RESUMEN
Sarcoidosis is a systemic inflammatory disease of unknown origin, which consists of the formation of multiple sterile noncaseating granulomas. Inhaled antigens are believed to initiate disease in prone individuals, considering that almost all patients present pulmonary or mediastinal lymph node disease. Extrapulmonary manifestations are common and diverse: practically any organ system can be affected, and treatment can range from simple watchful waiting to intense immunosuppression. In this article, we review current concepts about sarcoidosis in an overview, focusing on recognition and treatment of its major clinical phenotypes.
Asunto(s)
Sarcoidosis , Humanos , Sarcoidosis/diagnóstico , Sarcoidosis PulmonarRESUMEN
En el último tiempo, la inmunoterapia se ha convertido en una opción terapéutica para diversos tipos de neoplasias, aumentando la sobrevida en muchos casos, pero también los efectos adversos asociados. Existen tres tipos de inmunoterapia utilizados en cáncer: Terapia de células T con receptor de antígeno quimérico (CAR-T), destacando como reacciones adversas el síndrome liberador de citoquinas (CRS) y el síndrome de neurotoxicidad (ICANS); Anticuerpos monoclonales (AcM), cuyos efectos adversos más comunes están relacionados con reacciones de hipersensibilidad; y los Inhibidores de puntos de control inmunitario (ICI) con toxicidad pulmonar claramente reportada. Para un correcto manejo de estas reacciones adversas se requiere un alto índice de sospecha, un adecuado diagnóstico diferencial y un tratamiento oportuno, basado principalmente en corticoides y guiado por criterios de gravedad. Se presenta el caso de un paciente con reacción granulomatosa sarcoidea posterior al uso de Nivolumab.
In recent times, immunotherapy has emerged as a therapeutic option for various neoplasms, significantly improving survival rates in many cases, albeit with associated adverse effects. There are three types of immunotherapy commonly used in cancer treatment: Chimeric Antigen Receptor T-cell Therapy (CAR-T), notable for adverse reactions such as Cytokine Release Syndrome (CRS) and Immune Effector Cell-Associated Neurotoxicity Syndrome (ICANS); Monoclonal Antibodies (mAbs), with the most common adverse effects being hypersensitivity reactions; and Immune Checkpoint Inhibitors (ICI), with well-documented pulmonary toxicity. Adequate management of these adverse reactions requires a high index of suspicion, accurate differential diagnosis, and timely treatment, primarily based on corticosteroids and guided by severity criteria. We present a case of a patient with granulomatous sarcoid-like reaction following the use of Nivolumab.
Asunto(s)
Humanos , Masculino , Persona de Mediana Edad , Nivolumab/efectos adversos , Inhibidores de Puntos de Control Inmunológico/efectos adversos , Enfermedades Pulmonares/inducido químicamente , Neoplasias/tratamiento farmacológico , Sarcoidosis Pulmonar/inducido químicamente , Inmunoterapia/efectos adversosRESUMEN
BACKGROUND: Erythema Nodosum (EN) is the most common skin manifestation in sarcoidosis and has often been associated with a good prognosis. OBJECTIVES: To compare the clinical characteristics and treatment-related features in patients with sarcoidosis according to whether or not EN was seen as a presenting symptom at the time of diagnosis. METHODS: A 20-year single-center retrospective study was performed. The following two groups were identified: one group with EN as one of the presenting symptoms at the time of diagnosis of sarcoidosis (EN group) and a second group without EN as a presenting symptom at diagnosis (non-EN group). The clinical characteristics and treatment modalities were collected from the medical records. RESULTS: A total of 122 patients (31 in the EN group, 91 in the non-EN group) were included. Radiological stages of pulmonary disease were significantly lower in the EN group. Articular involvement was more common in the EN group (p = 0.001), whereas other systemic organ involvements (p = 0.025), especially neurological involvement (p = 0.036), were significantly more common in the non-EN group. In the EN group, a higher percentage of patients were managed without systemic therapy (71.0% vs. 54.9%) and spontaneous remission was more frequent (25.0% vs. 14.1%), however, this wasn't statistically significant. STUDY LIMITATIONS: Retrospective design. CONCLUSIONS: The lower radiological stage of pulmonary sarcoidosis and lower frequency of systemic organ involvement in patients with EN augment the prognostic value of EN highlighted in the literature. However, this study couldn't confirm that the patients with EN would need less systemic therapy in the course of their disease.
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Eritema Nudoso , Sarcoidosis Pulmonar , Sarcoidosis , Humanos , Pronóstico , Estudios RetrospectivosRESUMEN
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.
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Enfermedades Pulmonares , Sarcoidosis Pulmonar , Sarcoidosis , Brasil/epidemiología , Humanos , Enfermedades Pulmonares/complicaciones , Persona de Mediana Edad , Pronóstico , Estudios Retrospectivos , Sarcoidosis Pulmonar/diagnósticoAsunto(s)
Humanos , Femenino , Anciano de 80 o más Años , Sarcoidosis Pulmonar/diagnóstico , Sarcoidosis Pulmonar/fisiopatología , Pulmón/diagnóstico por imagen , Signos y Síntomas , Tórax/diagnóstico por imagen , Radiografía Torácica , Tomografía Computarizada por Rayos X/métodos , Sarcoidosis Pulmonar/clasificaciónRESUMEN
Necrotizing sarcoid granulomatosis (NSG) is a rare and under-recognized cause of granulomatous disease, described as a variant of typical nodular sarcoidosis. It can be asymptomatic when the patient has a single pulmonary nodule or may be accompanied by cough, fever, and dyspnea, or even symptoms due to the involvement of other organs such as the eyes, liver, and central nervous system. The histopathological analysis is essential for the differential diagnosis of other infectious and non-infectious causes of granuloma and to determine the appropriate treatment. NSG is characterized by the presence of a granuloma with extensive coagulative necrosis associated with the occurrence of vasculitis. We present the case of a patient diagnosed with NSG who had an unusual outcome with recurrent pulmonary thromboembolisms followed by hemodynamic instability and death.
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Humanos , Femenino , Adulto , Sarcoidosis Pulmonar/patología , Granuloma , Granulomatosis con Poliangitis/patología , Diagnóstico DiferencialRESUMEN
BACKGROUND: Diagnosis of extra-pulmonary sarcoidosis can be difficult, and a biopsy is usually required. We evaluated the utility of endobronchial ultrasound-transbronchial needle aspiration (EBUS-TBNA) in patients with suspected extra-pulmonary sarcoidosis with thoracic lymph nodes ≤10 mm on chest computed tomography (CT) and no or minimal pulmonary infiltrates. METHODS: The Cleveland Clinic bronchoscopy registry was screened. Patients with thoracic lymph nodes >10 mm on short axis or significant pulmonary infiltrates in the chest CT scan were excluded. Two separate analyses using expert consensus (before and after release of bronchoscopy results) were the reference standard. RESULTS: 15 patients met the inclusion criteria. 40% had suspected ocular, 33% cardiac and 27% neurologic sarcoidosis. Six patients (40%) had EBUS-TBNA compatible with sarcoidosis. When the reference standard was the consensus diagnosis blinded to bronchoscopy results, the sensitivity, specificity, positive predictive value and negative predictive value of EBUS-TBNA were 56%, 83%, 83%, and 56% respectively. The combination of a positive EBUS-TBNA and BAL CD4/CD8 improved the specificity from 83 to 100%, but the difference was not statistically significant (p = 0.074). When the reference standard was the consensus diagnosis with the bronchoscopic results, the sensitivity, specificity, positive predictive value and negative predictive value of EBUS-TBNA were 75%, 100%, 100%, and 78% respectively. CONCLUSIONS: In patients with suspected extra-pulmonary sarcoidosis, the EBUS-TBNA may be useful in the diagnosis of patients with thoracic lymph nodes ≤10 mm and no or minimal pulmonary infiltrates on chest CT. Larger and prospective studies are needed to validate our findings.
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Broncoscopía/métodos , Biopsia por Aspiración con Aguja Fina Guiada por Ultrasonido Endoscópico/métodos , Ganglios Linfáticos/patología , Sarcoidosis Pulmonar/patología , Adulto , Anciano , Femenino , Humanos , Ganglios Linfáticos/diagnóstico por imagen , Linfadenopatía , Masculino , Persona de Mediana Edad , Sarcoidosis Pulmonar/diagnóstico por imagen , Sensibilidad y Especificidad , Tórax , Tomografía Computarizada por Rayos XAsunto(s)
Etanercept , Infliximab , Pulmón , Nódulos Pulmonares Múltiples , Sarcoidosis Pulmonar , Espondilitis Anquilosante/tratamiento farmacológico , Adulto , Biopsia/métodos , Líquido del Lavado Bronquioalveolar/citología , Líquido del Lavado Bronquioalveolar/microbiología , Diagnóstico Diferencial , Sustitución de Medicamentos/métodos , Etanercept/administración & dosificación , Etanercept/efectos adversos , Femenino , Humanos , Infliximab/administración & dosificación , Infliximab/efectos adversos , Pulmón/diagnóstico por imagen , Pulmón/patología , Nódulos Pulmonares Múltiples/diagnóstico por imagen , Nódulos Pulmonares Múltiples/etiología , Remisión Espontánea , Sarcoidosis Pulmonar/diagnóstico , Sarcoidosis Pulmonar/tratamiento farmacológico , Sarcoidosis Pulmonar/etiología , Sarcoidosis Pulmonar/fisiopatología , Tomografía Computarizada por Rayos X/métodos , Resultado del Tratamiento , Inhibidores del Factor de Necrosis Tumoral/administración & dosificación , Inhibidores del Factor de Necrosis Tumoral/efectos adversos , Privación de TratamientoAsunto(s)
Eritema Nudoso , Sarcoidosis Pulmonar , Sarcoidosis , Eritema Nudoso/diagnóstico , HumanosRESUMEN
La sarcoidosis es una enfermedad croÌnica y multisisteÌmica de etiologiÌa poco clara. La presentacioÌn es variable, de acuerdo con la procedencia geograÌfica del paciente, pero predomina en personas afrodescendientes y escandinavas. Las anormalidades toraÌcicas son muy frecuentes en los pacientes con sarcoidosis; la afectacioÌn maÌs comuÌn es ganglionar y la menos comuÌn es la del corazoÌn. Las manifestaciones radioloÌgicas maÌs frecuentes en el compromiso toraÌcico por sarcoidosis son las adenomegalias hiliares y mediastinales, asiÌ como noÌdulos pulmonares de distribucioÌn perilinfaÌtica.
Sarcoidosis is a chronic, multisystemic disease of unclear etiology. The presentation is variable according to the geographical origin of the patient, predominantly in Afro-descendant and Scandinavian patients. Chest abnormalities are very frequent in patients with sarcoidosis, taking into account that the most common involvement is lymphatic and the least common is heart involvement. The most frequent radiological manifestations in thoracic involvement due to sarcoidosis are hilar and mediastinal adenomegalies as well as pulmonary nodules with perilymphatic distribution.
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Humanos , Tomografía Computarizada Multidetector , Sarcoidosis , Radiografía Torácica , Sarcoidosis PulmonarAsunto(s)
Livedo Reticularis/patología , Sarcoidosis/patología , Enfermedades de la Piel/patología , Eritema/patología , Femenino , Granuloma/patología , Humanos , Persona de Mediana Edad , Sarcoidosis Pulmonar/diagnóstico por imagen , Sarcoidosis Pulmonar/patología , Tomografía Computarizada por Rayos X , Vasculitis/patologíaAsunto(s)
Humanos , Femenino , Persona de Mediana Edad , Sarcoidosis/patología , Enfermedades de la Piel/patología , Livedo Reticularis/patología , Vasculitis/patología , Tomografía Computarizada por Rayos X , Sarcoidosis Pulmonar/patología , Sarcoidosis Pulmonar/diagnóstico por imagen , Eritema/patología , Granuloma/patologíaRESUMEN
La sarcoidosis es una enfermedad granulomatosa no caseificante, multisistémica, de causa desconocida, que compromete al pulmón y a los ganglios linfáticos mediastinales entre el 90 y el 95% de los casos. También puede afectar otros órganos, como las glándulas salivales, piel, ojos, hígado, bazo, corazón, huesos y sistema nervioso central. La sarcoidosis tiene una baja prevalencia en Latinoamérica y es subdiagnosticada debido a la alta frecuencia de otros trastornos similares, como tuberculosis, lepra y micosis profundas. El diagnóstico presuntivo se establece con hallazgos imagenológicos característicos dentro de un contexto clínico apropiado y se confirma con la evidencia histológica de granulomas no caseificantes de células epiteliales, en ausencia de otras etiologías. Los hallazgos torácicos incluyen la afectación pulmonar, ganglionar y bronquial, los cuales son detectados a través de la radiografía (Rx) y tomografía computada (TC) de tórax, siendo esa última más sensible y específica. En este artículo, resaltamos la importancia de reconocer los patrones de presentación típicos y atípicos de la sarcoidosis en Rx y TC, así como la relevancia de las imágenes torácicas como elemento clave en el algoritmo diagnóstico de esa patología. También describimos la utilidad de la resonancia magnética (RM), como método adicional para el diagnóstico en casos de afectación cardíaca y el papel de la tomografía por emisión de positrones (PET-CT) en el seguimiento terapéutico.
Sarcoidosis is a non-caseating granulomatous, multisystemic disease of unknown cause that involves the lung and mediastinal lymph nodes in 90-95% of cases. It can also affect other organs such as the salivary glands, skin, eyes, liver, spleen, heart, bones and the central nervous system. Sarcoidosis has a low prevalence in Latin America and it is underdiagnosed due to the high frequency of other similar disorders such as tuberculosis, leprosy and deep mycosis. The presumptive diagnosis is established based on characteristic imaging findings within an appropriate clinical setting and is confirmed by histological evidence of non-caseating epithelioid cell granulomas, in the absence of other etiologies. Thoracic imaging findings include pulmonary, nodal and bronchial involvement, which are detected on chest radiography (CXR) and computed tomography (CT), this last one having a higher sensitivity and specificity. In this article, we highlight the importance of recognizing the typical and atypical presentation patterns of sarcoidosis on CXR and CT, as well as the relevance of thoracic images as key elements in the diagnostic algorithm of this pathology. We also describe the usefulness of magnetic resonance (MR) imaging as an additional method for diagnosis in cases of cardiac involvement and the role of positron emission tomography (PET-CT) in therapeutic follow-up.
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Humanos , Sarcoidosis , Sarcoidosis/diagnóstico por imagen , Espectroscopía de Resonancia Magnética/métodos , Radiografía/métodos , Tomografía Computarizada por Rayos X/métodos , Sarcoidosis Pulmonar/diagnóstico por imagen , Tomografía de Emisión de Positrones/métodos , Sarcoidosis/diagnóstico , Radiografía TorácicaRESUMEN
Introducción: La tuberculosis (TBC) y la sarcoidosis presentan similitudes en cuanto a su manifestación clínica, radiología e histología. El término tuberculosis-sarcoidosis, hace referencia a una entidad con diferentes manifestaciones, en donde la TBC puede preceder, seguir o coexistir con el curso de la sarcoidosis. Presentación del caso: Mujer de 47 años, con antecedentes de miomectomía y colecistectomía, con historia de cuatro meses de dolor abdominal tipo cólico, de localización difusa asociado a hiporexia y baja de peso de 15 kilogramos. Paciente consultó por empeoramiento de cuadro inicial, acompañado de cefalea frontal intensa, quedando hospitalizada para estudio por especialistas. Evolucionó el primer día con paresia facial periférica derecha, compromiso del trigémino izquierdo V1-V2, hipoestesia abdominal izquierda T10 a L1, síntomas deglutorios del nervio gloso-cutáneo lateral izquierdo. Se decidió continuar estudio con punción lumbar, informándose: proteínas: 0,81 mg/dl, glucosa: 62 mg/dl, 100% mononucleares, tinción Ziehl Nielsen y Gram negativa. Tomografía de tórax identificó adenopatías mediastínicas. Se realizó biopsia ganglionar mediastínica que informó focos de necrosis caseosa con tinción Ziehl Nielsen (+). Se decidió inicio de tratamiento antituberculoso y corticoidal, evolucionando al mes en malas condiciones: con dolor neuropático costal y uveítis bilateral, por ello se decidió agregar metrotrexato (MTX). Por nula respuesta al tratamiento y por tratarse de una enfermedad resistente a corticoides y MTX, se decidió manejo con infliximab, con buena respuesta clínica. Discusión: Estas entidades pueden ser confundidas con una serie de condiciones neurológicas, músculo-esqueléticas o vasculares, requiere de un manejo multidisciplinario precoz, aunque por lo general, la respuesta a tratamiento clásico es escasa.
Introduction: Tuberculosis (TBC) and sarcoidosis have many similarities in their clinical manifestation, radiology and histology. The concept tuberculosis-sarcoidosis, refers to a clinical entity with different kinds of presentations, where TBC may precede, follow or coexist with the course of Sarcoidosis. Case report: 47 year old woman with a history of myomectomy and cholecystectomy, with a history of 4 months of difuse crampy abdominal pain, with hyporexia and 15 kilogram weight loss. Worsening of the pain made her go to the emergency unit, also presented frontal intense headache. In that moment she is admitted in the hospital to be studied for specialists. The symptoms evolved the next day with peripheral right facial paresis, left trigeminal commitment V1-V2, left abdominal hypoesthesia T10 to L1, swallowing symptoms of left lateral cutaneous nerve gloso. It was decided to continue study with lumbar puncture informing: protein: 0.81 mg / dl, glucose 62 mg / dl, 100% mono-nuclear Ziehl Nielsen and Gram negative. Chest CT identified mediastinal lymph nodes. In their biopsy, cheesy foci of necrosis, Ziehl Nielsen (+) was reported. She started corticoidaland antituberculosis treatment but she evolved in poor conditions; adding neuropathic costal pain and bilateral uveitis, so methotrexate (MTX) was added. Because of the none responding symptoms and dealing with a corticoid- and resistent disease it was decided to start with Infliximab, with great clinical response. Discussion: These entities can be confused with several neurological, muscle, or vascular conditions, it requires a multidisciplinary approach early, although generally, the classic response to treatment is low.
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Humanos , Femenino , Persona de Mediana Edad , Sarcoidosis/tratamiento farmacológico , Tuberculosis Pulmonar/tratamiento farmacológico , Tuberculosis Pulmonar/diagnóstico por imagen , Sarcoidosis Pulmonar/diagnóstico por imagen , Metilprednisolona/administración & dosificación , Prednisona/administración & dosificación , Radiografía TorácicaRESUMEN
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...