Barriers to timely diagnosis of interstitial lung disease in the real world: the INTENSITY survey

Abstract

BackgroundThe diagnosis of idiopathic pulmonary fibrosis (IPF) and other interstitial lung diseases (ILD) presents significant clinical challenges. To gain insights regarding the diagnostic experience of patients with ILD and to identify potential barriers to a timely and accurate diagnosis, we developed an online questionnaire and conducted a national survey of adults with a self-reported diagnosis of ILD.MethodsA pre-specified total of 600 subjects were recruited to participate in a 40-question online survey. E-mail invitations containing a link to the survey were sent to 16 427 registered members of the Pulmonary Fibrosis Foundation. Additionally, an open invitation was posted on an online forum for patients and caregivers (www.inspire.com). The recruitment and screening period was closed once the pre-defined target number of respondents was reached. Eligible participants were adult U.S. residents with a diagnosis of IPF or a non-IPF ILD.ResultsA total of 600 eligible respondents met the eligibility criteria and completed the survey. Of these, 55% reported ≥ 1 misdiagnosis and 38% reported ≥ 2 misdiagnoses prior to the current diagnosis. The most common misdiagnoses were asthma (13.5%), pneumonia (13.0%), and bronchitis (12.3%). The median time from symptom onset to current diagnosis was 7 months (range, 0–252 months), with 43% of respondents reporting a delay of ≥ 1 year and 19% reporting a delay of ≥ 3 years. Sixty-one percent of respondents underwent at least one invasive diagnostic procedure.ConclusionsWhile a minority of patients with ILD will experience an appropriate and expedient diagnosis, the more typical diagnostic experience for individuals with ILD is characterized by considerable delays, frequent misdiagnosis, exposure to costly and invasive diagnostic procedures, and substantial use of healthcare resources. These findings suggest a need for physician education, development of clinical practice recommendations, and improved diagnostic tools aimed at improving diagnostic accuracy in patients with ILD.Electronic supplementary materialThe online version of this article (10.1186/s12890-017-0560-x) contains supplementary material, which is available to authorized users.

Diagnostic test interpretation and referral delay in patients with interstitial lung disease

“…A majority of patients report waiting more than 1 year from the time of symptom onset to ILD diagnosis and often undergo multiple physician evaluations before arriving at the correct diagnosis [4]. Even with recognition of this problem, little headway has been made over the last decade [5,6].…”

“…Studies to date characterizing diagnostic delays in patients with ILD have largely relied on patient questionnaires and insurance claims data [4][5][6][7]. While informative, these studies do not assess physician-level factors that may influence such delays.…”

Experiences of Individuals Awaiting Lung Transplantation

“…This disease represents a growing problem in the world and is the second most common respiratory disease in the United States 3,4 Other worldwide severe respiratory diseases are cystic fibrosis and interstitial lung disease. [5][6][7][8] Some diseases are congenital, such as cystic fibrosis, whereas others can be acquired through smoking, such as COPD. 9,10 Lung transplantation is an established intervention for patients with advanced and life-threatening respiratory disease for which standard medical treatment is no longer effective or for which there is no effective standard medical treatment.…”

Up-regulation of THY1 attenuates interstitial pulmonary fibrosis and promotes lung fibroblast apoptosis during acute interstitial pneumonia by blockade of the WNT signaling pathway

“…AIP is a type of idiopathic interstitial pneumonia, whose pathogenesis remains unclear [22]. In addition, the diagnosis of idiopathic pulmonary fibrosis (IPF) and other ILD has been confronted with enormous clinical challenges [23]. It has been reported that pulmonary fibrosis usually manifests itself as a fibro-proliferative lung disease with an irreversible chronic progression and dismal prognosis [24].…”