This document provides clinical recommendations for the diagnosis of idiopathic pulmonary fibrosis (IPF). It represents a collaborative effort between the American Thoracic Society, European ...Respiratory Society, Japanese Respiratory Society, and Latin American Thoracic Society.
The evidence syntheses were discussed and recommendations formulated by a multidisciplinary committee of IPF experts. The evidence was appraised and recommendations were formulated, written, and graded using the Grading of Recommendations, Assessment, Development, and Evaluation approach.
The guideline panel updated the diagnostic criteria for IPF. Previously defined patterns of usual interstitial pneumonia (UIP) were refined to patterns of UIP, probable UIP, indeterminate, and alternate diagnosis. For patients with newly detected interstitial lung disease (ILD) who have a high-resolution computed tomography scan pattern of probable UIP, indeterminate, or an alternative diagnosis, conditional recommendations were made for performing BAL and surgical lung biopsy; because of lack of evidence, no recommendation was made for or against performing transbronchial lung biopsy or lung cryobiopsy. In contrast, for patients with newly detected ILD who have a high-resolution computed tomography scan pattern of UIP, strong recommendations were made against performing surgical lung biopsy, transbronchial lung biopsy, and lung cryobiopsy, and a conditional recommendation was made against performing BAL. Additional recommendations included a conditional recommendation for multidisciplinary discussion and a strong recommendation against measurement of serum biomarkers for the sole purpose of distinguishing IPF from other ILDs.
The guideline panel provided recommendations related to the diagnosis of IPF.
Introduction
The objective of this study was to analyze mortality, possible predictors of long‐term survival, and health‐related quality of life of a large chronic hypersensitivity pneumonitis (CHP) ...patient sample.
Methods
Longitudinal study in patients diagnosed with CHP during 2004‐2013, followed for at least 1 year. Patients remaining alive and consenting to participate had a follow‐up visit during 2015, including a complete pulmonary function study and the EuroQol‐5D and Beck Depression and Anxiety Inventories.
Results
Out of the 160 patients finally included, 87 remained alive. Seventy‐three had died or underwent lung transplantation at the time of the study with a median survival of 7.0 (4.4‐14.5) years. A Cox proportional risk model showed that factors associated with lower survival were as follows: increased age, a low percentage of lymphocytes in bronchoalveolar lavage (BAL), a decreased transfer factor of the lung for carbonmonoxide (DLCO), presence of honeycomb in the high‐resolution chest scan (HRCT), and the usual interstitial pneumonia (UIP) histologic pattern. At follow‐up, all patients presented an EuroQol‐5D score <0.8 and 21(50%) and 9(28.6%) subjects presented a probable anxiety and depressive syndrome, respectively.
Conclusion
CHP is a severe disease with a bad mid‐term prognosis. Lymphocyte values in BAL and DLCO values at baseline, presence of honeycomb in HRCT, and UIP histologic pattern were found to be predictors of survival. Early accurate diagnosis of the disease is fundamental for prompt initiation of antigen avoidance.
Chronic hypersensitivity pneumonitis is a severe disease with a bad mid‐term prognosis. Lymphocyte values in BAL and DLCO values at baseline, presence of honeycomb in HRCT, and UIP histologic pattern were found to be predictors of survival. At follow‐up, all patients presented an EuroQol‐5D score < 0.8 and a probable anxiety and depressive syndrome.
The clinical features of idiopathic pulmonary fibrosis (IPF) and chronic hypersensitivity pneumonitis can be indistinguishable; the need to eliminate occult environmental factors known to cause ...pulmonary fibrosis in patients suspected to have IPF during diagnostic evaluation is evident. We aimed to investigate occult, putative causes in the environments of patients diagnosed with IPF using tests beyond those conventionally used.
In this case-cohort study, 60 consecutive patients diagnosed with IPF on the basis of the 2000 American Thoracic Society (ATS) and the European Respiratory Society (ERS) criteria were prospectively followed up every 4 months for 6 years between Jan 1, 2004, and Dec 31, 2009. At each visit a uniformly applied questionnaire was administered to these 60 patients to identify occult antigen exposure known to cause hypersensitivity pneumonitis. Patients underwent specific IgG determination, bronchoalveolar lavage, bronchial challenge testing with suspected antigens, and re-review of histopathological features in existing and subsequently obtained surgical lung biopsy samples and from lung explants. Specimens obtained from suspected sources from the patient's environment were subjected to cultures in microbiology laboratory. These clinical data and discussions among pulmonologists and radiologists familiar with IPF were used to confirm the diagnosis in accordance with 2011 ATS, ERS, Japanese Respiratory Society, and Latin American Thoracic Association guidelines; 46 of the 60 patients had IPF according to the 2011 guidelines, and our analyses in this study were focused on these 46 patients.
20 of the 46 (43%, 95% CI 29-58) patients with IPF according to 2011 guidelines had a subsequent diagnosis of chronic hypersensitivity pneumonitis: nine patients had positive bronchial challenge testing (eight of whom were also IgG positive and six of these patients also had surgical lung biopsy showing a pattern consistent with chronic hypersensitivity pneumonitis); seven were IgG positive plus had histopathology on surgical lung biopsy that was consistent with hypersensitivity pneumonitis; one was IgG positive plus had greater than 20% lymphocytes in bronchoalveolar lavage fluid; and three had findings on surgical lung biopsy that were consistent with subacute hypersensitivity pneumonitis (and IgG positive). Altogether, 29 of 46 patients diagnosed with IPF who had met the 2011 criteria had lung tissue available for histopathology (surgical lung biopsy in 28 patients and explanted lung in two patients, one of whom also had surgical biopsy) during the study period, and 16 of the 20 patients with chronic hypersensitivity pneumonitis had histopathological features on surgical lung biopsy that were consistent with this diagnosis. 26 of the 46 patients remained with a diagnosis of IPF.
Almost half of patients diagnosed with IPF on the basis of 2011 criteria were subsequently diagnosed with chronic hypersensitivity pneumonitis, and most of these cases were attributed to exposure of occult avian antigens from commonly used feather bedding. Our results reflect findings in one centre with recognised expertise in chronic hypersensitivity pneumonitis, and further research and studies at other centres are warranted.
Fondo de Investigaciones Sanitarias; Fundació Privada Cellex; SEPAR 2010.
Reliable methods are needed to diagnose hypersensitivity pneumonitis. The aim of the study was to establish the diagnostic yield of specific inhalation challenge (SIC) in patients with ...hypersensitivity pneumonitis. All patients with suspected hypersensitivity pneumonitis in whom SIC was performed (n=113) were included. SIC was considered positive when patients showed a decrease of >15% in forced vital capacity (FVC) or >20% in diffusing capacity of the lung for carbon dioxide, or a decrease of 10% to 15% in FVC accompanied by a temperature increase of 0.5°C within 24 h of inhalation of the antigen. SIC was positive to the agents tested in 68 patients: 64 received a diagnosis of hypersensitivity pneumonitis and SIC results were considered false-positive in the remaining four patients. In the SIC-negative group (n=45), 24 patients received a diagnosis of hypersensitivity pneumonitis and SIC results were considered false-negative, and 21 patients were diagnosed with other respiratory diseases. The sensitivity and specificity of the test were 72.7% and 84%, respectively. Having hypersensitivity pneumonitis caused by an antigen other than birds or fungi predicted a false-negative result (p=0.001). In hypersensitivity pneumonitis, positive SIC testing virtually confirms the diagnosis, whereas negative testing does not rule it out, especially when the antigenic sources are not birds or fungi.
Background ARDS can produce a loss of lung function with persistent sequelae. This study aimed to evaluate health-related quality of life (HRQL) in survivors of ARDS compared with a healthy reference ...population and to determine the middle/long-term radiographic abnormalities and functional status, as well as their relation to observed HRQL, in these patients. Methods This was a prospective study carried out in three ICUs. HRQL in patients was determined with the Nottingham Health Profile immediately after ARDS diagnosis and 6 months after diagnosis. Patients underwent complete respiratory function testing, chest CT scan study, and the 6-min walk test. Results Follow-up was conducted in 38 patients with ARDS. Survivors of ARDS presented a poorer overall HRQL vs the general population, mainly because of lower scores in the dimensions related to mobility, energy, and social isolation. Limitations in daily life activities were documented in 40%. Respiratory function was altered in 67%, with a restrictive respiratory pattern in 58%. Radiologic study disclosed alterations in 76% (mainly reticular pattern). Patients were able to cover only 366 m (318-411 m) in the 6-min walk test and had a minimum pulse oximetry of 93% (90%-94%). A significant correlation was documented between the overall quality of life at first and at 6 months ( r = 0.68, P < .01). Conclusions Survivors of ARDS after 6 months had a poorer HRQL than the healthy population and showed mild radiographic and functional involvement. Early HRQL study in these patients enabled early detection of those who would present more long-term HRQL morbidity.
The diagnosis of hypersensitivity pneumonitis remains a dilemma because of the absence of any characteristic features able to distinguish it from other interstitial lung diseases. We analyze the ...current role of the specific inhalation challenge (SIC) in the diagnosis of this entity.
Few descriptions of the use of SIC for the diagnosis of hypersensitivity pneumonitis have been published in recent years. In fact, hypersensitivity pneumonitis is still diagnosed on the basis of clinical criteria, as there is no agreement on the diagnostic utility of SIC. Two major reviews carried out in the past year have concluded that this test is not standardized and is usually unnecessary; however, a third study found that the test can indeed recreate the symptoms and functional abnormalities in the laboratory, and may therefore be of considerable use in the diagnosis of hypersensitivity pneumonitis.
Hypersensitivity pneumonitis remains a diagnostic challenge. Given that the main cause of the disease is sensitization and hyper-responsiveness to specific antigens in susceptible individuals, SIC is an obvious candidate as the gold standard for diagnosis of this entity. The present review analyzes the reasons for the test's limited use, assesses its diagnostic utility, and proposes a basis for its standardization.
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