RESUMO
BACKGROUND: Usual interstitial pneumonia can present with a probable pattern on high-resolution computed tomography (HRCT), but the probability of identifying usual interstitial pneumonia by surgical lung biopsy in such cases remains controversial. We aimed to determine the final clinical diagnosis in patients with a probable usual interstitial pneumonia pattern on HRCT who were subjected to surgical lung biopsy. METHODS: HRCT images were assessed and categorized by three radiologists, and tissue slides were evaluated by two pathologists, all of whom were blinded to the clinical findings. The final clinical diagnosis was accomplished via a multidisciplinary discussion. Patients with a single layer of honeycombing located outside of the lower lobes on HRCT were not excluded. RESULTS: A total of 50 patients were evaluated. The most common final clinical diagnosis was fibrotic hypersensitivity pneumonitis (38.0%) followed by idiopathic pulmonary fibrosis (24.0%), interstitial lung disease ascribed to gastroesophageal reflux disease (12.0%) and familial interstitial lung disease (10.0%). In the group without environmental exposure (n = 22), 10 patients had a final clinical diagnosis of idiopathic pulmonary fibrosis (45.5%). Irrespective of the final clinical diagnosis, by multivariate Cox analysis, patients with honeycombing, dyspnoea and fibroblastic foci on surgical lung biopsy had a high risk of death. CONCLUSIONS: The most common disease associated with a probable usual interstitial pneumonia pattern on HRCT is fibrotic hypersensitivity pneumonitis followed by idiopathic pulmonary fibrosis and interstitial lung disease ascribed to gastroesophageal reflux disease. In patients without environmental exposure, the frequencies of usual interstitial pneumonia and a final clinical diagnosis of idiopathic pulmonary fibrosis are not sufficiently high to obviate the indications for surgical lung biopsy.
Assuntos
Alveolite Alérgica Extrínseca/diagnóstico por imagem , Fibrose Pulmonar Idiopática/diagnóstico por imagem , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Pulmão/diagnóstico por imagem , Tomografia Computadorizada por Raios X , Idoso , Alveolite Alérgica Extrínseca/patologia , Biópsia/efeitos adversos , Diagnóstico Diferencial , Feminino , Humanos , Fibrose Pulmonar Idiopática/patologia , Pulmão/patologia , Doenças Pulmonares Intersticiais/patologia , Masculino , Pessoa de Meia-Idade , Probabilidade , Estudos RetrospectivosRESUMO
Prediction models for survival at baseline evaluation have been proposed in idiopathic pulmonary fibrosis (IPF) but include diffusion capacity of the lung for carbon monoxide, a test not available in many places. The aim of the present study was to develop a simple new mortality risk scoring system for patients with IPF at initial evaluation without diffusion capacity of the lung for carbon monoxide measurement.A total of 173 patients, 72% males, mean age 70 years, 64% smokers/ex-smokers, were included in a retrospective study. The diagnosis was made by surgical lung biopsy in 40 (23%); in the remaining patients, a usual interstitial pneumonia pattern was present in high-resolution computed tomography. Patients with forced expiratory volume in 1 second/forced vital capacity ratio (FEV1/FVC) <0.70 were excluded. Dyspnea was evaluated by magnitude of task on the Mahler scale (Chest 1984). Peripheral oxygen saturation was measured by oximetry at rest and at the end of a 4âminutes step test or a 6-minute walk test.At the end of the follow-up period, 154 (89%) of the patients had died. Based on the univariate Cox proportional-hazards model, survival (P ≤ .10) was related directly to the dyspnea score, presence of cough, lower values of FVC% and FEV1%, lower rest and oxygen desaturation during exercise, and greater FEV1/FVC. By Cox multivariate analysis, the results remained correlated to the survival dyspnea score, FVC%, and exercise peripheral oxygen saturation. A score, using these variables, was developed and was able to discriminate among 3 groups, with high, low, and intermediate survival curves.A prognostic score, taking into account dyspnea, FVC%, and oxygen desaturation during exercise, can estimate survival in IPF.
Assuntos
Monóxido de Carbono/metabolismo , Fibrose Pulmonar Idiopática/mortalidade , Pulmão/metabolismo , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Fibrose Pulmonar Idiopática/metabolismo , Fibrose Pulmonar Idiopática/fisiopatologia , Pulmão/fisiopatologia , Masculino , Pessoa de Meia-Idade , Modelos de Riscos Proporcionais , Testes de Função Respiratória , Estudos Retrospectivos , Medição de RiscoRESUMO
BACKGROUND: Airway-centered Interstitial Fibrosis (ACIF) is a common pathologic pattern observed in our practice. OBJECTIVES: The objectives of this study are to describe the causes associated with ACIF in a large sample of patients and its effect on survival. METHODS: A retrospective study in three centers of interstitial lung disease in São Paulo, between January of 1995 and December of 2012. The surgical lung biopsy specimens were reviewed by three pathologists. The clinical, functional and tomographic findings were analyzed by a standardized protocol. RESULTS: There were 68 cases of ACIF, most of them women. The mean age was 57 ± 12 yr. Dyspnea, cough, restrictive pattern at spirometry and oxygen desaturation at exercise were common. A reticular pattern with peribronchovascular infiltrates was found in 79% of the cases. The etiologies of ACIF were hypersensitivity pneumonitis in 29 (42.6%), gastroesophageal reflux disease in 17 (25.0%), collagen vascular disease in 4 (5.9%), a combination of them in 15 cases and idiopathic in 3 (4.4%). The median survival was 116 months (95% CI = 58.5 - 173.5). Lower values of oxygen saturation at rest, presence of cough and some histological findings--organizing tissue in the airways, fibroblastic foci and microscopic honeycombing--were predictors of worse survival. CONCLUSIONS: ACIF is an interstitial lung disease with a better survival when compared with IPF. The main etiologies are HP and GERD. The oxygen saturation at rest, the presence of cough and some histological findings are predictors of survival.
Assuntos
Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/etiologia , Fibrose Pulmonar/diagnóstico , Fibrose Pulmonar/etiologia , Idoso , Estudos de Coortes , Feminino , Humanos , Doenças Pulmonares Intersticiais/mortalidade , Masculino , Pessoa de Meia-Idade , Prognóstico , Fibrose Pulmonar/mortalidade , Estudos Retrospectivos , Taxa de Sobrevida/tendênciasRESUMO
BACKGROUND AND AIM: PaO2 during exertion is a significant predictor of IPF survival. The aim of study was to assess oxygen desaturation for predicting survival in IPF patients at the end of a 4-minute step test. METHODS: A longitudinal study was done in 59 patients with IPF from February 1998 to January 2005. Upon initial examination, lung function testing was performed, as were 4-minute step tests, in which patients stepped up and down on a 20-cm single step at a self-paced rate. In the final minute, oxygen saturation by pulse oximetry (SpO2) was measured. RESULTS: Median survival was 58 months. SpO2 at rest and during stepping, dyspnea score, as well as FVC% and DLCO% were found to be significant predictors of mortality. Desaturation to 89% or less,correlated with a hazard ratio of 2.39 for IPF mortality (95% CI, 1.16-3.63; p < 0.0001). In patients with such desaturation, four-year survival was 39%, compared to 96% in those with no desaturation. In a multivariate Cox analysis, only SpO2 during stepping remained significant (p < 0.0001). A DLco < 45% of predicted (n=40 patients) correlated with a hazard ratio of 2.23 for mortality (95% CI, 0.73-3.71; p < 0.0001). When the analysis was repeated including DLco, SpO2 and DLco remained significant (p < 0.05). CONCLUSION: Desaturation to 89% or less in a 4-min step test is a strong predictor of mortality in IPF patients.
Assuntos
Teste de Esforço , Oxigênio/sangue , Fibrose Pulmonar/mortalidade , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Estimativa de Kaplan-Meier , Estudos Longitudinais , Masculino , Pessoa de Meia-Idade , Oximetria , Consumo de Oxigênio , Prognóstico , Fibrose Pulmonar/fisiopatologia , Análise de SobrevidaRESUMO
BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is the most common interstitial lung disease and is usually associated with a poor prognosis. IPF patients have a significant impairment in health-related quality of life (HRQL), both in terms of physical and psychological functioning. A substantial number of dyspnea scales are available to clinicians for clinical and research use. The objective of the present study was to investigate how some commonly used dyspnea scales correlate with measurements of HRQL in IPF patients. MATERIAL/METHODS: A group of 30 patients with IPF who attended a specialized outpatient respiratory clinic were interviewed during a stable period of the disease. HRQL was assessed by the SF-36 questionnaire. Dyspnea was measured by the baseline dyspnea index (BDI), Modified Medical Research Council Scale (MRC), Oxygen Cost Diagram (OCD), clinical element of the Clinical, Roentgenographic and Physiologic score (CRP), and a new dyspnea scale (NS) based on estimates of required oxygen consumption for usual activities. RESULTS: Patients showed impairments in all HRQL domains except the Pain Index. All dyspnea scales showed significant correlations with both physical and mental HRQL domains. The highest Spearman's coefficients between dyspnea ratings and SF-36 components were obtained using the BDI. CONCLUSIONS: Dyspnea ratings correlate with HRQL measurements in IPF patients. Dyspnea scales can be used as a measure of HRQL in special circumstances.