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Resumen: Las enfermedades pulmonares intersticiales (EPI), son un grupo heterogéneo de trastornos, caracterizados por inflamación y/o fibrosis, que afectan el intersticio pulmonar. Históricamente, se basaban en características histopatológicas de biopsias pulmonares, pero con el avance de la tomografía computarizada de alta resolución (TCAR), el enfoque ha cambiado, permitiendo identificar patrones de enfermedad que sugieren distintas etiologías. La clasificación de las EPI ha evolucionado, y actualmente se clasifican según etiología, patrones histopatológicos y/o radiológicos y curso evolutivo. Las neumonías intersticiales idiopáticas (NII) son un grupo de EPI de origen desconocido, y la fibrosis pulmonar idiopática (FPI) es la más común dentro de las NII. Se ha desarrollado el concepto de enfermedad pulmonar intersticial fibrosante progresiva (EPI-FP), que describe un fenotipo de EPI con progresión a pesar del tratamiento. El diagnóstico se basa en una historia clínica detallada, examen físico, pruebas de función pulmonar, serología autoinmune y técnicas de imagen como la TCAR. El tratamiento varía según la etiología y puede incluir terapia inmunomoduladora o antiinflamatoria, terapia modificadora de la enfermedad con agentes antifibróticos y, en casos avanzados, trasplante pulmonar. La atención médica debe ser holística, incluyendo rehabilitación pulmonar, prevención de infecciones y oxigenoterapia.El objetivo de este artículo es realizar una actualización clínica de las enfermedades pulmonares intersticiales y comentar los avances en las estrategias diagnósticas y terapéuticas. Abstract: Interstitial lung diseases (ILDs) are a heterogeneous group of disorders that affect the lung interstitium, characterized by inflammation and/or fibrosis. Historically, they were based on histopathological characteristics of lung biopsies, but with the advancement of high-resolution computed tomography (HRCT), the approach has shifted, allowing for the identification of disease patterns that suggest different etiologies. The classification of ILDs has evolved, and they are currently classified according to etiology, histopathological and/or radiological patterns, and evolutionary course. Idiopathic interstitial pneumonias (IIPs) are a group of ILDs of unknown origin, with idiopathic pulmonary fibrosis (IPF) being the most common within the IIPs. The concept of progressive fibrosing interstitial lung disease (PF-ILD) has been developed, describing a phenotype of ILD with progression despite treatment. Diagnosis is based on a detailed clinical history, physical examination, pulmonary function tests, autoimmune serology, and imaging techniques such as HRCT. Treatment varies according to etiology and may include immunomodulatory or anti-inflammatory therapy, disease-modifying therapy with antifibrotic agents and, in advanced cases, lung transplantation. Medical care should be holistic, including pulmonary rehabilitation, infection prevention, and oxygen therapy. This article aims to provide a clinical update on interstitial lung diseases and comment on advances in diagnostic and therapeutic strategies.

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In 1992, the concept of antisynthetase syndrome was proposed for the first time, an autoimmune disease included on the inflammatory myopathies. It is characterized by interstitial lung disease, non-erosive arthritis, myositis, Raynaud syndrome, fever of unknown origin and mechanic's hands. There are 10 known antisynthetase antibodies, anti-Jo-1 is the most prevalent. Since 1975, criteria to classify the inflammatory myopathies were established, but in 2010 the criteria for the diagnosis of antisynthetase syndrome were published by Connors et al. At present, specific antibodies, clinical findings and muscle disease criteria need to be met for the diagnosis of antisynthetase syndrome, considered a different disease than dermatomyositis and polymyositis. It has been reported that clinical findings will differ among patients according to the type of antibody present or positive. There are no international consensus nor randomized trials for the management of patients with antisynthetase syndrome, but there are case reports of positive outcomes in patients treated with different immunomodulatory drugs. [ABSTRACT FROM AUTHOR]

ILD patients can be positive to highly specific autoantibodies of connective tissue diseases (CTD). Among them stand out myositis-specific and associated autoantibodies (MSA/MAA). There is limited knowledge about treatment response and prognosis of ILD patients positive to MSA/MAA (MSA/MAA-ILD). Our aim was to describe clinical, radiological and pulmonary function (PF) of MSA/MAA-ILD Latin-American patients and risk factors associated to PF at onset and long term follow up. Multicentric retrospective study of MSA/MAA-ILD patients evaluated between 2016 and 2018 in 3 ILD clinics in Latin America. Clinical, functional and tomographic variables were described. Variables associated with poor baseline PF and associated with functional improvement (FI) were analyzed in a multivariate logistic regression model. We included 211 patients, 77.4% female, mean age 57 years old. Most frequent MSA/MAA were Ro-52 and Jo-1. Poor baseline PF was associated to ILD as initial diagnosis and NSIP/OP HRCT pattern. 121 patients were included in the follow up PF analysis: 48.8% remained stable and 33% had a significant FI. In multivariate analysis, OP pattern on HRCT was associated with FI. Systemic symptoms from the beginning and the absence of sclerodactyly showed a trend to be associated with FI. Worse baseline PF could be related to the absence of extra-thoracic symptoms and "classic" antibodies in CTD (ANA), which causes delay in diagnosis and treatment. In contrast, FI could be related to the presence of extra-thoracic signs that allow timely diagnosis and therapy, and more acute and subacute forms of ILD, such as OP pattern. [ABSTRACT FROM AUTHOR]

El síndrome antisintetasa es una miopatía inflamatoria autoinmune poco frecuente que se caracteriza por la presencia de autoanticuerpos anti sintetasa ARNt (generalmente anti JO1) asociado frecuentemente a miositis, enfermedad pulmonar intersticial, poliartritis, manos de mecánico y fenómeno de Raynaud. Se reporta el caso de una mujer de 45 años que presenta este síndrome con características fenotípicas de dermatomiositis y responde de forma favorable luego de la administración de tratamiento con glucocorticoides asociado a metotrexato.

Introducción: Las miopatías inflamatorias idiopáticas (MII) conforman un grupo de patologías crónicas autoinmunes, con manifestaciones clínicas heterogéneas pero con la particularidad de afectar preferentemente al músculo. Dentro de este grupo, tenemos al síndrome antisintetasa (SAS), patología asociada a la presencia de anticuerpos antisintetasa (ACAS) afectación de las articulaciones, los pulmones y de forma particular la piel de las manos. Objetivos: Determinar la prevalencia del SAS en el Servicio de Reumatología del Hospital de Clínicas, durante el período de enero a noviembre del 2014 y describir las principales características clínicas de presentación del SAS. Materiales y Métodos: Es un estudio descriptivo y retrospectivo que incluyó la revisión de historias clínicas de los pacientes con MII que consultaron en el Departamento de Reumatología del Hospital de Clínicas, donde se incluyó los pacientes que cumplían con los criterios para alguna MII poniendo énfasis el diagnóstico de SAS. Resultados: En el año 2014 se registraron 5.418 consultas, de las cuales 28 pacientes tenían un diagnóstico de miopatía inflamatoria idiopática, correspondiendo 22 pacientes (70%) al sexo femenino y 6 (21%) al sexo masculino. Del total de las miopatías ,14 casos (50%) presentaba un síndrome de superposición. Del total de casos de síndromes de superposición, y aplicando los criterios para SAS, pudimos constatar que 4/14 casos (29%) cumplían criterios de clasificación para SAS. Conclusión: La prevalencia puntual de casos diagnosticados de SAS en el Servicio de Reumatología fue de 0,07%, con presentación más frecuente en mujeres, con una edad de 35 años en promedio, con una clínica predominante de miositis, artritis y afectación pulmonar, sea en fase crónica o aguda.

A 52-year-old male patient developed RA in March 2009 at the age of 43, with symmetric polyarthritis and active synovitis affecting hands, knees, ankles and both feet without symptoms or signs suggestive of extra-articular features. Laboratory investigations showed negative RF and positive anti-CCP antibodies, negative ANA, negative anti-dsDNA antibodies; the X-rays of both hands showed typical erosive changes in RA and fulfilled the new ACR/EULAR (2010) criteria of RA. The patient achieved remission on a combination of DMARDs. He did well until January 2017 when he developed acute onset of progressive chest pain, dyspnea, and acute respiratory failure. High-resolution CT of the lung showed extensive areas of ground glass veiling, and interstitial subpleural infiltrates were found consistent with aggressive interstitial lung disease (ILD). Autoantibodies against extractable nuclear antigens were screened and showed positive results for anti-RO and anti-Jo1 autoantibodies. The positive anti-Jo1was an expression of anti-synthetase syndrome complicating the RA course and explained the rapidly aggressive course of ILD. [ABSTRACT FROM AUTHOR]

O síndrome anti-sintetase caracteriza-se clinicamente por polimiosite ou dermatomiosite, podendo associar-se a artrite, fenómeno de Raynaud e doença pulmonar intersticial. Laboratorialmente é marcada pela presença de anticorpos anti-sintetase, sendo mais frequente o anti-Jo-1. Apresentamos o caso de um homem com diagnóstico efectuado há 13 anos, no contexto de internamento por uma “pneumonia”, com queixas de dispneia para esforços e cansaço fácil com 3 meses de evolução. Durante o internamento é identificada uma doença pulmonar intersticial e, posteriormente surge um quadro de mialgias com elevação marcada das enzimas musculares. A electromiografia mostrou um padrão sugestivo de miopatia e identificou-se anticorpos anti-Jo-1, estabelecendo-se o diagnóstico de síndrome anti-sintetase. Sob terapêutica imunossupressora desde então, mantém seguimento em consulta externa, encontrando-se clinicamente bem.

We report the case of a female adult with a history of interstitial pneumonia that debutes with systemic symptoms, skin rash, "mechanics hands", muscle weakness, and polyarthralgia. Through laboratory studies, histopathology of the skin, high resolution computed tomography and electromyogram, we arrived at the antisynthetase syndrome diagnosis. The antisynthetase syndrome is a rare autoimmune inflammatory of the myopathies group with high mortality and poor response to therapy, whose evolution depends on the lung involvement. [ABSTRACT FROM AUTHOR]

Introduction: The antisynthetase syndrome has been described in adults with an average age of 45 to 60 years, but it can affect children, predominantly females. Its features include myositis, polymyositis, interstitial lung disease and the presence of aminoacyl tRNA antibodies of which the Anti Jo-1 is the most common. Case Report: A 6 year old girl had a two year history of fever, arthritis syndrome, progressive muscle weakness, Raynaud's phenomenon, myalgia, malar heliotrope rash, Gottron's papules and "mechanic hands", tachypnea, finger clubbing. The patient was oxygen-dependent. X-ray and computerized axial tomography showed images suggestive of lung fibrosis. She had elevated creatine: 4530 IU/mL, and positive Jo-1 antibody. There was a myopathic electromyography pattern. A muscle biopsy was compatible with polymyositis. Juvenile dermatomyositis with anti-synthetase syndrome was diagnosed. She was treated with methylprednisolone, prednisone, intravenous human immunoglobulin and cyclophosphamide. During the first year of treatment her muscle strength was recovered, fever and skin lesions disappeared, respiratory function became normal and there was no need for oxygen. Discussion. The antisynthetase syndrome includes polymyositis and dermatomyositis, interstitial lung disease, polyarthritis, Raynaud's phenomenon, "mechanic hands" and the presence of serum anti-synthetase antibodies. Treatment should be comprehensive and holistic, in order to restore lung capacity, prevent progression of damage and improve life quality. [ABSTRACT FROM AUTHOR]

Copyright of Revista Española de Medicina Nuclear e Imagen Molecular is the property of Elsevier B.V. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Kaposi's sarcoma (KS) is a malignant vascular tumor widely known as a complication of acquired immunodeficiency syndrome (AIDS) but also related to immunosuppression in renal transplants, and less frequently, to other diseases. We describe a case of KS in a patient affected by antisynthetase syndrome treated with steroids. [ABSTRACT FROM AUTHOR]

Background and Objectives: ILD patients can be positive to highly specific autoantibodies of connective tissue diseases (CTD). Among them stand out myositis-specific and associated autoantibodies (MSA/MAA). There is limited knowledge about treatment response and prognosis of ILD patients positive to MSA/MAA (MSA/MAA-ILD). Our aim was to describe clinical, radiological and pulmonary function (PF) of MSA/MAA-ILD Latin-American patients and risk factors associated to PF at onset and long term follow up., Methods: Multicentric retrospective study of MSA/MAA-ILD patients evaluated between 2016 and 2018 in 3 ILD clinics in Latin America. Clinical, functional and tomographic variables were described. Variables associated with poor baseline PF and associated with functional improvement (FI) were analyzed in a multivariate logistic regression model., Results: We included 211 patients, 77.4% female, mean age 57 years old. Most frequent MSA/MAA were Ro-52 and Jo-1. Poor baseline PF was associated to ILD as initial diagnosis and NSIP/OP HRCT pattern. 121 patients were included in the follow up PF analysis: 48.8% remained stable and 33% had a significant FI. In multivariate analysis, OP pattern on HRCT was associated with FI. Systemic symptoms from the beginning and the absence of sclerodactyly showed a trend to be associated with FI., Conclusions: Worse baseline PF could be related to the absence of extra-thoracic symptoms and "classic" antibodies in CTD (ANA), which causes delay in diagnosis and treatment. In contrast, FI could be related to the presence of extra-thoracic signs that allow timely diagnosis and therapy, and more acute and subacute forms of ILD, such as OP pattern., (Copyright © 2021 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.)