Síndrome de DRESS con compromiso pulmonar extenso: una presentación inhabitual / Drug rash with eosinophilia and systemic symptoms or DRESS syndrome: report of one case

Drug rash with eosinophilia and systemic symptoms or DRESS Syndrome is a rare, serious and potentially fatal adverse drug reaction. It is characterized by widespread morbilliform and edematous skin lesions, associated with eosinophilia, lymphadenopathy and internal organ involvement and unusually associated with pulmonary symptoms. We report a 47-year-old male with DRESS syndrome, manifested with typical skin lesions and extensive pulmonary involvement, responding satisfactorily to systemic corticosteroids.

Enfermedad pulmonar intersticial por IgG4 / IgG4-related interstitial lung disease

Las enfermedades relacionadas con inmunoglobulina G4 (IgG4) son un grupo heterogéneo de cuadros clínicos que pueden afectar un solo órgano o tener compromiso sistémico. Se presenta el caso de una mujer de 55 años, internada por un cuadro de tos seca, fiebre, disnea progresiva e insuficiencia respiratoria. La tomografía de tórax evidenció áreas de vidrio esmerilado difusas, patrón reticular y consolidación alveolar. Recibió tratamiento habitual para neumonía de la comunidad y corticoides sistémicos, presentando buena evolución y otorgándose el alta. Al mes se reinterna por estenosis subglótica, progresión de infiltrados pulmonares y aumento del volumen palpebral y de las glándulas submaxilares. Se realizaron biopsias transbronquial y de glándulas salivales que mostraron infiltración por IgG4. También se detectó aumento de los niveles de IgG4 en plasma. Recibió tratamiento con inmunosupresores evolucionando con buena respuesta.

IgG4-related disease is a heterogeneous group of diseases that can affect a single organ or manifest as a systemic disease. We present the case of a 55-year-old female, admitted for dry cough, fever, progressive dyspnea and respiratory failure. Chest CT showed areas of diffuse ground glass, reticular pattern and alveolar consolidation. She received treatment for community acquired pneumonia and systemic corticosteroids with good response. One month later, she was admitted again due to subglottic stenosis, progression of pulmonary infiltrates, and increased palpebral and submaxillary glands volume. Transbronchial and salivary gland biopsies showed infiltration by IgG4. Increased levels of plasma IgG4 were also detected. Immunosuppressive therapy was given with good response.

Hard metal lung disease: a case series / Doença pulmonar por metal duro: uma série de casos

ABSTRACT Objective: To describe diagnostic and treatment aspects of hard metal lung disease (HMLD) and to review the current literature on the topic. Methods: This was a retrospective study based on the medical records of patients treated at the Occupational Respiratory Diseases Clinic of the Instituto do Coração, in the city of São Paulo, Brazil, between 2010 and 2013. Results: Of 320 patients treated during the study period, 5 (1.56%) were diagnosed with HMLD. All of those 5 patients were male (mean age, 42.0 ± 13.6 years; mean duration of exposure to hard metals, 11.4 ± 8.0 years). Occupational histories were taken, after which the patients underwent clinical evaluation, chest HRCT, pulmonary function tests, bronchoscopy, BAL, and lung biopsy. Restrictive lung disease was found in all subjects. The most common chest HRCT finding was ground glass opacities (in 80%). In 4 patients, BALF revealed multinucleated giant cells. In 3 patients, lung biopsy revealed giant cell interstitial pneumonia. One patient was diagnosed with desquamative interstitial pneumonia associated with cellular bronchiolitis, and another was diagnosed with a hypersensitivity pneumonitis pattern. All patients were withdrawn from exposure and treated with corticosteroid. Clinical improvement occurred in 2 patients, whereas the disease progressed in 3. Conclusions: Although HMLD is a rare entity, it should always be included in the differential diagnosis of respiratory dysfunction in workers with a high occupational risk of exposure to hard metal particles. A relevant history (clinical and occupational) accompanied by chest HRCT and BAL findings suggestive of the disease might be sufficient for the diagnosis.

RESUMO Objetivo: Descrever aspectos relacionados ao diagnóstico e tratamento de pacientes com doença pulmonar por metal duro (DPMD) e realizar uma revisão da literatura. Métodos: Estudo retrospectivo dos prontuários médicos de pacientes atendidos no Serviço de Doenças Respiratórias Ocupacionais do Instituto do Coração, localizado na cidade de São Paulo, entre 2010 e 2013. Resultados: Entre 320 pacientes atendidos no período do estudo, 5 (1,56%) foram diagnosticados com DPMD. Todos os pacientes eram do sexo masculino, com média de idade de 42,0 ± 13,6 anos e média de tempo de exposição a metal duro de 11,4 ± 8,0 anos. Os pacientes foram submetidos a avaliação clinica, história ocupacional, TCAR de tórax, prova de função pulmonar, broncoscopia com LBA e biópsia pulmonar. Todos apresentaram distúrbio ventilatório restritivo. O achado de imagem à TCAR de tórax mais frequente foi de opacidades em vidro fosco (em 80%). Em 4 pacientes, o LBA revelou presença de células gigantes multinucleadas. Em 3, foi diagnosticada pneumonia intersticial por células gigantes na biópsia pulmonar. Houve o diagnóstico de pneumonia intersticial descamativa associada à bronquiolite celular em 1 paciente e de pneumonite de hipersensibilidade em 1. Todos foram afastados da exposição e tratados com corticoide. Houve melhora em 2 pacientes e progressão da doença em 3. Conclusões: Apesar de ser uma entidade rara, a DPMD deve ser sempre considerada em trabalhadores com risco ocupacional elevado de exposição a metais duros. A história clínica e ocupacional associada a achados em TCAR de tórax e LBA sugestivos da doença podem ser suficientes para o diagnóstico.

Doenças intersticiais pulmonares no CTI: Quando pensar? Como conduzir? / Interstitial lung disease in ICU: When to think? How to manage?

As doenças intersticiais pulmonares podem ser secundárias a um processo agudo ou resultar de uma inflamação e cicatrização progressiva do parênquima pulmonar. Elas podem ser desafiadoras na terapia intensiva, tanto para o diagnóstico quanto para o tratamento. A exclusão de causas reversíveis de insuficiência respiratória e medidas de suporte são os pontos principais da terapia. É fundamental a revisão das imagens radiológicas prévias e a realização de anamnese detalhada. A coleta de lavado broncoalveolar por broncoscopia e/ou biopsia cirúrgica podem ser necessárias. Nas exacerbações agudas é recomendado corticosteróide e antibióticos. Frequentemente é necessária a ventilação mecânica para melhorar a troca gasosa e a baixa complacência pulmonar. A insuficiência respiratória é um final comum das doenças intersticiais crônicas e apresentam um mau prognóstico. Devido a isto, a ventilação mecânica deve ser oferecida nos casos com processos patológicos reversíveis ou nos candidatos à transplante pulmonar...

Interstitial lung disease (ILD) can be an acute process or the result of progressive inflammation and scarring of the pulmonary parenchyma. They can be challenging in the ICU both to diagnose and treatment. Exclusion of reversible causes of respiratory failure while administering supportive care are the mainstay treatment of patients with ILD with respiratory failure. It is fundamental to review previous imaging, detailed medication and occupational history. Bronchoscopy with bronchoalveolar lavage and/or surgical lung biopsy may be recommended. If an exacerbation syndrome is identified, corticosteroids and antibiotics are advised. Often mechanical ventilation is needed to control poor compliance and gas exchange. Respiratory failure is a common end of chronic ILD and carries a poor outcome. Mechanical ventilation should only be offered in select cases, most notably patients with reversible processes or those considered candidates for lung transplantation...

Rheumatoid arthritis associated interstitial lung disease: a review / La artritis reumatoide asociada a la enfermedad intersticial pulmonar: una revisión

Rheumatoid arthritis is a common inflammatory disease affecting about 1% of the population. Interstitial lung disease is a serious and frequent complication of rheumatoid arthritis. Rheumatoid arthritis associated interstitial lung disease (RA-ILD) is characterized by several histopathologic subtypes. This article reviews the proposed pathogenesis and risk factors for RA-ILD. We also outline the important steps involved in the work-up of RA-ILD and review the evidence for treatment and prognosis.

La artritis reumatoide es una enfermedad inflamatoria común, que afecta cerca del 1% de la población. La enfermedad pulmonar intersticial es una complicación frecuente y seria de la artritis reumatoide y esta asociación está caracterizada por diferentes subtipos histológicos. Este artículo analiza la patogénesis y los factores de riesgo de esta asociación. Además señala los esfuerzos realizados y las evidencias para el diagnóstico y tratamiento.

Treatment Response and Long Term Follow-up Results of Nonspecific Interstitial Pneumonia

The purpose of this study was to investigate the long-term clinical course of non-specific interstitial pneumonia (NSIP) and to determine which factors are associated with a response to steroid therapy and relapse. Thirty-five patients with pathologically proven NSIP were included. Clinical, radiological, and laboratory data were reviewed retrospectively. The male-to-female ratio was 7:28 (median age, 52 yr). Thirty (86%) patients responded to steroid therapy, and the median follow-up was 55.2 months (range, 15.9-102.0 months). Five patients (14%) showed sustained disease progression and three died despite treatment. In the five with sustained disease progression, NSIP was associated with various systemic conditions, and the seropositivity of fluorescent antinuclear antibody was significantly associated with a poor response to steroids (P = 0.028). The rate of relapse was 25%, but all relapsed patients improved after re-treatment. The initial dose of steroids was significantly low in the relapse group (P = 0.020). In conclusion, progression is associated with various systemic conditions in patients who show progression. A low dose of initial steroids is significantly associated with relapse.

Lymphocytic Interstitial Pneumonia in Primary Sjogren's Syndrome: A Case Report

Sjogren's syndrome (SS) is an autoimmune disorder in which lymphocytes infiltrate the exocrine glands, resulting in the development of sicca symptoms. Lymphocytes may also invade various other organs and cause diverse symptoms. Interstitial pneumonia has been observed frequently in SS patients. Typically, the pneumonia responds well to systemic steroids, and fatal cases are rare. We experienced a case of lymphocytic pneumonia accompanied by SS and treated with cyclophosphamide pulse therapy, and we present details of the case herein.

A Case of Multicentric Castleman's Disease Having Lung Lesion Successfully Treated with Humanized Anti-interleukin-6 Receptor Antibody, Tocilizumab

This report presents the case of a patient demonstrating multicentric Castleman's disease (MCD) with a lung lesion that was successfully treated with an anti-interleukin-6 receptor antibody, tocilizumab in combination with corticosteroid and tacrolimus. A 43-yr-old female with abnormal shadows on a chest X-ray was referred to the hospital for further examination. She was diagnosed as having MCD based on the characteristic pathology of inguinal lymph node, lung lesions, laboratory data, and undifferentiated arthritis. Corticosteroid and rituximab therapy did not fully ameliorate the symptoms; thus, the therapeutic regimen was changed to include tocilizumab, oral corticosteroid and tacrolimus. This regimen resulted in clinical remission and the dose of tocilizumab and corticosteroid could be tapered. Tocilizumab in combination with corticosteroid and tacrolimus may therefore be a beneficial treatment regimen for lung lesions associated with MCD.

Familial Interstitial Lung Disease in Two Young Korean Sisters

Most of the interstitial lung diseases are rare, chronic, progressive and fatal disorders, especially in familial form. The etiology of the majority of interstitial lung disease is still unknown. Host susceptibility, genetic and environmental factors may influence clinical expression of each disease. With familial interstitial lung diseases, mutations of surfactant protein B and surfactant protein C or other additional genetic mechanisms (e.g. mutation of the gene for ATP-binding cassette transporter A3) could be associated. We found a 21 month-old girl with respiratory symptoms, abnormal radiographic findings and abnormal open lung biopsy findings compatible with nonspecific interstitial pneumonitis that is similar to those of her older sister died from this disease. We performed genetic studies of the patient and her parents, but we could not find any mutation in our case. High-dose intravenous methylprednisolone and oral hydroxychloroquine were administered and she is still alive without progression during 21 months of follow-up.

Enfermedades pulmonares intersticiales difusas idiopáticas: Diagnóstico y tratamiento / Interstitial lung diseases: diagnosis and treatment

Las enfermedades pulmonares intersticiales difusas idiopáticas (EPIDI) en pediatría son poco frecuentes. Objetivo: Describir los hallazgos clínicos, radiológicos y el patrón histológico, además de la respuesta al tratamiento en 9 pacientes con EPIDI, enrolados entre los años 1997-2002 en nuestra unidad. Material y Método: Los 9 pacientes fueron sometidos a una evaluación de laboratorio e inmunológica, incluyendo estudios microbiológicos mediante lavado broncoalveolar (LBA), con la finalidad de detectar enfermedades sistémicas. El diagnóstico fue hecho por biopsia pulmonar a través de Video Toracoscopía Asistida (VTA). El sitio de la biopsia fue elegido mediante tomografía axial computarizada (TAC) de tórax de alta resolución y corte fino. Resultados: Al momento del diagnóstico los pacientes tenían en promedio 5 años de edad, 6 de sexo femenino. Todos presentaban tos, taquipnea, .Velcro. e infiltrados intersticiales en la Rx y en la TAC de tórax. En promedio la latencia en el diagnóstico fue de 3,2 años. Cinco niños iniciaron los síntomas antes de los 3 meses de edad. Tres pacientes tenían una inmunodeficiencia primaria (Síndrome cartílago-pelo, Síndrome velo-cardio-facial y Síndrome hiper Ig M) y uno presentaba una enfermedad autoinmune. La biopsia pulmonar a través de VTA no presentó complicaciones y orientó el diagnóstico en todos los casos. El patrón histopatológico encontrado en la serie fue: 7 neumonitis intersticial crónica inespecífica (NICI), uno neumonitis intersticial linfoide (NIL) y uno neumonitis intersticial descamativa (NID). El paciente con NID recibió pulsos de Metilprednisolona y uno con NICI prednisona, ambos presentaron una breve latencia del diagnóstico y al término de dos meses de tratamiento mostraron remisión completa. Los pacientes que presentaban una inmunodeficiencia primaria durante el tratamiento con prednisona o cloroquina, desarrollaron una fibrosis pulmonar severa, insuficiencia respiratoria y finalmente fallecieron (2 años de evolución). Un niño que en la biopsia sólo se encontró fibrosis no recibió tratamiento. Los otros tres pacientes se mantuvieron estables, tratados con cloroquina más prednisona en dos casos y con prednisona en el otro. Conclusión: En esta serie, la biopsia pulmonar a través de VTA, técnica que presentó escasa morbilidad, permitió confirmar el diagnóstico en todos los casos.

Experiencia clínica de la utilidad del lavado broncoalveolar en pediatría / Bronchoalveolar lavage in children

Introducción: Se ha descrito que el lavado broncoalveolar (LBA) tiene utilidad en la determinación etiológica de pacientes con neumonías de evolución inhabitual y en pacientes inmunodeprimidos. La información publicada es en su mayoría extranjera, existiendo escasa información en Chile. Objetivo: revisar restrospectivamente la experiencia clínica de LBA realizados en el servicio de pediatría del Hospital Clínico de la Universidad Católica entre abril 1993 y julio 2001. pacientes y métodos: Se utilizó un fibrobroncoscopio olympus de 3,6 mm de diámetro externo, con una vieocámara sony DXC-C1 utilizando para el LBA 4 a 6 alícuotas de 1 cc/kg (volumen máximo: 15 ml) de suero fisiológico al 0,9 por ciento c/u, tomando muestras de secreción bronquial, las que fueron enviadas para estudio viral, bacteriológico, tuberculosis, hongos y pneumocystis carinii. Resultados: se realizaron un total de 97 LBA, analizándose el resultado de 82 LBA de 74 pacientes. hubo 60 LBA en inmunosuprimidos, cuyos diagnósticos fueron: neumonía 15, neumonía intersticial 9, neumonía con atelectasia 3, distrés respiratorio agudo 3. de estos 30 LBA, hubo un total de 14 resultados positivos (46,6 por ciento), 10 para germen patógeno (33,3 por ciento); CMV 5, reacción de polimerasa en cadena (PCR) (+) pneumocystis carinii 2, CMV + pneumocystis carinii2. De los 52 LBA en inmunocompetentes los diagnïsticos fueron de neumonía con o sin atelectasia 32, sospecha de hemosiderosis 7, tuberculosis 4, bronquiectasias 3 y 6 misceláneos. Hubo un total de 21 de 44 cultivos positivos (47,7 por ciento), aislándose haemophilus influenzae en 7, pseudomona aeruginosa 4, CMV 3 y staphylococcus aureus 2. El LBA cambió la conducta en 29 pacientes (35,3 por ciento) y ésta incluyó: cambio de terapia antibiótica en 14 pacientes, inicio de ganciclovir en 7. Conclusiones: el LBA es una herramienta de gran ayuda en el paciente que presenta una neumonía inhabitual, en especial en inmunodeprimidos, ya que al determinar un diagnóstico preciso nos permite una elección adecuada de tratamiento

Lymphocytic interstitial pneumonitis associated with autoimmune hepatitis.

A 49-year-old woman was diagnosed as autoimmune hepatitis and started on steroids and azathioprine. Subsequently, she developed fever; chest radiograph showed lower lobe nodular opacities. Bronchoalveolar lavage and transbronchial lung biopsy confirmed the diagnosis of lymphocytic interstitial pneumonitis.

Neumonía intersticial no específica / Diffuse idiopathic interstitial lung disease

Se presenta un paciente, clínicamente calificado como broncoquiolitis obliterante con neumonía demostró una neumonía intersticial inespecífica, entidad poco frecuente, recientemente descrita, cuyo reconocimiento es importante por responder a tratamiento corticoidal, al contrario de la fibrosis intersticial usual, afección más frecuente con la cual ha sido confundida bajo la categoría clínica de fibrosis pulmonar idiopática