Delayed Access and Survival in Idiopathic Pulmonary Fibrosis

Lamas, Daniela; Kawut, Steven M.; Bagiella, Emilia; Philip, Nisha; Arcasoy, Selim M.; Lederer, David J.

doi:10.1164/rccm.201104-0668oc

Cited by 256 publications

(221 citation statements)

References 19 publications

Supporting

Mentioning

201

Contrasting

Unclassified

Order By: Relevance

“…In a Danish cohort, the median duration of symptoms before first visit to a referral centre was 28 months [10]. A prospective study reported a median diagnosis delay of 26 months [11]. Delay had a negative impact on patients, who felt that their symptoms were inappropriately addressed for too long [11].…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

The lived experience with idiopathic pulmonary fibrosis: a qualitative study

et al. 2016

View full text Add to dashboard Cite

The disease course in idiopathic pulmonary fibrosis (IPF) is variable, but patients experience a progressive decline in lung function and increased symptom burden leading to death. Little is known about the patients' experience and their needs during the disease course or about the burden on family caregivers. Both patients and family caregivers face an altered life as the disease progresses. The aim of our study was to increase knowledge of life with IPF for patients and family caregivers.This study had a qualitative descriptive design using in-depth dyadic interviews with IPF patients (n=25) and family caregivers (n=24). We used the five-step analysis from the framework method and analysed the data on three levels: the patient, the family caregivers and couple level.The following six themes emerged as the main results: information and disclosure, reactional dyssynchrony, perpetual vigilance, emotional ambivalence, gradual and tacit role shift, and adapted coping strategies.Our findings suggest that IPF patients need information at the time of diagnosis, but some issues should be paced as the disease progresses. A palliation plan was demanded by patients and their caregivers. Further efforts are required to provide palliative care to IPF patients starting at the time of diagnosis. @ERSpublications IPF patients and caregivers need information, but full disclosure should be gradual and palliation initiated early

show abstract

Section: Introductionmentioning

confidence: 99%

“…A prospective study reported a median diagnosis delay of 26 months [11]. Delay had a negative impact on patients, who felt that their symptoms were inappropriately addressed for too long [11]. When IPF is finally diagnosed, patients often distrust the healthcare system [8] and indicate that they lack information on the diagnosis [12].…”

Section: Introductionmentioning

confidence: 99%

The lived experience with idiopathic pulmonary fibrosis: a qualitative study

et al. 2016

View full text Add to dashboard Cite

show abstract

“…In a cohort study of 129 patients by Lamas et al [28], a longer delay was associated with an increased risk of death, median delay was 2.2 years (inter-quartile range 1.0-3.8 years), HR 1.3, and 95% confidence interval (CI) 1.03-1.6. This is the only study which discusses the mortality associated with a delay in diagnosis.…”

mentioning

confidence: 96%

“…As in our study, many patients who received anti-tuberculosis medications resulted in delayed diagnosis and treatment. The study by Lamas et al [28] reflects the importance of early diagnosis and early referral for treatment for reducing all-cause mortality.…”

mentioning

confidence: 99%

Idiopathic Pulmonary Fibrosis: A Study of 46 Patients from Western India: Clinical Presentations and Survival

Natarajan¹,

Subramanian²

2015

Turk Thorac J

View full text Add to dashboard Cite

OBJECTIVES:Idiopathic pulmonary fibrosis (IPF) is the most common form of interstitial lung disease (ILD) diagnosed all over the world as well as in India. The objective was to study the clinical presentations, treatment options, and survival of patients diagnosed with IPF. MATERIALS AND METHODS:This was a 6-year retrospective observational study. All patients diagnosed with ILD underwent a comprehensive evaluation to confirm the diagnosis of IPF and were subsequently included in the study. Clinical data and laboratory data were recorded. Patients were treated as per current guidelines. RESULTS:Out of the total number of patients (n=123) diagnosed with ILD, 46 (37.4%) patients were included in the study [Males, n=19 (41%) and females, n=27 (59%)]. Sixty-seven percent (n=31) of the patients were in the age group of 61-80 years. The mean age of males and females was 68.36 and 66.25 years, respectively. Clubbing was observed in 67% (n=29) of the patients. The mean forced vital capacity (FVC) was 52%. High-resolution computed tomography (HRCT) of the chest showed a definite usual interstitial pneumonia (UIP) pattern of honeycombing in 60% (n=28) of the patients. Desaturation on a 6 min walk distance was noted in 60% (n=28) of the patients. Ten patients wrongly received-anti tuberculosis treatment (21%) before they were diagnosed with IPF. The median delay in diagnosis was 20 months. The 5-year survival rate was 25%; the average survival was 39 months. Patients receiving pirfenidone showed a survival benefit compared with those having received triple therapy. Survival in IPF was significantly low compared with that in other ILDs. Twenty-four (52%) patients died during the study. CONCLUSION:IPF is a disease which affects the fourth to seventh decade of the Indian population with low survival. KEYWORDS: Idiopathic pulmonary fibrosis, survival, pirfenidone INTRODUCTIONInterstitial lung diseases (ILD) are a group of disorders characterized by varying degrees of fibrosis and inflammation of the lung parenchyma or interstitium [1]. Combining clinical, radiological, and pathological information is pivotal to accurate diagnosis in interstitial lung diseases. Idiopathic pulmonary fibrosis (IPF) is the most common form of ILD diagnosed worldwide as well as in India. IPF portends a poor prognosis as well as increased morbidity and poor quality of life. In India where lung transplantation is in a nascent stage, patients presenting with IPF have a poor prognosis. We present the analysis of our data from the western Indian population.The objective was to study the clinical presentations, treatment options, and survival of patients diagnosed with IPF. MATERIAL AND METHODS Design and setting:This was a retrospective observational study of patients presented to our specialty respiratory clinics in the period from January 2008 to March 2014. Data analysis was performed subsequently.Eligibility: All patients presented to the outpatients department with a history and examination suggesting interstitial lung disease were confirmed with...

show abstract

“…Exclusion of these entities relies primarily on history taking, autoimmune serology tests, HRCT features and the use of multidisciplinary discussions. 1,25 The 2011 Statement specifically recommends against bronchoalveolar lavage cellular analysis and transbronchial biopsy for the exclusion of chronic HP. 1 Awareness of this recommendation is likely reflected in only 8% of respondents reporting that bronchoscopy was useful in the diagnosis of IPF in 2013 (compared to 22% in 2009).…”

Section: Discussionmentioning

confidence: 99%