Pulmonary sarcoidosis with arterial involvement

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.

Compromiso torácico de la sarcoidosis en tomografía computarizada: claves clínicas y radiológicas / Thoracic Involvement of Sarcoidosis in Computed Tomography: Radiological Manifestations and Key Findings

La sarcoidosis es una enfermedad crónica y multisistémica de etiología poco clara. La presentación es variable, de acuerdo con la procedencia geográfica del paciente, pero predomina en personas afrodescendientes y escandinavas. Las anormalidades torácicas son muy frecuentes en los pacientes con sarcoidosis; la afectación más común es ganglionar y la menos común es la del corazón. Las manifestaciones radiológicas más frecuentes en el compromiso torácico por sarcoidosis son las adenomegalias hiliares y mediastinales, así como nódulos pulmonares de distribución perilinfática.

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.

Sarcoidosis al descubierto: Lo que deberíamos informar en las imágenes torácicas / Sarcoidosis Uncovered: What we should Report in Thoracic Image

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.

Pulmonary Sarcoidosis That Developed During the Treatment of a Patient With Crohn Disease by Using Infliximab

For inflammatory bowel disease (IBD), antitumor necrosis factor treatment offers a new direction for both patients and medical doctors. This treatment has dramatically improved the quality of life for patients with ulcerative colitis and Crohn disease (CD). However, with increasing usage and longer follow-up periods, a wider range of possible adverse effects may be encountered. We report an unusual case of pulmonary sarcoidosis developed during the treatment of a patient with CD by using infliximab. A 30-year-old male who had been treated for CD with infliximab for 18 months was admitted due to abnormal opacities on chest radiography. Chest computed tomography displayed clustered small nodules in both lobes and enlarged multiple lymph nodes. The patient was diagnosed with sarcoidosis from the results of a biopsy of the subcarinal lymph node. Lung lesions were improved five months after infliximab was stopped.

Tuberculosis - sarcoidosis, un desafío diagnóstico: reporte de un caso / Tuberculosis - sarcoidosis, a challenge diagnosis: case report

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.

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...

Endobronchial Ultrasound-guided Transbronchial Needle Aspiration versus Standard Bronchoscopic Modalities for Diagnosis of Sarcoidosis: A Meta-analysis / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) is an effective technique used to precisely detect enlarged mediastinal lymph nodes. The efficacy of EBUS-TBNA versus standard modalities for the diagnosis of sarcoidosis remains to be elucidated. In this meta-analysis, we compared the efficacies of these methods.</p><p><b>METHODS</b>We searched PubMed, Embase, The Cochrane Library, Wanfang, Cpvip, CNKI, and the bibliographies of the relevant references. We analyzed the data obtained with Revman 5.2 (Nordic Cochrane Center, Copenhagen, Denmark) and Stata 12.0 software (Stata Corporation, College Station, TX, USA). The Mantel-Haenszel method was used to calculate the pooled odds ratio (OR) and 95% confidence intervals (CIs).</p><p><b>RESULTS</b>Sixteen studies with a total of 1823 participants met the inclusion criteria, and data were extracted regarding the diagnostic yield of each approach. The ORs for EBUS-TBNA versus transbronchial lung biopsy (TBLB) for the diagnosis of sarcoidosis ranged from 0.26 to 126.58, and the pooled OR was 5.89 (95% CI, 2.20-15.79, P = 0.0004). These findings indicated that EBUS-TBNA provided a much higher diagnostic yield than TBLB. The pooled OR for EBUS-TBNA + TBLB + endobronchial biopsy (EBB) versus TBNA + TBLB + EBB was 1.54 (95% CI, 0.61-3.93, P = 0.36), implying that there was no significant difference between their diagnostic yields. However, clinical heterogeneity was reflected in the nature of the studies and in the operative variables.</p><p><b>CONCLUSIONS</b>The results of this meta-analysis suggest that EBUS-TBNA + TBLB + EBB could be used for the diagnosis of sarcoidosis, if available. At medical centers without EBUS-TBNA, TBNA + TBLB + EBB could be used instead.</p>

Granulomatous Lung Disease: A Novel Complication following Metallosis from Hip Arthroplasty / 대한고관절학회지

A case of a female patient with local and systemic complications of metallosis, following catastrophic wear of a revised hip arthroplasty, is presented. The patient had a history of a fractured ceramic-on-ceramic implant, exchanged with a metal-on-polyethylene prosthesis. Systemic complications included sarcoidosis-like reactions, presenting as granulomatous lung disease, along with chorioretinitis, erythema nodosum, and cardiomyopathy. High local and circulating cobalt and chromium levels established the diagnosis. The patient underwent extensive debridement and implant revision. One year postoperatively, she had no respiratory symptoms or functional impairment. Local and systemic complications of metallosis after hip arthroplasty should be promptly recognized and treated operatively.

A Case of Papillary Thyroid Carcinoma Coexistent with Thyroid Sarcoidosis / 대한갑상선학회지

Sarcoidosis is a systemic granulomatous disease that primarily affects the lung and lymphatic system of the body. The involvement of thyroid gland with sarcoidosis is uncommon. Moreover, sarcoidosis with thyroid cancer are rarely reported in the world. We encountered papillary thyroid carcinoma (PTC) coexistent with pulmonary sarcoidosis. A 35-year-old female with a medical history of pulmonary sarcoidosis visited the endocrinology department for evaluation of the thyroid nodule. Thyroid ultrasonography showed multiple markedly hypoechoic nodules in the left thyroid lobe. Then ultrasonogram-guided fine needle aspiration of left thyroid nodule was positive for papillary carcinoma. She underwent total thyroidectomy with left complete cervical lymph node dissection. The histopathology was confirmed PTC combined with non-caseating granulomatous inflammation suggesting sarcoidosis on thyroid mass and lymph node. We report here a case of PTC coexistent with thyroid involvement of pulmonary sarcoidosis.

Gastric Involvement of Pulmonary Sarcoidosis / 이화의대지

Gastric sarcoidosis is a rare disease accounting for 0.1~0.9% of all sarcoidosis cases. It presents either as a systemic disease or as an isolated finding. Diagnosis is established with biopsy of a lesion. It is important to distinguish between sarcoidosis and a sarcoid-like reaction, which can be caused by Crohn's disease, foreign body reaction, fungal infection, tuberculosis, or malignancy. We report a 60-year-old woman with both gastric and pulmonary sarcoidosis.

Signos radiológicos: el signo de la galaxia / Radiology signs: the galaxy sign

El signo de la galaxia representa una lesión tipo masa, compuesta por innumerables nódulos granulomatosos coalescentes, más concentrados en el centro que en la periferia. Inicialmente se describió en paciente con sarcoidosis, y se denominó el signo de la galaxia sarcoidea. Actualmente se sabe que puede estar presente en otras entidades como la tuberculosis, el cáncer pulmonar y la fibrosis pulmonar masiva de las neumoconiosis. Es importante su identificación y adecuada interpretación, dado que en asociación con otros signos tomográficos permite una orientación al diagnóstico de sarcoidosis.

The galaxy sign represents a mass-like lesion, composed of innumerable coalescing granulo-matous nodules, more concentrated in the center than at the periphery. It was initially described in patients with sarcoidosis, and was referred to as the "sarcoid galaxy" sign. It is now known to be present in other entities such as tuberculosis, lung cancer and the pulmonary fibrosis mass of pneumoconiosis. Its identification and adequate interpretation is important, given that in association with other tomographic signs it allows an orientation toward the diagnosis of sarcoidosis.

Case Report of Pulmonary Sarcoidosis Suspected to be Pulmonary Metastasis in a Patient with Breast Cancer / Journal of the Korean Cancer Association, 대한암학회지

Standard endocrine therapy and chemotherapy can induce long-term remission in breast cancer patients; however, breast cancer can recur at any site. Pulmonary nodules with lymphadenopathy in advanced cancer patients are likely to be assumed as metastases. A 44-year-old woman with a history of breast cancer was presented to our institution with abnormal findings on 18-fluorodeoxyglucose positron emission tomography imaging, which suggested lung metastasis. She had previously been diagnosed with breast cancer (T1N2M0, Stage IIIa, intraductal carcinoma, triple negative cancer). Histological analysis of the mediastinal lymph node biopsy demonstrated sarcoidosis, showing a chronic, non-caseating, granulomatous inflammation. Our case highlights the need for non-malignant diagnoses in those with prior malignancies, and the need for histological evaluations in the event of first recurrence following potentially curative therapy.

Subcutaneous Sarcoidosis in a Patient with Myasthenia Gravis / 대한피부과학회지

Subcutaneous sarcoidosis is rare and presents as tender or painless nodules that mostly occur on the extremities. We report a 47-year-old female patient presenting with a 1-month history of multiple asymptomatic subcutaneous nodules on the extremities. The patient had a history of myasthenia gravis for 5 years. Histopathological findings from the subcutaneous nodules showed non-caseating granulomas compatible with sarcoidosis. On inspection for internal involvement, pulmonary sarcoidosis was also detected by chest computed tomography, bronchoalveolar lavage, and transbronchial lung biopsy. The concurrence of sarcoidosis and myasthenia gravis is rare and has not been reported in the Korean literature. It may be supposed that the concurrence of these 2 separate entities is coincidental, but it has been reported that myasthenia gravis with specific antibodies is associated with sarcoidosis. This suggests that a common immunopathogenic mechanism may exist between these 2 diseases.

An unusual case of childhood sarcoidosis: Un caso clínico infrecuente / Sarcoidosis infantil

Sarcoidosis is a systemic granulomatous disease of unknown etiology that may affect many systems, mainly lungs. Most of the patients present at stages I and II lung involvement. Pulmonary infltrates without hilar lymphadenopathy (state III) rarely occurs. Extrapulmonary organ involvement is common in pediatric sarcoidosis. The aim of this report is to present an unusual case of childhood sarcoidosis with stage III lung involvement without any extrapulmonary organ involvement. A 7-year-old girl presented with the complaints of malaise, fatigue, weight loss and dyspnea. There was patchy, bilateral ground glass view at high resolution computer tomography. Video assisted thoracoscopic lung biopsy was performed and histopathological examination showed nonnecrotising epitheloid-cell granulomas with giant cells. She did not have any hilar or extrapulmonary organ involvement and pulmonary sarcoidosis at stage III was diagnosed. Sarcoidosis should be considered in the differential diagnosis of children with interstitial lung disease.

La sarcoidosis es una enfermedad granulomatosa sistémica de etiología desconocida que puede afectar varios órganos, principalmente el pulmón. La mayoría de los pacientes presentan estadios I y II de compromiso pulmonar. Los infltrados pulmonares, sin linfadenopatías hiliares (estadio III), son infrecuentes. El compromiso de órganos extrapulmonares es común en la sarcoidosis infantil. El objetivo es presentar un caso infrecuente de sarcoidosis infantil con compromiso pulmonar en estadio III sin afectación de los órganos extrapulmonares. Una niña de 7 años consultó por malestar general, fatiga, pérdida de peso y disnea. En la tomografía computarizada de alta resolución se observó un infltrado bilateral de tipo esmerilado. Se realizó una biopsia pulmonar por videotoracoscopia. La histopatología mostró granulomas no necrosantes de células epitelioides con células gigantes. No había compromiso hiliar ni de otros órganos, por lo que el diagnóstico fue de sarcoidosis en estadio III. La sarcoidosis debe considerarse en el diagnóstico diferencial de los niños con enfermedad intersticial pulmonar.

An unusual case of childhood sarcoidosis: an unusual clinical case / An unusual case of childhood sarcoidosis: an unusual clinical case.

Sarcoidosis is a systemic granulomatous disease of unknown etiology that may affect many systems, mainly lungs. Most of the patients present at stages I and II lung involvement. Pulmonary infltrates without hilar lymphadenopathy (state III) rarely occurs. Extrapulmonary organ involvement is common in pediatric sarcoidosis. The aim of this report is to present an unusual case of childhood sarcoidosis with stage III lung involvement without any extrapulmonary organ involvement. A 7-year-old girl presented with the complaints of malaise, fatigue, weight loss and dyspnea. There was patchy, bilateral ground glass view at high resolution computer tomography. Video assisted thoracoscopic lung biopsy was performed and histopathological examination showed nonnecrotising epitheloid-cell granulomas with giant cells. She did not have any hilar or extrapulmonary organ involvement and pulmonary sarcoidosis at stage III was diagnosed. Sarcoidosis should be considered in the differential diagnosis of children with interstitial lung disease.

A Case of Pulmonary Sarcoidosis with Endobronchial Nodular Involvement / 결핵및호흡기질환

Sarcoidosis is a multisystemic disorder of unknown cause that is characterized pathologically by noncaseating granulomas. Diagnosis is based on the exclusion of other infectious, interstitial, and neoplastic diseases and on the typical pathology. Although the lungs and mediastinal lymph nodes are almost involved, endobronchial nodular lesions of sarcoidosis with lung involvements are rare. We report a case of sarcoidosis with lung involvements and endobronchial nodules as confirmed by bronchial biopsy.

Sarcoidose pulmonar: uma atualização / Pulmonary sarcoidosis: an update

Na Medicina, como em muitas áreas da ocupação humana, o oculto tende a encantar. Talvez isso explique, em parte, o grande interesse em torno da sarcoidose, uma doença repleta de incógnitas e desafios ao seu entendimento. Esta revisão teve como objetivo apresentar os principais avanços no entendimento da imunopatogenia, diagnóstico e tratamento da sarcoidose pulmonar. Vários estudos têm enfatizado a importância do alelo DRB1*03 do antígeno leucocitário humano e da exposição ambiental na patogenia da doença. No tocante ao diagnóstico, o ultrassom endoscópico transesofágico, o ultrassom endobrônquico e a tomografia por emissão de pósitrons com 18F fluordesoxiglicose têm sido apresentados como exames promissores, inclusive na avaliação da resposta ao tratamento. Várias recomendações baseadas em evidências têm sugerido o uso de imunobiológicos nos casos de resistência aos corticosteroides, especialmente na sarcoidose refratária ao tratamento. O melhor entendimento da relação entre sarcoidose e exposição ambiental pode auxiliar na diferenciação dos vários fenótipos da doença. É possível que uma abordagem diagnóstica e terapêutica distinta possa ser utilizada em um futuro próximo, com base nesses fenótipos.