoidose na Sérvia

Objective: The aim of this study was to use a Serbian-language version of the disease-specific, self-report Sarcoidosis Health Questionnaire (SHQ), which was designed and originally validated in the United States, to assess health status in sarcoidosis patients in Serbia, as well as validating the instrument for use in the country. Methods: This was a cross-sectional study of 346 patients with biopsy-confirmed sarcoidosis. To evaluate the health status of the patients, we used the SHQ, which was translated into Serbian for the purposes of this study. We compared SHQ scores by patient gender and age, as well as by disease duration and treatment. Lower SHQ scores indicate poorer health status. Results: The SHQ scores demonstrated differences in health status among subgroups of the sarcoidosis patients evaluated. Health status was found to be significantly poorer among female patients and older patients, as well as among those with chronic sarcoidosis or extrapulmonary manifestations of the disease. Monotherapy with methotrexate was found to be associated with better health status than was monotherapy with prednisone or combination therapy with prednisone and methotrexate. Conclusions: The SHQ is a reliable, disease-specific, self-report instrument. Although originally designed for use in the United States, the SHQ could be a useful tool for the assessment of health status in various non-English-speaking populations of sarcoidosis patients.

Objetivo: O objetivo deste estudo foi utilizar uma versão no idioma sérvio do Sarcoidosis Health Questionnaire (SHQ), um questionário de autorrelato doença-específico, concebido e originalmente validado nos EUA, para verificar o estado de saúde de pacientes com sarcoidose na Sérvia, além de validar o instrumento para uso no país. Métodos: Estudo transversal com 346 pacientes com sarcoidose confirmada por biópsia. Para avaliar o estado de saúde dos pacientes, utilizamos o SHQ, o qual foi traduzido para o sérvio para os propósitos deste estudo. Comparamos os escores do SHQ por gênero, idade, duração da doença e tratamento. Escores do SHQ mais baixos indicam pior estado de saúde. Resultados: Os escores do SHQ demonstraram diferenças no estado de saúde entre os subgrupos de pacientes avaliados. O estado de saúde foi significativamente pior entre as mulheres e pacientes mais velhos, assim como entre aqueles com sarcoidose crônica ou com manifestações extrapulmonares da doença. A monoterapia com metotrexato associou-se com melhor estado de saúde do que a monoterapia com prednisona ou a terapia combinada com prednisona e metotrexato. Conclusões: O SHQ é um instrumento de autorrelato doença-específico confiável. Embora originalmente concebido para uso nos EUA, o SHQ pode ser uma ferramenta útil na avaliação do estado de saúde de populações de pacientes com sarcoidose em vários países de língua não inglesa.

Sarcoidosis and chronic hepatitis C: treatment with prednisone and colchicine

Abstract Sarcoidosis is a disease which still has uncertain etiology. Possible environmental causes are cited in the literature, like organic and inorganic particles and infectious agents. Recent studies have demonstrated the occurrence of sarcoidosis in patients with chronic C hepatitis; however, this association remains without statistical or causal evidence. In this report a case of sarcoidosis associated with chronic hepatitis C will be described, with subcutaneous lesions, considered rare, and good response to treatment with colchicine and prednisone. The hepatitis C virus was isolated in sarcoid tissue and the association between the two diseases will be discussed.

Sarcoidosis en un atleta de alto rendimiento con patrón recidivante en "árbol en gemación" / Sarcoidosis with a recurrent tree-in-budpattern in a high-performance athlete

La sarcoidosis es una enfermedad granulomatosa crónica sistémica de origen desconocido que afecta principalmente el pulmón, pero podría afectar cualquier órgano. Su diagnóstico es de exclusión haciendo necesaria una estricta correlación clínica, radiológica y patológica para su aproximación diagnóstica, adecuado tratamiento y seguimiento. Presentamos un caso de un paciente joven, atleta de alto rendimiento, con una manifestación radiológica infrecuente...

Sarcoidosis is a chronic systemic granulomatous disorder of unknown origin. It predominantly affects the lungs, but it can affect any organ. Sarcoidosis is a diagnosis of exclusion. A strict clinical, radiological and histopathological correlation is required for diagnosis and adequate treatment and follow-up. Herein we present the case of a young high-performance male athlete with an unusual radiological finding...

Eritema nodoso / Erythema nodosum

El eritema nodoso es la paniculitis septal más frecuente con nódulos de 1 a 5 cm, inflamatorios, simétricos, dolorosos, eritematosos, casi siempre en la superficie extensora distal de los miembros inferiores. Suelen remitir en forma espontánea, la evolución crónica es rara y se describen múltiples causas de esta patología. Se presentan dos casos: el primero, una mujer de 28 años con cuadro clínico de quince días por absceso en la mama izquierda que requirió drenaje quirúrgico y tratamiento antibiótico, con posterior apariciónde lesión en miembro inferior derecho asociada con dolor y fiebre. El segundo, hombre de 49 años de raza negra remitido por cuadro de veinte días con pérdida de peso, dolor torácico opresivo y aparición de nódulos en miembros inferiores, dolorosos a la palpación...

Erythema nodosum is the most common type of septal panniculitis, characterized by inflammatory, symmetric, tender, erythematous, 1 to 5 cm nodules typically located on the distal anterior aspect of the lower extremities. They often resolve spontaneously, chronic progression is rare and multiple causes have been described for this condition. We report two cases, the first in a 28 year-old woman with complaints for the past 15 days due to a left breast abscess which required surgical drainage and antibiotic treatment, followed by a lesion in the lower right limb associated with pain and fever. The second in a 48 year-old black man, referred for presenting, weight loss, oppressive chest pain and painful nodules in the lower limbs for the past 20 days...

Sarcoidosis aguda: variante de Síndrome de Lõfgren sin eritema nodoso / Acute sarcoidosis: erythema nodosum-free variant of Lõfgren's Syndrome

El síndrome de Lõfgren, es una variante aguda de la sarcoidosis, que se caracteriza por fiebre, eritema nodoso, adenomegalias hiliares pulmonares y artritis. En general, tiene un curso benigno y autolimitado, que contrasta con las formas crónicas que requieren uso de corticoides y tienen tendencia a la recidiva. Se describe aquí el caso clínico de un paciente joven, de sexo masculino, con artritis pero sin eritema nodoso, lo que dificultó el planteo diagnóstico de síndrome de Lõfgren. Se realiza además una breve descripción comparativa entre la presentación clínica de la sarcoidosis crónica y el síndrome de Lõfgren...

Musculoskeletal and cutaneous sarcoidosis: exuberant case report

Sarcoidosis is a multisystem granulomatous disease of unknown cause. The osteoarticular involvement in sarcoidosis is rare and is often associated with cutaneous and long-standing chronic multisystem disease. More common in black women, osseous sarcoidosis is difficult to diagnose, with an incidence of 3 to 13%. The most characteristic radiological clinical picture evidences rounded, well-defined cysts, with no periosteal reaction and without peripheral sclerosis. The small bones of hands and feet are the most frequently involved sites. This report aims to demonstrate a rare case of osteoarticular sarcoidosis with characteristic clinical presentation, and highlight the importance of detecting osteoarticular involvement in this pathology.

Musculoskeletal involvement in sarcoidosis / Acometimento músculo-esquelético na sarcoidose

Sarcoidosis is a multisystem inflammatory disorder of unknown cause. It most commonly affects the pulmonary system but can also affect the musculoskeletal system, albeit less frequently. In patients with sarcoidosis, rheumatic involvement is polymorphic. It can be the presenting symptom of the disease or can appear during its progression. Articular involvement is dominated by nonspecific arthralgia, polyarthritis, and Löfgren's syndrome, which is defined as the presence of lung adenopathy, arthralgia (or arthritis), and erythema nodosum. Skeletal manifestations, especially dactylitis, appear mainly as complications of chronic, multiorgan sarcoidosis. Muscle involvement in sarcoidosis is rare and usually asymptomatic. The diagnosis of rheumatic sarcoidosis is based on X-ray findings and magnetic resonance imaging findings, although the definitive diagnosis is made by anatomopathological study of biopsy samples. Musculoskeletal involvement in sarcoidosis is generally relieved with nonsteroidal anti-inflammatory drugs or corticosteroids. In corticosteroid-resistant or -dependent forms of the disease, immunosuppressive therapy, such as treatment with methotrexate or anti-TNF-α, is employed. The aim of this review was to present an overview of the various types of osteoarticular and muscle involvement in sarcoidosis, focusing on their diagnosis and management.

A sarcoidose é um distúrbio inflamatório multissistêmico de causa desconhecida, frequentemente afetando o sistema pulmonar e também o sistema músculo-esquelético, mas de forma menos frequente. Em pacientes com sarcoidose, o acometimento reumático é polimórfico, podendo ser o sintoma de apresentação da doença ou aparecer durante sua progressão. O acometimento articular é dominado por artralgia inespecífica, poliartrite e síndrome de Löfgren, que é definida como a presença de adenopatia pulmonar, artralgia (ou artrite) e eritema nodoso. Manifestações esqueléticas, especialmente dactilite, aparecem principalmente como complicações de sarcoidose crônica e em vários órgãos. O acometimento muscular na sarcoidose é raro e geralmente assintomático. O diagnóstico de sarcoidose reumática baseia-se em achados radiográficos e de ressonância magnética, embora o diagnóstico definitivo seja feito pelo estudo anatomopatológico de amostras de biópsia. O acometimento músculo-esquelético na sarcoidose é geralmente aliviado com o uso de anti-inflamatórios não esteroidais ou corticosteroides. Em formas da doença resistentes ao corticosteroide ou corticosteroide dependentes, a terapia de imunossupressão, como o tratamento com metotrexato ou anti-TNF-α, é utilizada. O objetivo desta revisão foi apresentar uma visão geral dos vários tipos de acometimento osteoarticular e muscular na sarcoidose, com foco no diagnóstico e manejo.

Delayed diagnosis of sarcoidosis is common in Brazil / Diagnóstico tardio da sarcoidose é comum no Brasil

OBJECTIVE: To determine the frequency of and the factors related to delayed diagnosis of sarcoidosis in Brazil. METHODS: We evaluated patients with a biopsy-proven diagnosis of sarcoidosis, using a questionnaire that addressed the following: time since symptom onset and since the first medical visit; and the number and specialty of the physicians visited. We divided the patients by the timeliness of the diagnosis-timely (< 6 months) and delayed (≥ 6 months)-comparing the two groups in terms of systemic and pulmonary symptoms; extrathoracic involvement; spirometric data; radiological staging; level of education; income; and tuberculosis (diagnosis and treatment). RESULTS: We evaluated 100 patients. The median number of physicians consulted was 3 (range, 1-14). In 11 cases, sarcoidosis was diagnosed at the first visit. In 54, the first physician seen was a general practitioner. The diagnosis of sarcoidosis was timely in 41 patients and delayed in 59. The groups did not differ in terms of gender; race; type of health insurance; level of education; income; respiratory/systemic symptoms; extrathoracic involvement; and radiological staging. In the delayed diagnosis group, FVC was lower (80.3 ± 20.4% vs. 90.5 ± 17.1%; p = 0.010), as was FEV1 (77.3 ± 19.9% vs. 86.4 ± 19.5%; p = 0.024), misdiagnosis with and treatment for tuberculosis (≥ 3 months) also being more common (24% vs. 7%, p = 0.032, and 20% vs. 0%; p = 0.002, respectively). CONCLUSIONS: The diagnosis of sarcoidosis is often delayed, even when the imaging is suggestive of sarcoidosis. Delayed diagnosis is associated with impaired lung function at the time of diagnosis. Many sarcoidosis patients are misdiagnosed with and treated for tuberculosis. .

OBJETIVO: Avaliar a frequência do diagnóstico tardio de sarcoidose no Brasil e os fatores relacionados a esse atraso. MÉTODOS: Avaliamos pacientes com diagnóstico de sarcoidose confirmado por biópsia utilizando um questionário que abordava o tempo entre o início dos sintomas e a data da primeira consulta médica; e o número e especialidades dos médicos consultados. Sintomas sistêmicos e pulmonares, envolvimento extratorácico, dados espirométricos, estadiamento radiológico, escolaridade, renda individual e diagnóstico/tratamento de tuberculose foram comparados entre os pacientes com diagnóstico precoce (< 6 meses até o diagnóstico) e tardio (≥ 6 meses). RESULTADOS: Foram incluídos 100 pacientes. A mediana do número de médicos consultados foi 3 (variação: 1-14). O diagnóstico de sarcoidose foi feito na primeira consulta em 11 casos. Um clínico geral foi inicialmente consultado em 54 casos. O diagnóstico de sarcoidose foi precoce em 41 casos e tardio em 59. Não houve diferença entre os grupos no tocante ao gênero, raça, tipo de seguro saúde, escolaridade, renda, sintomas sistêmicos e respiratórios, envolvimento extratorácico e estadiamento radiológico. Os pacientes com diagnóstico tardio apresentavam menor CVF (80,3 ± 20,4% vs. 90,5 ± 17,1%; p = 0,010) e VEF1 (77,3 ± 19,9% vs. 86,4 ± 19,5%; p = 0,024), além de mais frequentemente receberem diagnóstico de tuberculose (24% vs. 7%; p = 0,032) e tratamento para tuberculose (≥ 3 meses; 20% vs. 0%; p = 0,002). CONCLUSÕES: O diagnóstico de sarcoidose é tardio em muitos casos, mesmo quando há achados de imagem sugestivos. O diagnóstico tardio está associado a menor função pulmonar na época do diagnóstico. Vários ...