Acometimento da função ventricular direita em pacientes com esclerose sistêmica e doença pulmonar intersticial: avaliação pelo doppler tecidual / Impairment of right ventricular function in patients with systemic sclerosis and interstitial lung disease: tissue doppler evaluation

Introdução: A esclerose sistêmica (ES) é uma doença autoimune do tecido conjuntivo que cursa com fibrose e disfunção microvascular. O envolvimento dos órgãos viscerais, incluindo os pulmões e o coração, é a principal causa de óbito na ES. Nesse contexto, analisamos a relação entre os parâmetros ventriculares direitos (VD) pela ecocardiografia com Doppler tecidual e o acometimento pulmonar em pacientes com ES. Métodos: Os pacientes que preencheram os Critérios de Classificação da ES de 2013 foram submetidos à ecocardiografia com Doppler tecidual para avaliação da função sistólica (fração de ejeção) ventricular esquerda (VE), enquanto a função sistólica do VD foi avaliada por meio da fração de variação de área do VD (fractional area change ­ FAC), velocidade (sistólica) do Doppler tecidual, índice de desempenho miocárdico (IDM) e excursão sistólica do plano anular tricúspide (TAPSE). A pressão sistólica pulmonar foi estimada por insuficiência tricúspide. A tomografia computadorizada de alta resolução (TCAR) de tórax avaliou a presença de fibrose pulmonar. De acordo com os resultados da TCAR, os pacientes foram divididos em 2 subgrupos: Grupo I, incluindo pacientes com fibrose pulmonar (n=26), e Grupo II sem fibrose (n=17). Resultados: Entre os 43 pacientes com ES, a maioria era do sexo feminino (86%) com idade de 51±12 anos. Todos os pacientes apresentavam função ventricular sistólica normal, avaliada pela FEVE>55% e FAC VD>35%. Não houve diferença significativa em termos de idade ou duração da doença para os grupos. Exceto pela diminuição das velocidades do Doppler tecidual em pacientes com fibrose pulmonar, todos os índices de desempenho do VD foram semelhantes. Conclusão: Em pacientes com ES e fibrose pulmonar, o Doppler tecidual identifica acometimento miocárdico longitudinal precoce do VD, apesar do desempenho sistólico radial preservado do VD.(AU)

Introduction: Systemic sclerosis (SSc) is an autoimmune tissue connective disease that courses with fibrosis and microvascular dysfunction. Involvement of the visceral organs, including the lungs and heart, is the main cause of death among patients with SSc. In this context, here we analyzed the relationship between right ventricle (RV) parameters assessed by tissue Doppler echocardiography and lung involvement in patients with SSc. Methods: Patients fulfilling the 2013 SSc Classification Criteria underwent tissue Doppler echocardiography for the assessment of left ventricular (LV) systolic function (ejection fraction) and RV fractional area change (FAC), tissue Doppler s' (systolic) velocity, myocardial performance index, and tricuspid annular plane systolic excursion for the assessment of RV systolic function. Pulmonary systolic pressure was estimated using tricuspid regurgitation. Chest high-resolution computed tomography was used to evaluate the presence of pulmonary fibrosis. The patients were divided into two subgroups accordingly: Group I, patients with pulmonary fibrosis (n=26); and Group II, those without fibrosis (n=17). Results: Among the 43 patients with SSc, most were female (86%), and the mean age was 51 ± 12 years. All patients had normal systolic ventricular function as evidenced by an LV ejection fraction > 55% and an RV FAC > 35%. No significant intergroup difference was noted in age or disease duration. Except for a decreased tissue Doppler s' velocity in patients with lung fibrosis, all indexes of RV performance were similar. Conclusion: In patients with SSc and pulmonary fibrosis, tissue Doppler identified early RV longitudinal myocardial involvement despite preserved RV radial systolic performance.(AU)

Complete Atrioventricular Block and Cardiopulmonary Involvement in Rapidly Progressive Systemic Sclerosis

Abstract The heart and lung are target organs in systemic sclerosis (SSc) and similar symptoms (dyspnea and cough) may make the differential diagnosis between the two lesions difficult. In addition, complete atrioventricular block (CAVB) is a rare complication of this disease. This case report is about a patient with SSc and pulmonary fibrosis who was admitted to the emergency room with CAVB, heart failure (HF) and progressive worsening of the underlying disease.

Combined pulmonary fibrosis and emphysema: an increasingly recognized condition / Combinação de fibrose pulmonar e enfisema: uma doença cada vez mais reconhecida

Combined pulmonary fibrosis and emphysema (CPFE) has been increasingly recognized in the literature. Patients with CPFE are usually heavy smokers or former smokers with concomitant lower lobe fibrosis and upper lobe emphysema on chest HRCT scans. They commonly present with severe breathlessness and low DLCO, despite spirometry showing relatively preserved lung volumes. Moderate to severe pulmonary arterial hypertension is common in such patients, who are also at an increased risk of developing lung cancer. Unfortunately, there is currently no effective treatment for CPFE. In this review, we discuss the current knowledge of the pathogenesis, clinical characteristics, and prognostic factors of CPFE. Given that most of the published data on CPFE are based on retrospective analysis, more studies are needed in order to address the role of emphysema and its subtypes; the progression of fibrosis/emphysema and its correlation with inflammation; treatment options; and prognosis.

A combinação de fibrose pulmonar e enfisema (CFPE) é cada vez mais reconhecida na literatura. Os pacientes são geralmente fumantes pesados ou ex-fumantes nos quais a TCAR de tórax revela enfisema nos lobos superiores e, concomitantemente, fibrose nos lobos inferiores. Esses pacientes comumente apresentam dispneia grave e baixa DLCO, não obstante os volumes pulmonares relativamente preservados em exames espirométricos. Hipertensão arterial pulmonar de moderada a grave e aumento da incidência de câncer de pulmão também são comuns nesses pacientes. Infelizmente, ainda não existe um tratamento eficaz para a CFPE. O objetivo desta revisão é discutir o que se sabe atualmente a respeito da patogênese, das características clínicas e dos fatores prognósticos da CFPE. Como a maioria dos dados publicados baseia-se em análise retrospectiva, são necessários mais estudos sobre o papel do enfisema e seus subtipos, a progressão da fibrose/enfisema e sua correlação com a inflamação, as opções de tratamento e o prognóstico em pacientes com CFPE.

Silicose: mecanismos de atuação da partícula de sílica no tecido pulmonar / Silicosis: mechanisms of action of silica particle in lung tissue

A inalação da partícula de sílica leva a um processo inflamatório exacerbado, com formação de granulomas e deposição de colágeno no parênquima pulmonar. A silicose é uma doença fibrosante, progressiva e irreversível, que leva o indivíduo a óbito devido a falência respiratória. Existem diversos mecanismos pelos quais a partícula de sílica exerce sua toxicidade, causando lesão no tecido pulmonar, sendo os principais descritos: a citotoxicidade direta, ativação de geração de oxidantes, estimulação da secreção de citocinas e quimiocinas, estimulação da secreção de fatores fibrogênicos e morte celular por apoptose. Embora a silicose seja passível de prevenção, não há um tratamento eficiente para minimizar os efeitos deletérios da mesma. Esta revisão elucida os principais mecanismos pelos quais a silicose se desenvolve, com o intuito de elaborar uma terapia eficaz.

Inhalation of silica particles leads to an exacerbated inflammatory process with formation of granulomas and collagen deposition in the lung parenchyma. Silicosis is a fibrosing disease, progressive and irreversible, leading the individual died due to respiratory failure. There are several mechanisms by which the particle silica exerts its toxicity, causing injury to the lung tissue, and the principal described: a direct cytotoxicity, activation of oxidant generation, stimulation of secretion of cytokines and chemokines, stimulating the secretion of fibrogenic factors and cell death by apoptosis. Although silicosis is preventable, no effective treatment to minimize the deleterious effects of same. This review elucidates the major mechanisms by which silicosis develops, in order to develop an effective therapy

Fibrosis pulmonar asociada a vasculitis con anticuerpos anticitoplasmáticos positivos / Pulmonary fibrosis associated with anti-neutrophil cytoplasmic antibody-positive vasculitis

Las complicaciones pulmonares más conocidas de las vasculitis con anticuerpos anticitoplasmáticos de los neutrófilos (ANCA) positivos (VAA), son la hemorragia alveolar, los granulomas y la estenosis de la vía aérea. En los últimos años han aparecido algunos informes aislados que muestran la asociación con fibrosis pulmonar (FP), sugiriendo que ésta sería otra complicación de las VAA. En este trabajo informamos dos casos con dicha asociación describiendo sus características clínicas, tomográficas e inmunológicas. Dado que en la asociación de FP y VAA notificada en los últimos años, la FP puede ser su primera manifestación, podría ser necesaria la búsqueda de ANCA en pacientes con FP, como causa de la misma y por el posible desarrollo posterior de vasculitis.

The most frequently observed pulmonary complications of vasculitis (AAV) with anti-neutrophil cytoplasmic positive antibodies (ANCA) are alveolar hemorrhage, granulomas and airway stenosis. In recent years, some reports have been published that show the association of vasculitis with pulmonary fibrosis (PF), suggesting that it may be another complication of AAV. We report and describe here two cases with such association, and their clinical, tomographic and immunological characteristics. Given that in the association between PF and AAV, as reported in the last years, PF could be the first manifestation of AAV, the search for ANCA in patients with PF may be necessary, as a cause of it and for the possible subsequent development of vasculitis.

Intoxicación pediátrica por paraquat (gramoxone) supervivencia en dos casos / Pediatric intoxication with paraquat (gramoxone) survival of two children

El paraquat es un plaguicida agricola bipiridilo, cuyos efectos tóxicos más severos son año hepato-renal y fibrosis pulmonar irreversible. Se reportan dos casos de intoxicación en niños, por ingestión accidental de paraquat; ellos desarrollaron sialorrea, vómitos azul verdoso, lesión digestiva cáustica. dificultad respiratoria, daño hepato-renal, alteraciones radiológicas y presencia de paraquat en orina y sangre. Ambos pacientes recibieron el tratamiento disponible entre 24-48 horas despues de la ingestion y tuvieron una evolución satisfactoria. El tratamiento consistió basicamente en diálisis gastrointestinal, diuresis forzada ácida, N-acetilcisteína y dexametasona.

Paraquat is a bipyridyl agricultural pesticide, whose more severe effects are hepatic-renal damage and irreversible pulmonary fibrosis. Two children with paraquat accidental poisoning are reported; they developed syalorrhea, blue greenish vomiting, caustic digestive injury, breathing difficulty, liver and kidney damage, radiographic changes and presence of paraquat in blood and urine. Both patients received the available treatment 24-48 hours after ingetion and had a satisfactory autcome. The consisted basically of gatrointestinal dialysis, acidic forced diuresis, N-acetylcysteine and dexamethasone.

Evolución funcional respiratoria en dos pacientes con enfisema y fibrosis pulmonar / Functional respiratory evolution in two patients with emphysema and pulmonary fibrosis

La combinación de enfisema y fibrosis pulmonar es una condición frecuentemente subdiagnosticada. Los estudios funcionales aislados pueden generar interpretaciones inadecuadas. No hemos hallado comunicaciones de casos que documenten la evolución espirométrica de estos pacientes. Se presentan dos casos de fibrosis y enfisema combinados que permiten observar la evolución funcional a largo plazo y comprender los valores espirométricos actuales en forma más precisa. Los hallazgos más relevantes son: 1) espirometría con discretas alteraciones funcionales en presencia de disnea marcada y, en un paciente, necesidad de oxigenoterapia crónica, 2) evolución funcional con seudonormalización del patrón espirométrico obstructivo inicial, posiblemente como consecuencia del desarrollo de fibrosis. Un defecto obstructivo leve en un paciente con diagnóstico de obstrucción crónica al flujo aéreo y marcado deterioro de su condición clínica y clase funcional debería alertar sobre la posibilidad de fibrosis pulmonar asociada. La tomografía y la disponibilidad de estudios funcionales previos permiten comprender esta condición.

Combined pulmonary fibrosis and emphysema (CPFE) is a frequently under-diagnosed condition. Isolated pulmonary function tests (PFT) can give rise to misinterpretations. We have found no reports on these patients’ spirometric progression. We describe two cases of CPFE, showing long-term functional evolution to have a more accurate understanding of current spirometric values. The most relevant findings are: 1) spirometry with discrete functional alterations in the presence of a marked dyspnea and the need, in one patient, for chronic oxygen therapy; and 2) functional evolution reflecting "pseudonormalisation" of the initial obstructive spirometric pattern, possibly as a result of fibrosis development. A mild obstructive defect in a patient with chronic airflow limitation and marked impairment of his/her clinical status and functional class should alert on the possibility of associated pulmonary fibrosis. A computed tomography (CT) and previous PFTs will allow a better understanding of this condition.

Noninvasive estimation of clinically asymptomatic pulmonary hypertension in idiopathic pulmonary fibrosis.

OBJECTIVES: To determine the prevalence of pulmonary hypertension in patients with idiopathic pulmonary fibrosis (IPF) by noninvasive methods. PATIENTS AND METHODS: Twenty seven patients with IPF attending the Chest Clinic over a period of one-and-a-half-years underwent echocardiography for evidence of pulmonary hypertension, which was defined as pulmonary artery systolic pressure > or = 40 mmHg by Doppler echocardiography, or pulmonary acceleration time < or = 100 milliseconds or two-dimensional echocardiographic findings of right ventricular hypertrophy or overload. RESULTS: Two patients with clinical evidence of pulmonary hypertension were excluded from analysis. Their mean age (n = 25) was 53.8 years. The mean duration of symptoms before presentation was 2.1 years. Pulmonary hypertension was diagnosed by echocardiography in nine of the 25 patients (36%). There was a statistically significant difference between the duration of illness, arterial oxygen tension (PaO2) levels and forced vital capacity in patients with pulmonary hypertension than in those without pulmonary hypertension. Logistic regression analysis showed a significant association between the duration of illness, decreasing forced vital capacity and hypoxemia with the development of pulmonary hypertension. CONCLUSIONS: Echocardiography detects pulmonary hypertension in clinically asymptomatic individuals, and should be used routinely for the diagnosis of pulmonary hypertension in patients with IPF.

Insuficiencia respiratoria progresiva secundaria a fibrosis pulmonar en una escolar: Caso clínico / Pulmonary fibrosis in childhood: Report of one case

Interstitial lung diseases are uncommon in children, and can be idiopathic or secondary to known causes, sharing common pathological findings. We report a girl with progressive respiratory insufficiency secondary to interstitial pneumonia and pulmonary fibrosis, with risk factors such as bronchopulmonary dysplasia and respiratory infections (respiratory syncytial virus and suspected Mycoplasma pneumoniae), that may have had an additive effect. Nasal bi-level Positive Airway Pressure was used in the last period of her disease. She died due to global respiratory failure at the age of 14 years.

Influence of colchicine on pulmonary silicotic fibrogenesis in rats.

With an aim to evaluate the antifibrotic action of colchicine in experimental model of pulmonary silicosis, the effect of colchicine on developing and developed pulmonary silicosis induced by quartz was studied in rats in vivo and on alveolar macrophages exposed to quartz particulates in vitro. A progressive increase in wet and dry weight of lungs exposed to quartz dust alone, and quartz dust and colchicine injected orally was investigated. An increase in collagen contents, with lapse in time, in animals exposed intratracheally to quartz dust, or exposed similarly to quartz dust but receiving colchicine simultaneously through oral route was observed. A blindfold evaluation of histological sections of lungs of silicotic animals with or without colchicine administration during development of lesions did not reveal any difference between two groups of silicotic rats. Administration of colchicine for 4 weeks after the lesions were developed neither inhibited nor retarded the laying down of collagen. The studies were extended to investigate the effect of colchicine on quartz-induced alveolar macrophage cytotoxicity. The presence of varying concentrations of colchicine in the culture medium did not significantly alter cytotoxic potential of quartz. The results reveal that colchicine administration during the development of and on developed silicosis does not significantly alter pathogenesis of silicotic lesions. At the cellular level colchicine does not modulate quartz-induced alveolar macrophage cytotoxicity, believed to be a significant event for the onset of pulmonary silicotic fibrogenesis.

Upper lobe fibrosis in ulcerative colitis.

We report a case of ulcerative colitis where chest radiograph and high-resolution computed tomography (HRCT) of the lungs revealed bilateral upper lobe fibrosis, which was misdiagnosed and treated as pulmonary tuberculosis. The diagnosis of ulcerative colitis was confirmed by sigmoidoscopy and rectal biopsy.

Successful outcome of pregnancy in idiopathic pulmonary fibrosis.

Idiopathic pulmonary fibrosis is typically seen in older individuals. Occurrence of pregnancy in idiopathic pulmonary fibrosis is uncommon and ends with abortion in case it happens. Here we report a case of idiopathic pulmonary fibrosis presenting at 38 weeks of pregnancy who successfully delivered a male child without any significant complications. The disease was active at presentation and early postpartum period.

Bronchoalveolar lavage (BAL) in newly diagnosed patients with collagen vascular diseases.

Collagen vascular diseases (CVD) are commonly associated with interstitial lung diseases. Bronchoalveolar lavage (BAL) fluid analysis has important diagnostic value when considered in conjunction with other information. The present study was undertaken in newly diagnosed patients with systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA) at presentation to characterise BAL cellular constituents and elucidate the cellular picture in patients with and without pulmonary symptoms and in those with and without radiological (high resolution computed tomography) features of interstitial lung disease. All the patients were non-smokers and had not received any form of treatment for their diseases. The means of percentages of lymphocytes, neutrophils, and macrophages were 23.3%, 6.2%, 70.5% respectively. There was a significant BAL lymphocyte predominance in patients with pulmonary symptoms, and a lymphocyte and neutrophil predominance in those having radiological evidence of interstitial lung disease.

Trasplante pulmonar: experiencia de un grupo quirúrgico / Lung transplantation: experience of a surgical team

Se informan los resultados de un programa de trasplante pulmonar en el Instituto Mexicano del Seguro Social y los requerimientos para obtener mejores resultados. El estudio se realizó en el Hospital General del Centro Médico Nacional La Raza y Hospital de Cardiología del Centro Médico Nacional Siglo XXI. Se incluyeron tres pacientes del sexo femenino con fibrosis intersticial difusa en estado terminal en lista de espera de trasplante unilateral de pulmón, que reunieron los criterios de inclusión. Entre julio de 1992 y noviembre de 1995, se efectuaron dos trasplantes unilaterales de pulmón en el Instituto Mexicano del Seguro Social provenientes de donaciones múltiples, y un trasplante de lóbulo pulmonar a partir de donador vivo no relacionado. El primer procedimiento se efectuó sin accidentes transoperatorios, pero la paciente falleció a las siete horas de operada por edema pulmonar unilateral de etiología multicausal. En el segundo caso, la paciente falleció en el transoperatorio por hemorragia de la anastomosis vascular por alteraciones de la pared de la rama derecha de su arteria pulmonar. El tercer caso evoluciona satisfactoriamente. En trasplantes, la falta de donadores es el principal problema.

Enfermedades pulmonares intersticiales: aproximación diagnóstica / Interstitial lung disease: diagnosis approach

Las enfermedades pulmonares intersticiales constituyen un conjunto muy heterogéneo, vasto y complejo de afecciones de muy diversas etiologías que causan disrrupción de las estructuras alveolares y se manifiestan por disnea e hipoxemia de ejercicio; alteración ventilatoria restrictiva e infiltrados pulmonares difusos. Las causas de enfermedad pulmonar intersticial son alrededor de 160, pero el 30 al 40 porciento de los casos corresponden a fibrosis pulmonar idiopática. El diagnóstico de las enfermedades pulmonares intersticiales descansa sobre las siguientes bases: Clínicas, sintomatología y semiología; radiología de tórax; tomografía computada; lavado broncoalveolar; biopsia pulmonar transparietal, transbronquial, por toracoscopía o quirúrgica. El pronóstico de la fibrosis pulmonar idiopática es malo y los resultados terapéuticos son pobres (25 porciento). En todos los pacientes hay que descartar la eventual participación de medicamentos y la exposición a riesgos profesionales

Insuficiencia renal y respiratoria en una embarazada con esclerosis sistémica progresiva: caso anatomo-clínico / Renal and respiratory failure in a pregnant woman with progressive systemic sclerosis: anatomical clinical case

Pregnancy in patients with systemic sclerosis may predispose to a fast progression of the disease. We report a woman with systemic sclerosis and a multiple visceral involvement that during the third trimester of pregnancy developed a respiratory failure caused by interstitial fibrosis complicated with alveolar hemorrhage, bronchopneumonia and respiratory distress syndrome and a renal failure. On admission, she was subjected to a cesarean section, delivering a 1205 g newborn. In spite of intensive care support, the multisystemic failure became unmanageable and the patient died 15 days after admission. Literature review, although sometimes controversial, indicates that pregnancy is a situation with definitive death risk for patients with systemic sclerosis, that requires a close follow up

Pneumonite alérgica extrínseca: relato de um caso / Extrinsic allergic pneumonitis: a case report

Os autores apresentam o caso de um paciente de 28 anos de idade, sexo masculino, que iniciou com o quadro de dispnéia, tosse seca e desconforto respiratório. Apresentava radiografia de tórax com um infliltrado intersticial difuso. Na primeira internaçäo houve regressäo espontânea dos sintomas, näo sendo possível realizar o diagnóstico. Cerca de um mês após, o paciente reinternou com os mesmos sintomas e quadro radiológico semelhante. A espirometria mostrou padräo restritivo, sendo entäo o paciente submetido a fibrobroncoscopia com biópsia transbrônquica e, posteriormente, biópsia a céu aberto. Após revisäo da história e o resultado da biópsia, foi confirmado o diagnóstico de pneumonite alérgica extrínseca. Os autores discutem os aspectos etiopatogênicos desta doença e seu diagnóstico

Tratamiento de la alteración funcional respiratoria en la esclerosis generalizada progresiva con D-penicilamina y colchicina

La alteración funcional respiratoria (AFR) es una causa importante de morbi-mortalidad en la esclerosis generalizada progresiva (EGP) y no contamos con tratamiento (tx) satisfactorio en la actualidad. Se incluyeron 10 pacientes con EGP según criterios del ACR y cuya gasometría arterial dinámica (GAD) mostraba alteración en la difusión; se excluyeron otras neumopatías, cardiopatías, hipertensión pulmonar y nefropatías. Se midió expansión torácica (ET), distancia dedo-pliegue muñeca (DDMP) y contracturas en flexión (CF) para evaluar la progresión en piel. Se administraron 450 mg/día de D-penicilamina (DPO) y 1 mg/día de Colchicina durante 26 meses promedio (7-37 m). (C). Se evaluaron con espirometría cada 3 meses y GAD y radiografía de tórax al inicio y al final del estudio. Para el análisis estadístico se utilizó t de student y prueba de Fisher. Fueron 9M y 1H con edades (28-68,x 48 años), evaluación 6-72,x 28 meses), ninguno era fumador, todos tuvieron ET normal y restricción en la espirometría que mejoró con el tx. (p = 0.006), así como la hipoxemia (p = 0.007), que fue más significativo en los pacientes con evolución menor de 2 años. No hubo progresión radiológica, ni mejoría significativa o correlación con otras alteraciones clínicas. Nuestro estudio sugiere utilidad de estos medicamentos en la AFR en la EGP, especialmente cuando se utiliza en forma temprana y a largo plazo

Lymphocyte alveolitis in HAM/TSP patients: preliminary report

Mielopatia por HTLV-I (HAM) tem sido descrita como doença sistêmica caracterizada pelo acometimento de vários órgäos além do sistema nervoso. Neste registro, estamos relatando o envolvimento pulmonar em pacientes com HAM