Sarcoidosis infantil: una rara enfermedad pediátrica: caso clínico / Childhood sarcoidosis: a rare pediatric disease: case report

Sarcoidosis is a systemic disease of unknown etiology. It affects various organs, including skin, eyes, joints, liver, heart, spleen and lymph nodes. Lungs are the organs most affected by this disorder and while symptomatology may vary thoughout life, most likely it is never diagnosed. It appears to be endemic in countries like England, US and Japan. The incidence is low in Mexico and Latin America. There is a genetic predisposition, where histocompatibility has a significant role. Histopathological diagnosis requires the presence of non-caseificating granulomatous lesions, with epiteloid cells with cytoplasmatic inclusions called Schaumann cells; these are diagnostic. The most severe complication is pulmonary fibrosis, which responds to steroids. We present the case of a school age child who was diagnosed as suffering from sarcoidosis after an open lung biopsy. Treatment with systemic steroids was initiated and is currenty asymptomatic, under observation and without complications secondary to treatment.

La sarcoidosis es un padecimiento multisistémico, de etiología desconocida. Afecta diversos órganos, entre los que se encuentran la piel, ojos, articulaciones, hígado, corazón, bazo y ganglios linfáticos. Los pulmones son el órgano más afectado en esta enfermedad, y aunque la sintomatología puede variar en cada etapa de la vida, lo común es que pase desapercibida en la mayoría de los casos. En algunos países como Inglaterra, Estado Unidos y Japón es endémica, en México y Latinoamérica su incidencia es baja. Existe una predisposición genética en la cual el complejo mayor de histocompatibilidad (HLA) tiene un papel predominante. El diagnóstico histopatológico requiere de la presencia en los tejidos de lesiones granulomatosas no caseificantes, células epiteloides e inclusiones citoplasmáticas llamadas células de Schaumann. La complicación más severa es la fibrosis pulmonar, por lo que el tratamiento con esteroides es de suma importancia. Presentamos el caso de un escolar al que se le realizo el diagnóstico de sarcoidosis pulmonar por biopsia pulmonar a cielo abierto. Al cual se le inicio tratamiento con esteroide sistémico y actualmente se encuentra bajo vigilancia, asintomático y sin complicaciones secundarias al tratamiento.

Pulmonary sarcoidosis and autoimmune hemolytic anemia: possible common immune pathogenesis

A 49-year-old female presented with autoimmune hemolytic anemia and positive warm antibodies. She was diagnosed with chronic pulmonary sarcoidosis. The patient was given prednisolone after which she had complete remission of autoimmune hemolytic anemia and stabilization of her pulmonary status. A review will follow on association of sarcoidosis with autoimmune disease and its possible role in the development of such phenomenon

Aspergilloma formation in cavitary sarcoidosis / Formação de uma aspergilloma em sarcoidose com cavitação

Pulmonary cavitation is rather uncommon in patients with sarcoidosis, and aspergilloma is even more uncommon in such cases. Here, we present the case of a 63-year-old female patient with cavitary lung disease who had been under treatment for TB for 9 months. A diagnosis of pulmonary sarcoidosis was established based on the fiberoptic bronchoscopy finding of noncaseating granuloma. Treatment with corticosteroids led to a dramatic improvement in symptoms. While under treatment for sarcoidosis, the patient developed an aspergilloma. She presented immediate skin test reactivity to Aspergillus fumigatus, as well as positivity for A. fumigatus serum precipitins. This is the first reported case of aspergilloma formation in a patient with cavitary sarcoidosis in India.

A cavitação pulmonar é rara em pacientes com sarcoidose, e o aspergiloma é ainda mais raro nestes casos. Apresentamos o caso de uma paciente de 63 anos com doença pulmonar cavitária em tratamento para a TB por 9 meses. Estabeleceu-se o diagnóstico de sarcoidose pulmonar com base nos achados de granuloma não-caseoso na fibrobroncoscopia. Houve grande melhora dos sintomas com o tratamento com corticosteroides. A paciente desenvolveu um aspergiloma durante o tratamento para a sarcoidose. Houve reação imediata ao teste cutâneo para Aspergillus fumigatus, assim como resultado positivo para precipitinas de A. fumigatus no soro. Este é o primeiro caso relatado de formação de aspergiloma em um paciente com sarcoidose com cavitação na Índia.

[Anti TNF beneficial of harmful for the lung?]

The Tumor Necrosis Factor is a pro-inflammatory cytokine which plays a key role in the pathogenesis of many diseases. Therefore, the anti Tumor necrosis factors have been used for treating many inflammatory pathologies such as rheumatoid arthritis or Crohn's disease with success. However, the use of these drugs has revealed during the formal years many side effects dominated by the tuberculosis. Lung cancer and asthma might be other side effects of the drugs. More recently they have been used for other pulmonary indications such as sarcoIdosis. Their benefits for treating asthma is being studied. Therefore, a wise use of these drugs is mandatory to benefit from good effects and avoid hamful

Sarcoidosis brocopulmonar: breve puesta al día y descripción de 8 casos / Bronchopulmonary sarcoidosis. Brief review and description of 8 cases

La sarcoidosis es una enfermedad de etiología desconocida, que afecta con mayor frecuencia a adultos jóvenes. El diagnóstico es clínico, radiológico e histopatológico y la presentación frecuente es la de síntomas constitucionales y respiratorios, acompañados de linfoadenopatías hiliares y mediastinales, con o sin compromiso del parénquima pulmonar. La confirmación es anatomopatológica con la demostración de granulomas no caseificantes en los tejidos afectados, con cultivos negativos para micobacterias y hongos. Presentamos los hallazgos clínicos, radiológicos e histopatológicos en 8 pacientes. Cuatro eran de sexo masculino y la edad promedio fue de 44 años (28-59). Dos de los pacientes se encontraban asintomáticos al momento del diagnóstico, cinco de ellos se presentaron con hallazgos toráxicos (tos, dolor retroesternal y arritmias) y uno con síntomas extratoráxicos (artritis de ambos tobillos). Todos presentaron adenopatías mediastinales en los estudios radiológicos y tomográficos y seis de ellos se encontraban en el estadio 2. En cuanto al tratamiento, dos pacientes remitieron espontáneamente, cuatro pacientes recibieron prednisona, uno requirió prednisona y azatioprina y de un paciente no contamos con registros de tratamiento. Uno de nuestros pacientes evolucionó desfavorablemente con aparición y recidiva de lesiones de eritema nodoso severas y bilaterales que motivaron la necesidad de tratamiento permanente con esteroides e inmunosupresores. Si bien se trata de una enfermedad de baja prevalencia en nuestro país, deberíamos mantener un alto índice de sospecha ante la presentación de síntomas respiratorios con lesiones parenquimatosas y adenopatías mediastinales, con o sin manifestaciones extratorácicas.

Sarcoidosis is a disease of unknown etiology affecting most frequently young adults. Diagnosis is based on clinical. Radiological and histopathological features and presentation is frequently of constitutional and respiratory symptoms, hilar and mediastinal adenopathies, with or without parenquimal involvement. Confirmation is histopathological with demonstration of non-caseificating granulomas in affected organs, with negative cultures for mycobacterium and fungi. We describe the clinical, radiological and histopatbological findings in 8 patients. Mean age was 44 years (28-59) and half the patients were male. Two patients were symptom-free at diagnosis, thoracic findings were present in five (cough, retroesternal pain and arrhytbmias) and one had extratboracic symptoms (ankle arthritis). All showed mediastinal adenopathies in radiological and tomographic images (six were in stage 2 and two in stage 1). 1/vo patients had spontaneous remission, four received oral steroids, one required oral steroids and azatbioprine and tbere was no treatment data on one patient. In one patient, recidiva of severe and bilateral erithema nodosum required long term treatment with steroids and immunosupressors. Although sarcoidosis is a low prevalent disease in our country, a high index of suspicion is advisable when facing patients with respiratory symptoms, parenquimal involvement and mediastinal adenopathies, with or without extrathoracic manifestations.

Utility of computed chest tomography (CT scan) in recurrent uveitis.

In recurrent uveitis CT scan could be a superior imaging modality when a primary pulmonary pathology is suspected. Two such cases are illustrated.

Sarcoidose gangliopulmonar: a propósito de dois casos / Ganglio-pulmonary sarcoidosis: apropos of 2 cases

Os autores relatam dois casos de sarcoidose com manifestaçoes intratorácicas envolvendo gânglios linfáticos e pulmoes (tipo radiológico II), além de comprometimento cutâneo peculiar em um dos pacientes. Abordam as bases clínicas para o seu reconhecimento e a importância dos exames complementares para o diagnóstico preciso desta doença multissistêmica, salientando os aspectos fundamentais de sua história natural, prognóstico e tratamento.

Effect of corticosteroid treatment on various serological & bronchoalveolar lavage abnormalities in patients with sarcoidosis.

Effect of daily oral prednisolone treatment was studied in 29 patients with pulmonary sarcoidosis. Twenty normal control subjects were also studied. Pretreatment absolute lymphocyte counts and proportion of lymphocytes in peripheral blood were significantly lower in patients with sarcoidosis as compared to normal controls. Total cell count and the proportion of lymphocytes in bronchoalveolar lavage (BAL) fluid were significantly higher in sarcoidosis. The proportion of CD3+ and CD4+ was significantly lower in peripheral blood and higher in BAL fluid in patients with sarcoidosis. Immunoglobulins (IgG, A and M) and complements (C3, C4 and CH50) were significantly higher both in peripheral blood and BAL fluid. Patients with sarcoidosis were treated with daily oral prednisolone (30 mg/day). Repeat studies were performed after an interval of 4-6 months in 20 patients with sarcoidosis. A significant increase in absolute lymphocyte counts in peripheral blood and decrease in the proportion of lymphocytes in BAL fluid occurred with prednisolone treatment. Proportion of CD3+, CD4+ and B cells increased in peripheral blood and decreased in BAL fluid. Complement and immunoglobulin levels revealed a significant reduction in peripheral blood and BAL fluid. It is concluded that patients with sarcoidosis have peripheral blood lymphopaenia and lymphocytic alveolitis. They have increased levels of complement and immunoglobulins both in the peripheral blood and BAL fluid. All these abnormalities show significant improvement with prednisolone treatment.