Eosinophilic gastroenteritis: Pathogenesis, diagnosis, and treatment / 中华医学杂志(英文版)

Eosinophilic gastroenteritis (EGE) is a gastrointestinal disorder of unclear etiology that is characterized by eosinophilic infiltration of the stomach and small intestine, and consists of mucosal, muscular, and serosal subtypes. Eosinophilic infiltration of the gastrointestinal tract is a fundamental histopathological characteristic of EGE and is driven by several T-helper type 2 (Th2)-dependent cytokines and induced by food allergy. Due to the lack of a diagnostic gold standard, EGE has a high rate of delayed diagnosis or misdiagnosis. However, several new diagnostic strategies have been developed, such as novel genetic biomarkers and imaging tests. Although dietary therapy and corticosteroids remain the common choices for EGE treatment, recent decades have seen the emergence of novel treatment alternatives, such as biologics that target particular molecules involved in the pathogenic process. Preliminary investigations and clinical trials have demonstrated the efficacy of biologics and provided additional insights for the era of refractory or corticosteroid-dependent EGE biologics.

Rinosinusitis recalcitrante por granulomatosis eosinofílica con poliangeítis: reporte de un caso clínico y revisión de la literatura / Chronic recalcitrant rhinosinusitis secondary to eosinophilic granulomatosis with polyangiitis: report of a case and literature review

RESUMEN Se expone el caso de un paciente de 40 años con diagnóstico de granulomatosis eosinofílica con poliangeítis subyacente a una rinosinusitis crónica recalcitrante. Se describe el caso y se discuten aspectos relevantes de la literatura al respecto.

ABSTRACT We report the case of a 40-year-old man with diagnosis of chronic recalcitrant rhino-sinusitis secondary to eosinophilic granulomatosis with polyangiitis. We described the case and discuss relevant aspects of the literature about it.

Fascitis eosinofílica: a propósito de un caso / Eosinophilic fasciitis: an account of a case

Eosinophilic fasciitis is a rare entity characterized by induration of skin and peripheral eosinophilia. The pathogenesis is still not well known, but is known association with hematological, autoimmune or neoplastic diseases. The complex and not well standardized treatment. For a patient of 66 years presented with a history of morphea...

La Fascitis Eosinofílica (FE) es una entidad muy infrecuente, caracterizada por induración de piel y eosinofilia periférica. La patogenia aún no es bien conocida, pero es sabida la asociación con enfermedades hematológicas, autoinmunes o neoplásicas. El tratamiento es complejo y no bien estandarizado. Se presenta el caso de una paciente de 66 años, con antecedentes de morfea...

Manifestaciones cardiovasculares de la toxocariasis humana / Cardiovascular manifestations of human toxocariasis

La toxocariasis es una infección parasitaria producida por un helminto que en el ser humano no alcanza su estadio adulto. El hombre es para sus especies, Toxocara canis y Toxocara cati, un hospedador paraténico. Dicha infección puede producir el síndrome de larva migrans visceral, el síndrome de larva migrans ocular y la toxocariasis inaparente. En el síndrome de larva migrans visceral el compromiso de órganos puede incluir hígado, pulmón, piel, sistema nervioso, musculoesquelético, riñón y corazón. Sobre este último, cada vez se reconoce más la importancia que pueden tener las manifestaciones cardiovasculares de la toxocariasis y la relevancia clínica de considerarlas. En el presente artículo, haciendo una búsqueda sistemática de información, se revisan los principales aspectos clinicopatológicos de las manifestaciones cardiovasculares de la toxocariasis incluyendo su fisiopatología, hallazgos de laboratorio, diagnóstico y opciones terapéuticas, con el objeto de llamar la atención acerca de la importancia de esta zoonosis y su relevancia para la medicina cardiovascular en adultos y en niños.

Toxocariasis is a parasitic infection produced by helminths that cannot reach their adult stage in humans. For their etiological species (Toxocara canis and Toxocara cati), man is a paratenic host. Infection by such helminths can produce a variety of clinical manifestations, such as: visceral larvae migrans syndrome, ocular larvae migrans syndrome and covert toxoca-riasis. In the visceral larvae migrans syndrome, the organs that are mainly involved include liver, lungs, skin, nervous system, muscles, kidneys and the heart. Regarding the latter, the importance of cardiovascular manifestations in toxocariasis, as well as its clinical relevance, has increasingly begun to be recognized. The current article is based on a systematic information search, focused mainly on the clinical and pathological aspects of cardiovascular manifestations in toxocariasis, including its pathophysiology, laboratory findings, diagnosis and therapeutical options, with the objective of highlighting its importance as a zoonosis and its relevance to the fields of cardiovascular medicine in adults and children.

Valor del estudio celular del esputo en el seguimiento de las enfermedades inflamatorias de la vía aérea / Value of sputum analysis in the management of inflammatory airway diseases

El análisis celular del esputo, espontáneo u obtenido mediante la técnica de esputo inducido, se ha transformado en una herramienta ampliamente difundida para la evaluación y orientación del tratamiento de las enfermedades inflamatorias de la vía aérea, principalmente asma, enfermedad pulmonar obstructiva crónica y bronquitis eosinofílica. Se han aportado evidencias sobre la utilidad de la técnica del esputo inducido, validada y estandarizada, para ser empleada en pacientes con dificultades para expectorar. Numerosas investigaciones dieron cuenta de la efectividad de basar las decisiones terapéuticas en el componente inflamatorio de la vía aérea mediante el recuento de células en el esputo. Varios estudios mostraron que, en pacientes con asma el análisis celular de esputo guía en la determinación de estrategias para disminuir las exacerbaciones y para mejorar la función pulmonar, aun en pacientes con asma grave, para disminuir el remodelamiento; también se ha descrito su utilidad en pacientes con EPOC, para la disminución de las exacerbaciones.

Cellular analysis of sputum either spontaneous or by induced sputum technique, has become a widespread tool for the evaluation and guidance of treatment of inflammatory diseases of the airway, primarily asthma, COPD and eosinophilic bronchitis. Induced sputum method is a validated, standardized and non-invasive technique, useful in patients with difficulties to expectorate. Its implementation is simple and cost effective. Numerous investigations have shown the effectiveness of basing treatment decisions on the inflammatory component of the airway by counting cells in sputum. Several studies have demonstrated that in patients with asthma, results of this analysis can guide in defining strategies to reduce exacerbations and to improve lung function even in patients with severe asthma, as well as to decrease the remodeling; in addition, a reduction in exacerbations in COPD patients, monitored by this sputum examination, has also been described.

Drug reaction with Eosinophilia and Systemic Symptoms (DRESS) / Drug-induced Hypersensitivity Syndrome (DIHS): a review of current concepts / Reação a drogas com eosinofilia e sintomas sistêmicos (DRESS) / Síndrome da hipersensibilidade induzida por droga (DIHS): revisão dos conceitos atuais

The Drug Reaction with Eosinophilia and Systemic Symptoms syndrome, also known as Drug Induced Hypersensitivity Syndrome presents clinically as an extensive mucocutaneous rash, accompanied by fever, lymphadenopathy, hepatitis, hematologic abnormalities with eosinophilia and atypical lymphocytes, and may involve other organs with eosinophilic infiltration, causing damage to several systems, especially to the kidneys, heart, lungs, and pancreas. Recognition of this syndrome is of paramount importance, since the mortality rate is about 10% to 20%, and a specific therapy may be necessary. The pathogenesis is related to specific drugs, especially the aromatic anticonvulsants, altered immune response, sequential reactivation of herpes virus and association with HLA alleles. Early recognition of the syndrome and withdrawal of the offending drug are the most important and essential steps in the treatment of affected patients. Corticosteroids are the basis of the treatment of the syndrome, which may be associated with intravenous immunoglobulin and, in selected cases, Ganciclovir. The article reviews the current concepts involving this important manifestation of adverse drug reaction.

A síndrome Reação a Drogas com Eosinofilia e Sintomas Sistêmicos, também conhecida como Síndrome da Hipersensibilidade Induzida por Droga apresenta-se clinicamente como uma erupção cutâneomucosa extensa tipo exantemática, associada a febre, linfadenopatia, hepatite, anormalidades hematológicas com eosinofilia e linfócitos atípicos, e pode envolver outros órgãos, produzindo insuficiência renal, infiltrado eosinofílico cardíaco e pulmonar, além de pancreatite. O reconhecimento desta síndrome é de suma importância, uma vez que, a taxa de mortalidade é de cerca de 10% a 20% e uma terapia específica pode ser necessária. Sua etiopatogenia está relacionada a drogas específicas, principalmente os anticonvulsivantes aromáticos, alterações imunes, reativação sequencial de herpesvirus e associação com alelos do HLA. O pronto reconhecimento da síndrome e a retirada da droga desencadeante são os passos mais importantes e essenciais no tratamento dos doentes acometidos. Os corticosteróides são as medicações de escolha para o tratamento da síndrome, podendo ser associados imunoglobulina intravenosa e em, alguns casos selecionados, Ganciclovir. O artigo traz uma revisão dos conceitos atuais que envolvem essa importante manifestação de reação adversa a drogas.

Sindrome dress inducido por paracetamol: reporte de un caso / Sindrome dress inducido por paracetamol: a case report

El síndrome DRESS (reacción a drogas con eosinofilia y síntomas sistémicos) es una reacción de hipersensibilidad tipo IV, caracterizada por fiebre, dermatitis exfoliativa, eosinofilia, linfadenopatía y compromiso visceral. El síndrome DRESS es una entidad poco frecuente que puede comprometer la vida del paciente, siendo de gran importancia su diagnóstico y tratamiento precoz para evitar un desenlace fatal. Los dermatólogos desempeñan un rol importante en la sospecha clínica de esta entidad, siendo habitualmente de manejo multidisciplinario. Se presenta el caso de una paciente con el síndrome DRESS secundario ala administración de Paracetamol, con buena evolución luego de suspendido el fármaco. Recibió tratamiento con corticoides durante dos semanas.

DRESS syndrome (drug reaction with eosinophilia and systemicsymptoms) is a type IV hypersensitivity reaction characterized by fever, exfoliative dermatitis, eosinophilia, lymphadenopathy and visceral involvement. DRESS syndrome is a rare condition that can compromise the patient's life, remain central in the diagnosis and early treatment to prevent a fatal outcome. Dermatologists play an important role in the clinical suspicion of this entity, being usually multidisciplinary. Present the case of a patient with secondary DRESS syndrome with good evolution after the drug suspended. Treated with steroids for two weeks.

Esofagitis eosinofílica: una entidad emergente / Eosinophilic Esophagitis: an emerging entity

La esofagitis eosinofílica (EE) es una enfermedad primaria del esófago, previamente confundida con el reflujo gastroesofágico (RGE), cuyo conocimiento se ha desarrollado principalmente en la última década. Se define como la presencia de síntomas de disfunción esofágica (principalmente disfagia e impactación alimentaria), asociados a por lo menos una biopsia esofágica con más de 15 eosinófilos por campo de mayor aumento (CMA), y la exclusión de RGE. Su prevalencia va en aumento y afecta principalmente a niños y hombres jóvenes de raza blanca con historia previa de atopía. La EE sería causada por una reacción alérgica a ciertos alimentos y/o aeroalérgenos mediada por citoquinas y con cambios genéticos involucrados. La presentación clínica varía con la edad siendo la disfagia el síntoma más frecuente en todos los grupos etarios. El diagnóstico es clínico, endoscópico y anatomopatológico. Se requiere de una endoscopía digestiva alta (EDA) para evaluar hallazgos característicos y tomar biopsias para el estudio histológico. Los tratamientos actuales incluyen medidas dietéticas basadas en la eliminación de la exposición de alérgenos alimentarios y uso de corticoesteroides tópicos. El objetivo de esta revisión es analizar el estado actual de la definición de EE, historia, epidemiología, fisiopatología, diagnóstico y principalmente ayudara mejorar su sospecha diagnóstica y manejo.

Eosinophilic esophagitis (EE) is a primary disease of the esophagus, previously mistaken with gastroesophageal reflux disease (GERD). Its knowledge has developed over the last decade. EE is defined as the presence ofesophageal dysfunction symptoms (mostly dysphagia and food impaction) associated to at least 1 esophageal biopsy with 15 or more eosinophils in 1 high-power field and absence of GERD. Its prevalence is rising, affecting principally white boys and young males with previous history of atopy. EE would be caused by an allergic reaction to certain food and aeroallergens mediated by citoquines with genetic changes involved. Clinical presentation varies with age being dysphagia the most common symptom in all age goups. The diagnosis is clinical, endoscopic and histopathologic. It requires an endoscopy to evaluate mucosal findings and to take the biopsies. Treatment includes elimination diets and topical steroids. The purpose of this review is to analyze the current state of the definition, history, epidemiology, fisiopathology and the diagnosis of EE, with an emphasis on improving its suspicion index and initial management.

Colitis eosinofílica: a propósito de 3 casos y revisión de la literatura / Eosinophilic colitis: report of three cases and literature review

Introducción: Existen afecciones inflamatorias que afectan el colon en forma infrecuente, con amplia variedad en su presentación clínica. Entre ellas, las colitis colágena, indeterminadas, infecciosas, císticas y eosinofílicas, entre otras. La colitis eosinofílica es una enfermedad crónica inflamatoria de etiología desconocida caracterizada por la infiltración masiva de eosinófilos en un segmento del tubo digestivo. Objetivo: Analizar los hallazgos clínicos, histológicos y terapéuticos asociados a la colitis eosinofílica. Diseño: Presentación de casos. Revisión de la literatura. Métodos: Revisión de registros en bases MEDLINE, LILACS y registros de AMA. Pacientes: Se analizaron 3 casos de presentación en los últimos 12 meses. Resultados: Caso 1: femenino, 40 años, dolor abdominal cólico y diarrea. Tomografía Computada: engrosamiento de colon derecho. Colonoscopía: colitis localizada en colon derecho con enantema, congestión sin ulceras. Parasitológico negativo. Caso 2: femenino 26 años, diarrea severa con deshidratación. Parasitológico positivo. Tratamiento con metronidazol sin respuesta. Colonoscopía: pancolitis moderada. Inicia tratamiento con corticoides. Caso 3: femenino 33 años, diarrea, pujo y tenesmo. Perdida de peso. VEDA normal. Colon por enema: dolicocolon. Colonoscopía: tiflitis con ileon normal. Parasitológico negativo. Tratamiento con mesalazina con buena respuesta. Todos tuvieron eosinofilia mayor al 8%. Todas las biopsias fueron del colon derecho y revelaron colitis crónica eosinofílica. Conclusiones: El diagnóstico definitivo es de necesidad para diferenciarlas de enfermedades inflamatorias del colon mediante colonoscopía y biopsia (en especial Enfermedad de Crohn). El tratamiento es sintomático y comprende una variedad de drogas (ketotifeno, corticoides, antihistamínicos, metronidazol, etc.)... (TRUNCADO)

Introduction: There is uncommon colitis with unfrequented presentation. Eosinophilic colitis is an inflammatory chronic bowel disease with unknown etiology. Objective: To evaluate clinical, histological and therapeutic findings and differential diagnosis of chronic eosinophilic colitis. Design: Case report and literature review. Patients: To analyze 3 cases in last 12 months. Results: Case 1: female, 40y, abdominal pain and diarrhea. CT: right colon involvement. Endoscopy: unspecific colitis. Positive Biopsy and negative parasitologic exam. Case 2: female, 26y, severe diarrhea, dehidratation. Positive parasitologic exam. Treatment with metronidazole without reponse. Endoscopy: mild pancolitis with positive biopsy. Treatment with steroids is instaured. Case 3: female, 33y, diarrhea and tenesmus, lost of weight. Upper endoscopy normal, lower endoscopy: cecal inflamation with positive biopsy. Negative parasitologic exam. All patients presented elevated blood eosinophilia and the biopsy were taken from right colon. Conclusions: Symptomatic treatment should be instituted with several drugs according to literature, steroids seems to be the better choice, but with secondary effects. Differential diagnosis should be done always, principally with Crohn’s disease. Current allergic exposition plus environment contamination could represent the physiopathology in some of these cases.

Fasciitis eosinofílica. Presentación de un caso clínico y conceptos actuales para el diagnóstico y tratamiento / Eosinophilic fasciitis. A report of a clinical case and the current concepts for its diagnosis and treatment

Eosinophilic fasciitis is a disorder characterised by induration of the skin due to chronic inflammation and fibrosis of the subcutaneous septa and muscular fascia. It is different from sclerodermia. This is a clinical entity that presents as swelling, tenderness and stiffness of the extremities associated with peripheral eosinophilia. We described a patient with this disorder with 18 month of evolution who had a bilateral carpal tunnel syndrome. Her disease appeared after steroid treatment. A review of medical literature demonstrated that similar clinical pictures are originated by different causes. Some authors propose to encompass this group of disorder under the designation of [quot]fasciitis-panniculitis syndrome[quot] instead of [quot]eosinophilic fasciitis[quot] although the well-known eosinophilic fasciitis is clearly recognized and demonstrated

Hepatic fascioliasis: case report and review

Os autores relatam um caso bem documentado de fascioliase hepatica (FH) tratado com exito com triclabendazole. As manifestacoes clinicas predominantes foram febre, eosinofilia marcante e dor abdominal. Triclabendazole foi dado em duas doses unicas orais de 10mg/kg cada. Nao houve efeitos colaterais, nem recaidas clinicas ou parasitologicas ate tres meses apos tratamento. Baseados nesta experiencia e em outros poucos dados revistos na literatura, triclabendazole poderia ser uma alternativa terapeutica valida no tratamento da fascioliase humana.

Eosinophilic Cellulitis (Wells' Syndrome)

Eosinophilic cellulitis is a rare dermatosis first described by Wells, and characterized by recurrent episodes of sudden outbreaks of erythematous cutaneous swellings often painful or pruritic. Microscopically, the lesion shows diffuse tissue eosinophilia and fibrinoid flame figures, evolution of associated focal necrobiosis, and formation of focal microgranulomas associated with eosinophils. The cause is still unknown but the triggers which have been thought to precipitate the disease include insect bites, parasitic infections such as toxocara, onchocerciasis, nasopharyngeal carcinoma, rheumatoid arthritis and spider bites. However many cases are idiopathic. The author experienced a case of eosinophilic cellulitis of a 52-year-old woman with multiple cutaneous tender plaques of cellulitis for approximately 10 years with history of repeated remission and recurrent episodes. Biopsy was taken from ulcerated edematous nodule of inguinal region under the clinical impression of deep fungal infection, pyoderma gangrenosum and polyarteritis nodosa. Cultures for fungal and common organisms were negative. Histologically, the entire dermis was infiltrated by numerous eosinophils and scattered histiocytes. There were scattered flame figures showing necrobiotic foci in the collagen with accumulation of eosinophils, granulated free cosinophilic granules and histiocytes.

Eosinophilic Cellulitis (Wells' Syndrome)

Eosinophilic cellulitis is a rare dermatosis first described by Wells, and characterized by recurrent episodes of sudden outbreaks of erythematous cutaneous swellings often painful or pruritic. Microscopically, the lesion shows diffuse tissue eosinophilia and fibrinoid flame figures, evolution of associated focal necrobiosis, and formation of focal microgranulomas associated with eosinophils. The cause is still unknown but the triggers which have been thought to precipitate the disease include insect bites, parasitic infections such as toxocara, onchocerciasis, nasopharyngeal carcinoma, rheumatoid arthritis and spider bites. However many cases are idiopathic. The author experienced a case of eosinophilic cellulitis of a 52-year-old woman with multiple cutaneous tender plaques of cellulitis for approximately 10 years with history of repeated remission and recurrent episodes. Biopsy was taken from ulcerated edematous nodule of inguinal region under the clinical impression of deep fungal infection, pyoderma gangrenosum and polyarteritis nodosa. Cultures for fungal and common organisms were negative. Histologically, the entire dermis was infiltrated by numerous eosinophils and scattered histiocytes. There were scattered flame figures showing necrobiotic foci in the collagen with accumulation of eosinophils, granulated free cosinophilic granules and histiocytes.

Kimora disease [angiolymphoid hyperplasia with eosinophilia]

Kimora disease is one of the rarely encountered skin disorders which was first described by kimora in [1969] as a nodular angiolymphoid hyperplasia with eosinophilia. Wills and Whimster [1969] reoprted few cases of angiolymphoid hyperplasia with eosinophilia as the same disease. It was suggested by Lie et al, and accepted by Wilson Jones that kimora disease and temporal arteritis are the same disease. Wilson Jones and Bleehan presented many cases of small angiomatous nodules, showing proliferated blood vessels and numerous eosinophils, designated as Pseudogenic granulomas, which were considered to belong to Kimora Recently Mui PK et al [1989] denied the identity of angiolymphoid hyperplasia with eosinophilia and reported the name of Kimora lymphadcnopathy which considered as a disease with good prognosis. All the reported cases were from Japan and Europe and no specific drug was used for it. This is the first report from Arab countries [Fig.4] which was improved by using methotrexate with predinsoplone