Manifestaciones pulmonares al diagnóstico de 170 pacientes con vasculitis asociadas a anticuerpos anticitoplasma de neutrófilos: cohorte retrospectiva de un centro universitario / Pulmonary manifestations at the diagnosis of 170 patients with vasculitis associated with anticytoplasmic neutrophil antibodies: retrospective cohort of a university center

INTRODUCCIÓN: Las Vasculitis Asociadas a Anticuerpos Anticitoplasma de Neutrófilos (VAA) son infrecuentes, pero de amplio espectro de presentación. Si bien el consenso de clasificación de Chapel Hill del año 2012, sigue vigente, la tendencia actual es clasificarlas de acuerdo al marcador inmunológico: anti-Proteinasa 3 (PR3) o anti-mieloperoxidasa (MPO). Las manifestaciones pulmonares clásicas son la hemorragia alveolar y los nódulos pulmonares. En los últimos 10 años se ha descrito la enfermedad pulmonar difusa (EPD). Los estudios epidemiológicos son escasos, y suelen representar en su mayoría poblaciones norteamericanas o europeas. El objetivo es describir las características del compromiso pulmonar al debut en VAA en un centro universitario. PACIENTES Y MÉTODO: De un total de 190 pacientes con diagnóstico de VAA se incluyeron 170 en seguimiento en nuestro centro. Se revisaron aspectos clínicos, demográficos, laboratorio e imagenológicos de los pacientes incluidos. RESULTADOS: De los 170 pacientes, 112 (65,88%) presentaron compromiso pulmonar. 106 (94,64%) de los pacientes fueron anticuerpos anti citoplasma de neutrófilos (ANCA) positivos; de estos, 56 (53,27%) MPO (+) y 39 (36,45%) PR-3 (+). Un tercio de los pacientes de ambos grupos presentó hemorragia alveolar. En los pacientes MPO (+) predomina la EPD (53,5%) y en PR-3 (+) los nódulos pulmonares (69,23%). Destaca la baja frecuencia de patología obstructiva asociada. CONCLUSIONES: El compromiso pulmonar en las VAA es prevalente y heterogéneo. En nuestra serie, destaca la frecuencia de EPD en VAA MPO (+), lo que releva la importancia del estudio con ANCA en paciente con diagnóstico y seguimiento por EPD.

INTRODUCTION: Antineutrophil Cytoplasmic Antibodies (ANCA) associated vasculitis (AAV) are uncommon, but of broad spectrum of presentation. Although the 2012 Chapel Hill classification consensus remains valid, the current trend is to classify them according to the immunological marker: anti-Proteinase 3 antibody (PR-3) or anti-Myeloperoxidase antibody (MPO). The classic pulmonary manifestations are alveolar hemorrhage and pulmonary nodules. Interstitial lung disease (ILD) has been described in the last 10 years. Epidemiological studies are scarce, and they usually represent mostly North American or European populations. The objective is to describe the characteristics of lung involvement upon debut in AAV in a university center. PATIENTS AND METHODS: Of a total of 190 patients diagnosed with AAV, 170 were included in follow-up at our center. Clinical, demographic, laboratory and imaging aspects of the included patients were reviewed. RESULTS: Of the 170 patients, 112 (65.88%) had lung involvement. 106 (94.64%) of the patients were ANCA (+); of these, 56 (53.27%) MPO (+) and 39 (36.45%) PR-3 (+). One third of the patients in both groups had alveolar hemorrhage. In MPO (+) patients, ILD predominates (53.5%) and in PR-3 (+) pulmonary nodules (69.23%). The low frequency of associated obstructive pathology stands out. CONCLUSIONS: Pulmonary manifestations in AAVs are frequent and heterogeneous. Locally, the association of ILD and AAV MPO (+) stands out, which highlights the importance of ANCA study in patients with diagnosis and follow-up by ILD.

Prevalencia y letalidad de enfermedades pulmonares intersticiales en la Región de Los Ríos, Chile / Prevalence and lethality of interstitial pulmonary diseases in the Region of Los Ríos, Chile

BACKGROUND: Idiopathic Pulmonary Fibrosis (IPF) is a subtype of interstitial lung disease (ILD) that significantly affects people's quality of life, whose prevalence and mortality has increased. AIM: To determine the prevalence and lethality of ILD in the Los Ríos Region between the years 2018 and 2019. MATERIAL AND METHODS: Review of a database of all patients attended at an outpatient clinic of the regional hospital. Those patients with the diagnosis of ILD and seen between 2018 and 2019 were selected. Mortality and its causes were verified with death certificates. RESULTS: In the study period, 339 cases with ILD, aged 71 ± 10 years (64% women) were identified. The calculated ILD prevalence was 84 cases per 100,000 inhabitants, with a higher predominancy in Futrono and Paillaco communes. IPF and Connective Tissue Diseases were the predominant subtypes. Overall lethality was 18%, with more deaths among patients with IPF (n = 31). CONCLUSIONS: In the Los Ríos Region, ILDs have a relevant prevalence and lethality.

Prevalence of latent tuberculosis infection among patients with interstitial lung disease requiring immunosuppression / Prevalência de infecção latente por tuberculose em pacientes com doença pulmonar intersticial com necessidade de imunossupressão

ABSTRACT Objective To characterize the prevalence of latent tuberculosis infection (LTBI) in patients with interstitial lung diseases (ILDs) requiring immunosuppression. Only 5 to 10% of individuals infected with Mycobacterium tuberculosis develop tuberculosis, and certain groups of patients have an increased risk of illness, such as the immunocompromised. Patients with ILDs are frequently treated with immunosuppressants and, therefore, might have a higher risk of developing the disease. Methods Prospective study conducted at the ILD reference center of the Federal University of Paraná from January 2019 to December 2020. The screening of LTBI was performed with the use of the tuberculin skin test (TST). Results The sample consisted of 88 patients, of whom 64.8% were women, with a mean age of 61.4 years. The most frequent diagnoses were autoimmune rheumatic disease ILD (38.6%) and hypersensitivity pneumonitis (35.2%). The most common immunosuppressant in use at the time of the TST was prednisone, either in combination with mycophenolate (19.3%) or alone (17.1%). The majority of participants had fibrotic lung disease, characterized by a reticular interstitial pattern on chest computed tomography (79.5%) and moderate to severe functional impairment (mean FVC 69.2%). A prevalence of LTBI of 9.1% (CI 95%, 2.1%-15.1%) was found, with a TST median of 13. Conclusion Patients with ILD who are treated with immunosuppressants are not commonly screened for LTBI, despite being under a greater risk of progression to active disease. This study suggests the need for a more cautious approach to these patients.

RESUMO Objetivo Caracterizar a prevalência de Infecção Latente por Tuberculose (ILTB) em pacientes com Doenças Pulmonares Intersticiais (DPIs) que necessitam de imunossupressão. Apenas 5 a 10% dos indivíduos infectados pelo Mycobacterium tuberculosis desenvolvem tuberculose, sendo que certos grupos de pacientes apresentam maior risco de doença, tais como os imunocomprometidos. Pacientes com DPIs são frequentemente tratados com imunossupressores, portanto, podem apresentar maior risco de desenvolver a doença. Métodos Estudo prospectivo conduzido no Centro de Referência para DPI da Universidade Federal do Paraná (UFPR), entre Janeiro de 2019 e Dezembro de 2020. O rastreio de ILTB foi realizado por meio da Prova Tuberculínica (PT). Resultados A amostra foi composta por 88 pacientes, dos quais 64,8% eram mulheres, com, em média, 61,4 anos de idade. Os diagnósticos mais frequentes foram DPI associada a doença reumática autoimune (DRAI) (38,6%) e pneumonite de hipersensibilidade (35,2%). Prednisona foi o imunossupressor mais comumente utilizado à época da PT, em combinação com micofenolato (19,3%) ou isoladamente (17,1%). A maioria dos participantes tinha doença pulmonar fibrótica, caracterizada por infiltrado reticular em tomografia computadorizada de tórax (79,5%), bem como comprometimento funcional moderado a grave (Capacidade Vital Forçada (CVF) média de 69,2%). Observou-se uma prevalência de ILTB de 9,1% (Intervalo de Confiança (IC) 95%, 2,1%-15,1%), com mediana da PT de 13. Conclusão Não é comum que pacientes com DPI tratados com imunossupressores sejam avaliados quanto à presença de ILTB, apesar de estarem sob um maior risco de progressão para doença ativa. Este estudo sugeriu a necessidade de uma abordagem mais cuidadosa em relação a esses pacientes.

Padrões de tomografia computadorizada de alta resolução na doença pulmonar intersticial (DPI): prevalência e prognóstico / High resolution computed tomography patterns in interstitial lung disease (ILD): prevalence and prognosis

RESUMO Objetivo Correlacionar a prevalência e o prognóstico de cada padrão de TCAR de pneumonia intersticial usual (PIU) típica, provável e indeterminada com o diagnóstico clínico multidisciplinar de doença pulmonar intersticial (DPI). Métodos Incluímos todos os pacientes com diagnóstico multidisciplinar de DPI com padrão de TCAR de PIU típica, PIU provável ou indeterminada para PIU. Dados clínicos e histopatológicos, teste de função pulmonar e status de sobrevida foram obtidos retrospectivamente. O diagnóstico final foi validado por uma equipe multidisciplinar. Resultados Foram incluídos no estudo 244 pacientes, com média de idade de 68 ± 13 anos sendo 52,5% do sexo masculino. Em um total de 106 pacientes com padrão típico de PIU, 62% tiveram o diagnóstico multidisciplinar de FPI, 20% de pneumonia por hipersensibilidade crônica (PHC) e 10% de DPI relacionada à doença do tecido conjuntivo (DPI-DTC). Dos 114 casos com provável PIU, DPI-DTC correspondeu a 39%, FPI a 31%, pneumonia intersticial descamativa a 11%, doença pulmonar relacionada a medicamentos a 9% e PHC a 8%. Nos 24 pacientes com TC indeterminada para PIU, o DPI-DTC foi o diagnóstico final em 33%, seguido por pneumonia intersticial descamativa (21%) e FPI (13%). Pacientes com PIU típica apresentaram maior probabilidade de morrer ou realizar transplante de pulmão no seguimento (17,9% e 11,3%, respectivamente). Conclusões FPI, PHC e DPI-DTC foram os principais diagnósticos diferenciais em pacientes com padrão de TCAR de PIU típica, provável e indeterminada. Pacientes com padrão de PIU típico na TCAR tiveram maior probabilidade de morrer ou realizar transplante de pulmão no seguimento.

ABSTRACT Objective To correlate the prevalence and prognosis of each HRCT pattern of typical, probable, and indeterminate usual interstitial pneumonia (UIP) with the clinical multidisciplinary diagnosis of interstitial lung disease (ILD). Methods We included all patients with a multidisciplinary diagnosis of ILD with an HRCT pattern of typical UIP, probable UIP, or indeterminate for UIP. Clinical and histopathological data, pulmonary function tests, and survival status were retrospectively obtained. The final diagnosis was validated by a multidisciplinary team. Results A total of 244 patients were included in the study, with a mean age of 68 ±13 years and being 52.5% males. In a total of 106 patients with typical UIP pattern, 62% had the multidisciplinary diagnosis of IPF, 20% had chronic hypersensitivity pneumonitis (CHP), and 10% had connective tissue disease-related ILD (CTD-ILD). Out of the 114 cases with probable UIP, CTD-ILD corresponded to 39%, IPF to 31%, desquamative interstitial pneumonia to 11%, drug-related lung disease to 9%, and CHP to 8%. In the 24 patients with CT indeterminate for UIP, CTD-ILD was the final diagnosis in 33%, followed by desquamative interstitial pneumonia (21%), and IPF (13%). Patients with typical UIP were more likely to die or had lung transplantation in the follow-up (17.9% and 11.3%, respectively). Conclusion IPF, CHP, and CTD-ILD were the main differential diagnoses in patients with HRCT patterns of typical, probable and indeterminate UIP. Patients with HRCT typical UIP pattern were more likely to die or had lung transplantation in the follow-up.

Familial pulmonary fibrosis: a heterogeneous spectrum of presentations / Fibrose pulmonar familiar: um espectro heterogêneo de apresentações

ABSTRACT Objective: To describe the clinical, functional, and radiological features of index cases of familial pulmonary fibrosis (FPF) in Brazil. Methods: We evaluated 35 patients with FPF - of whom 18 (51.4%) were women - with a median age of 66.0 years (range, 35.5-89.3 years). All of the patients completed a standardized questionnaire, as well as undergoing pulmonary function tests and HRCT of the chest. In 6 cases, lung tissue samples were obtained: from surgical biopsies in 5 cases; and from an autopsy in 1 case. Results: A history of smoking and a history of exposure to birds or mold were reported in 45.7% and 80.0% of the cases, respectively. Cough and marked dyspnea were reported by 62.8% and 48.6% of the patients, respectively. Fine crackles were detected in 91.4% of the patients. In 4 patients, the findings were suspicious for telomere disease. The median FVC and DLCO, as percentages of the predicted values, were 64.9% (range, 48.8-105.7%) and 38.9% (range, 16.0-60.0%), respectively. Nine patients had reduced DLCO despite having normal spirometry results. Regarding HRCT, patterns typical of usual interstitial pneumonia were found in 6 patients (17.1%). In 25 cases (71.5%), the HRCT features were consistent with a diagnosis other than idiopathic pulmonary fibrosis. In 11 cases (31.4%), the radiological patterns were uncharacteristic of interstitial lung disease. Of the six lung tissue samples analyzed, four showed interstitial pneumonia with bronchiolocentric accentuation, and, on the basis of the clinical and radiological data, the corresponding patients were diagnosed with hypersensitivity pneumonitis. Conclusions: Patients with FPF can present with a wide variety of clinical features. Most HRCT scans of these patients exhibit patterns not typical of usual interstitial pneumonia. The family history of fibrotic lung diseases should be investigated in all patients under suspicion, regardless of their age.

RESUMO Objetivo: Descrever as características clínicas, funcionais e radiológicas de um grupo de casos índice diagnosticados com fibrose pulmonar familiar (FPF) no Brasil. Métodos: Trinta e cinco pacientes com FPF (18 mulheres; 51,4%), com mediana de idade de 66,0 anos (variação: 35,5-89,3 anos), responderam a um questionário padronizado e foram submetidos a testes de função pulmonar e TCAR de tórax. Tecido pulmonar foi obtido para revisão em 6 casos: a partir de biópsias cirúrgicas em 5 e de autópsia em 1. Resultados: Antecedentes de tabagismo e de exposição a aves ou mofo foram referidos por 45,7% e 80,0% dos casos, respectivamente. Tosse e dispneia significante foram referidas por 62,8% e 48,6% dos pacientes, respectivamente. Estertores finos foram detectados em 91,4% dos indivíduos. Em 4 pacientes, os achados levantaram suspeitas de doença dos telômeros. As medianas da CVF e da DLCO foram, respectivamente, de 64,9% (variação: 48,8-105,7%) e 38,9% (variação: 16,0-60,0%) em porcentagem dos valores previstos. Apesar de espirometria normal, 9 pacientes exibiram DLCO reduzida. Em relação às TCAR, padrões típicos de pneumonia intersticial usual foram encontrados em 6 pacientes (17,1%). Em 25 casos (71,5%) os achados tomográficos foram mais consistentes com um diagnóstico de não relacionado a fibrose pulmonar idiopática. Em 11 pacientes (31,4%) o padrão radiológico foi incaracterístico para doença pulmonar intersticial. Das seis amostras de tecido pulmonar analisadas, quatro mostraram pneumonias intersticiais com acentuação bronquiolocêntrica e, em função de outros dados clínicos e radiológicos, pneumonite de hipersensibilidade foi diagnosticada. Conclusões: Pacientes com FPF podem apresentar características clínicas diversas. A maioria das TCAR desses pacientes exibe padrões não típicos de pneumonia intersticial usual. A pesquisa da história clínica de outros casos de pneumopatias fibrosantes na família deve ser feita em todos os pacientes em investigação, independentemente da idade.

Cánula nasal de alto flujo en lactantes: experiencia en una unidad de paciente crítico / High flow nasal cannula in infants: Experience in a critical patient unit

Introducción: La cánula nasal de alto flujo (CNAF) es un método de soporte respiratorio cada vez más utilizado en pediatría por sus resultados y seguridad. Objetivo: Determinar la efectividad de la CNAF, evaluar factores asociados a fracaso y complicaciones relacionadas con su uso en lactantes. Pacientes y método: Se analizaron los datos demográficos, clínicos, gasométricos, radiológicos y complicaciones de los pacientes conectados a CNAF en una unidad crítica entre junio de 2012 y septiembre de 2014. Se compararon los pacientes que fracasaron con los respondedores a CNAF, considerándose fracaso la necesidad de un mayor soporte respiratorio durante las primeras 48 h de conexión. Se utilizó test de Kolmogorov Smirnov, U de Mann-Whitney, Chi cuadrado, test exacto de Fisher, correlaciones y Modelo de regresión logística binaria para p ≤ 0,05. Resultados: Un total de 109 pacientes. Mediana de edad y peso: 1 mes (0,2-20 meses) y 3,7 kg (2-10 kg); percentil 95: 3,7 meses y 5,7 kg respectivamente. El diagnóstico y patrón radiológico más frecuente fue bronquiolitis (53,2%) e infiltrado intersticial (56%). Un 70,6% respondió. Hubo diferencia significativa entre fracaso y respuesta en el diagnóstico (p = 0,013), radiografía (p = 0,018), contexto de conexión (p < 0,0001), pCO2 (mediana 40,7 mm Hg [15,4-67 mm Hg] versus 47,3 mm Hg [28,6-71,3 mm Hg], p = 0,004) y horas de CNAF (mediana 60,75 h [5-621,5 h] versus 10,5 h [1-29 h], p < 0,0001). El OR de PCO2 ≥ 55 mm Hg para fracaso fue 2,97 (IC 95%: 1,08-8,17; p = 0,035). Ningún paciente falleció ni registró complicaciones. Conclusión: El porcentaje de éxito observado fue similar a lo publicado. En esta muestra el fracaso de CNAF solo se asoció a una pCO2 inicial ≥ 55 mm Hg. Su uso se consideró seguro al no reportarse complicaciones relacionadas a su utilización. Se requiere de un estudio multicéntrico, aleatorizado y controlado para contrastar estos resultados.

Introduction: The high flow nasal cannula (HFNC) is a method of respiratory support that is increasingly being used in paediatrics due to its results and safety. Objective: To determine the efficacy of HFNC, as well as to evaluate the factors related to its failure and complications associated with its use in infants. Patients and method: An analysis was performed on the demographic, clinical, blood gas, and radiological data, as well as the complications of patients connected to a HFNC in a critical care unit between June 2012 and September 2014. A comparison was made between the patients who failed and those who responded to HFNC. A failure was considered as the need for further respiratory support during the first 48 hours of connection. The Kolmogorov Smirnov, Mann-Whitney U, chi squared and the Exact Fisher test were used, as well as correlations and a binary logistic regression model for P ≤ .05. Results: The study included 109 patients, with a median age and weight: 1 month (0.2-20 months) and 3.7 kg (2-10 kg); 95 percentile: 3.7 months and 5.7 kg, respectively. The most frequent diagnosis and radiological pattern was bronchiolitis (53.2%) and interstitial infiltration (56%). Around 70.6% responded. There was a significant difference between failure and response in the diagnosis (P = .013), radiography (P = 018), connection context (P < .0001), pCO2 (median 40.7 mmHg [15.4-67 mmHg] versus 47.3 mmHg [28.6-71.3 mmHg], P = .004) and hours on HFNC (median 60.75 hrs [5-621.5 hrs] versus 10.5 hrs [1-29 hrs], P < .0001). The OR of the PCO2 ≥ 55 mmHg for failure was 2.97 (95% CI; 1.08-8.17; P = .035). No patient died and no complications were recorded. Conclusion: The percentage success observed was similar to that published. In this sample, the failure of HFNC was only associated with an initial pCO2 ≥ 55 mmHg. On there being no complications reported as regards it use, it is considered safe, although a randomised, controlled, multicentre study is required to compare and contrast these results.

Enfermedades pulmonares intersticiales en el niño / Interstitial pulmonary diseases seen in the child

Las enfermedades pulmonares intersticiales en el niño, comprenden un gran y heterogéneo grupo de raras enfermedades difusas pulmonares de morbilidad variada. Se caracterizan por cambios inflamatorios y fibróticos que causan remodelación de las paredes alveolares y de las vías respiratorias distales, y ocasionan un defecto restrictivo funcional y una alteración en el intercambio gaseoso, con hipoxemia progresiva. Son muchas las enfermedades que pueden afectar al intersticio pulmonar y múltiples las formas etiológicas causadas por una variedad de procesos patológicos, que incluyen, factores genéticos, asociación a enfermedades sistémicas, así como a respuestas inflamatorias o fibróticas a diferentes estímulos. Sin embargo, algunas veces su origen es desconocido, y se catalogan entonces como idiopáticas. Los neumólogos pediátricos han tratado de clasificar los casos de las enfermedades pulmonares intersticiales en las diferentes categorías descritas originalmente en adultos, pero, en realidad, existen formas del adulto que no se observan en la infancia, y formas únicas de presentación pediátrica. Se señala la importancia del conocimiento de estas entidades, particularmente las de origen desconocido o idiopáticas.

The interstitial pulmonary diseases seen in the child comprise a large heterogeneous group of rare diffuse pulmonary diseases of varied morbidity. They are characterized by inflammatory and fibrotic changes causing remodeling of alveolar walls and of the distal respiratory pathways, leading to restrictive functional defect and altered gas exchange with progressive hypoxemia. Many diseases can affect the pulmonary insterstice and the etiological forms caused by a variety of pathological processes are multiple including genetic factors, association to systemic diseases and inflammatory or fibrotic responses to different stimuli. However, the origin is sometimes unknown, so they are classified as idiopathic diseases. The pediatric pneumologists have tried to classify the interstitial pulmonary disease cases into the originally described categories for adults; however, there are adult forms that do not occur in childhood and unique pediatric presentations that are not seen in adulthood. The importance of knowledge about these diseases, particularly those of unknown or idiopathic origin was stressed in this article.

A Study on Pulmonary Complications of Systemic Sclerosis in Eastern India.

Aim. This study was undertaken to find out the characteristics of clinical, radiological and functional changes affecting the respiratory system in patients with systemic sclerosis (SSc) from eastern India, and the association of these characteristics with pulmonary hypertension. Methods. This was a cross-sectional, observational study involving 46 patients. Other than the routine tests, anti-nuclear antibody (ANA), spirometry, diffusing capacity of lung for carbon monoxide (DLCO) measurement, chest radiograph, highresolution computed tomography (HRCT) of thorax, 6-minute walk test and echocardiography were done. Results. Out of a total of 46 patients, 27 patients had diffuse cutaneous SSc (dcSSc) and 19 had limited cuteaneous SSc (lcSSc). Eleven patients had pulmonary hypertension. The HRCT revealed diffuse parenchymal lung disease (DPLD) in 32 (65%) cases. The ANA was positive in 83% cases. Anti-Scl70 was found in 41% of patients with dcSSc and anti-centromere antibody was found in 47% of patients with lcSSc. Spirometry revealed restrictive pattern in 30 patients; 9 had obstruction; and the rest were normal. The DLCO was abnormal in 38 patients. A strong correlation was found between reduction in DLCO and pulmonary artery systolic pressure (PASP). Also, a strong association was observed between a drop of >4% in oxygen saturation on 6-minute walk test and presence of pulmonary arterial hypertension (PAH). Conclusions. Majority of the patients with SSc had restrictive lung disease with abnormal DLCO and features resembling non-specific interstitial pneumonia. Nucleolar ANA was predominantly found in patients having PAH. Presence of DPLD had a negative association with presence of anti-centromere antibody. Reduction in DLCO and a fall of >4% in oxygen saturation on 6-minute walk test may be used as predictors of PAH in asymptomatic individuals.

Tobacco smoking related interstitial lung diseases.

Smoking is a leading cause of death worldwide and is the important proximate cause of the most common noncommunicable respiratory disease, chronic obstructive pulmonary disease (COPD). Smoking is causally related to the development of certain forms of interstitial lung diseases (ILDs) including desquamative interstitial pneumonia (DIP), respiratory bronchiolitis associated interstitial lung disease (RB-ILD), pulmonary Langerhans’ cell histiocytosis (LCH), idiopathic pulmonary fibrosis (IPF) and acute eosinophilic pneumonia (AEP), among others. It is important to understand this causal relationship, as well as the natural history and prognosis of these diseases. The response to treatment of ILDs in general is quite dismal. For most of the ILDs, the only definitive treatment measure remains oxygen therapy and lung transplantation. As lung transplantation is still in its infancy and is unaffordable for a majority of patients with ILD in India, most of these patients can only hope to receive palliative supportive care. However, patients with tobacco smoking related ILDs have better outcomes, and progression of these disease ceases with smoking cessation. We review here, the varied clinical, radiological, pathological features, and the progression and outcome in this group of ILDs. Better understanding of these diseases, including making “smoking cessation” a central goal of management will improve overall outcomes.

Clinical Role of Interstitial Pneumonia in Patients with Scrub Typhus: A Possible Marker of Disease Severity

Interstitial pneumonia (IP) frequently occurs in patients with scrub typhus, but its clinical significance is not well known. This study was designed to evaluate interstitial pneumonia as a marker of severity of the disease for patients with scrub typhus. We investigated clinical parameters representing the severity of the disease, and the chest radiographic findings for 101 patients with scrub typhus. We then compared these clinical factors between patients with and without IP. We also studied the relationship between IP and other chest radiographic findings. The chest radiography showed IP (51.4%), pleural effusion (42.6%), cardiomegaly (14.9%), pulmonary alveolar edema (20.8%), hilar lymphadenopathy (13.8%) and focal atelectasis (11.8%), respectively. The patients with IP (n=52) had higher incidences in episode of hypoxia (p=0.030), hypotension (p=0.024), severe thrombocytopenia (p=0.036) and hypoalbuminemia (p=0.013) than the patients without IP (n=49). The patients with IP also had higher incidences of pleural effusion (p<0.001), focal atelectasis (p=0.019), cardiomegaly (p<0.001), pulmonary alveolar edema (p=0.011) and hilar lymphadenopathy (p<0.001) than the patients without IP. Our data suggest that IP frequently occurs for patients with scrub typhus and its presence is closely associated with the disease severity of scrub typhus.

Persistent pneumonia in children.

With the aim to study the clinical profile and describe the predisposing causes of persistent pneumonia in Indian children, the clinical details and the investigations of children presenting with persistent pneumonia to Pediatric/Chest Clinic services of a tertiary care center in north India were reviewed. Nineteen children (16 boys) were evaluated for persistent pneumonia over a period of 5 years. Based on the clinical features and the results of the investigations, underlying illness could be identified in 16 (84.2%) children. The most frequent underlying causes for persistent pneumonia in children were asthma (26.3%) and post-tubercular bronchiectasis (26.3%).

Enfermedades intersticiales del pulmón: incidencia, etiología y estrategia diagnóstica en un centro especializado / Interstitial lung diseases: incidence, etiology and diagnostic strategy in a specialized center

Las enfermedades intersticiales del pulmón (EIP) son un grupo muy amplio y heterogéneo de enfermedades que comprometen difusamente el parénquima pulmonar afectando predominantemente al intersticio. Con el objetivo de determinar la incidencia y etiologías de las EIP y conocer la estrategia empleada para llegar al diagnóstico, se revisaron retrospectivamente las historia clínicas (HC) de los pacientes que consultaron por primera vez durante el período 2/1/99 al 30/12/00. Se seleccionaron las que cumplían con uno o más de los criterios aceptados para su diagnóstico. Se evaluó cada método de diagnóstico empleado, clasificándolos en métodos no invasivos (MNI): HC, Rx, TAC de tórax, pruebas de laboratorio y de función pulmonar; y métodos invasivos (MI): lavado broncoalveolar (LBA), biopsia transbronquial (BTB) y biopsia quirúrgica (BQ). De las 4518 HC revisadas, 88 (1,94 por ciento) presentaron EIP. En 67 pacientes (76 por ciento) se llegó a un diagnóstico específico (DE). Las etiologías más frecuentes fueron: TB 16 (18 por ciento), silicosis 14 (16 por ciento), sarcoidosis 8 (9 por ciento) y FPI 6 (6,8 por ciento). Utilizando solamente MNI se obtuvo el diagnóstico en 31 de los 67 pacientes (46 por ciento). En el resto, 36 pacientes (54 por ciento), el diagnóstico se hizo por MNI y MI. El LBA se realizó en la mitad de los casos (44 pacientes). En 13 (30 por ciento) permitió hacer DE y en 31 (70 por ciento) no contribuyó al diagnóstico. La BTB fue realizada en 32 pacientes (36 por ciento). En 9 (28 por ciento) permitió hacer el diagnóstico y en 23 (72 por ciento) no fue útil para el mismo. La BQ se practicó en 5 pacientes (6 por ciento) obteniéndose diagnóstico en 4 (80 por ciento). De los 21 pacientes sin DE, a 10 se le realizaron MI y a pesar de ello, no se pudo obtener el diagnóstico. Conclusiones: La incidencia de EIP fue baja (2 por ciento) si se la compara con otras enfermedades del aparato respiratorio. Las causas más frecuentes fueron: TB y silicosis. Esto podría deberse a que en nuestro hospital se derivan pacientes con estas patologías. En casi la mitad de los pacientes, el DE se realizó sólo por MNI. El rendimiento del LBA y la BTB fue de alrededor de 30 por ciento. En cambio, el de la BQ fue 80 por ciento. En 24 por ciento de los pacientes no pudo obtenerse DE. Esto podría deberse a que la BQ se realizó en sólo 5 pacientes. Recomendamos realizar la BQ con más frecuencia.

Infecçäo por Chlamydia trachomatis no período neonatal: aspectos clínicos e laboratoriais. Experiência de uma década: 1987-1998 / Chlamydia trachomatis infection in neonatal period: clinical and laboratorial aspects. Experience of a decade: 1987-1998

A infecção por C. trachomatis é adquirida pelo recém-nascido (RN) principalmente durante sua passagem pelo canal parto; 25 por cento a 50 por cento destes deverão desenvolver conjuntivite e 10 por cento a 20 por cento pneumonia. Objetivos. Verificar a incidência de infecção ocular por C. trachomatis nos RN internados com diagnóstico de conjuntivite, num período de 10 anos. - Observar a associação entre infecção ocular é pneumonia intersticial - Estudar os aspectos epidemiológicos e os métodos utilizados para o diagnóstico laboratorial. Casuística e Metodologia. Foram analisados os RN internados com diagnóstico de conjuntivite e/ ou pneumonia interticial internados na UCINE no período de 1987-1998. Os métodos de diagnóstico utilizados foram: a pesquisa direta do agente etiológico em raspado de conjuntiva, radiografia de tórax, sorologia para C. trachomatis no sangue pelo método de imunofluorescência para anticorpos IgG e IgM. Resultados. Estudamos as características de 20 RN que apresentaram infecção por C. trachomatis: 15 eram de termo (75 por cento) e cinco, pré-termos (25 por cento); houve predominância da infecção no sexo feminino (60 por cento); a pneumonia esteve presente em 15 dos 20 RN (75 por cento) e 12 apresentaram associação de conjuntivite e pneumonia. Não houve relação significante entre tipo de parto, idade materna, número de parceiros e a infecção, sendo que o antecedente materno de leucorreia esteve presente em 50 por cento dos casos. O diagnóstico sorológico esteve relacionado com a presença de pneumonia e a pesquisa direita com a conjuntivite. A incidência de conjuntivite por C. trachomatis entre os RN internados com esse diagnóstico durante o período de estudo foi de 17/100 (17 por cento). Conclusões. A. C. trachomatis é um importante agente patogênico e sua pesquisa é muito importante em RN com conjuntivite e/ou pneumonia intersticial mesmo na ausência de fatores de risco para doença sexualmente transmissível. A pesquisa direta em raspado de conjuntiva e o exame sorológico se mostraram importantes como métodos auxiliares do diagnóstico.

Artritis reumatoide: el punto de vista pulmonar / Rheumatoid arthritis: the pulmonary approach

La artritis reumatoide es una enfermedad que por su naturaleza sistémica afecta el pulmón. Las manifestaciones clínicas de la neumopatía reumatoide pueden ser sutiles. La prevalencia de neumopatía intersticial difusa varía, dependiendo del método diagnóstico que se utilice para su detección. La correlación clínico-serológica ayuda a sospechar y buscar intencionadamente afección pulmonar. La biopsia pulmonar debe realizarse si hay progresión lesional a pesar de un tratamiento adecuado, para descartar patología agregada y como ayuda en las decisiones terapéuticas

Enfermedad pulmonar intersticial difusa (EPID) en Colombia

Las EPID, aunque no muy comunes, son más frecuentes en Colombia de lo que en principio se piensa. Las condiciones locales, sin duda, influyen en su distribución y frecuencia y justifican su investigación en nuestro medio. Dadas las características de presentación y los métodos necesarios para el diagnóstico de las EPID, es necesaria la intervención del neumonologo en la mayoría de los casos. La cooperación interdisciplinaria permitirá conocer mejor el espectro de las EPID en nuestros país.

Síndrome de pulmones contraidos y neumonitis intersticial en LES: caso de evolución fatal

Se presentan el primer caso informado en la literatura de una complicación pulmonar infrecuente en pacientes con Lupus Eritematoso Sistémico, " El Síndrome de Pulmones Contraidos". El caso descrito presenta características especiales que se discuten ampliamente y permiten revisar el tema.