Dynamics in diagnoses and pharmacotherapy before and after diagnosing idiopathic pulmonary fibrosis

Abstract

BackgroundIdiopathic pulmonary fibrosis (IPF) is a well-characterised interstitial lung disease (ILD). Typically, the IPF diagnosis is delayed due to non-specific symptoms, but can also be delayed due to treatment attempts on false indication, or due to treatment targeting common comorbidities. This observational study aimed to assess the dynamics in the medication and diagnosis patterns in the period before and after an IPF diagnosis.MethodsWe identified all Danish patients with IPF during 2002 to 2017. We evaluated new and ongoing drug treatments and incident diagnoses 36 months before, and 12 months after an IPF diagnosis by use of Danish nationwide registries. To aid interpretation, ten random controls were recruited for each case.ResultsA total of 650 IPF patients were identified (median age 73 years [IQR 65–78], 70.3% males). Prior to the IPF diagnosis, the most prevalent diagnoses were dyspnea and non-IPF ILDs. For drug use, IPF patients had higher initiation rates for antibiotics, oral corticosteroids and mucolytics. In terms of drug volume, IPF patients used more respiratory drugs, antibiotics, immunosuppressants, corticosteroids, proton pump inhibitors, benzodiazepines and opium alkaloids within the 6 months preceding their IPF diagnosis, compared to the controls. Overall drug use decreased after an IPF diagnosis, mainly due to a reduced glucocorticoid and cardiovascular drug use.ConclusionAmong IPF patients, an increased drug use was observed for diagnoses with symptoms overlapping those of IPF, particularly this was observed during the last 6 months before an IPF diagnosis. This emphasises the need for an increased IPF awareness.

Using thoracic ultrasound to detect interstitial lung disease in patients with rheumatoid arthritis: a protocol for the diagnostic test accuracy AURORA study

“…The diagnosis of ILD in patients with RA may be masked, as symptoms of ILD are compatible with COPD (dyspnoea, cough, recurrent clinical pneumonia). ILD may easily be mistaken for COPD and vice versa which has previously been pointed out for other ILD subtypes as, for example, IPF 64. Currently, there are no studies on patients with RA with unexplained respiratory symptoms, nor any studies on screening for respiratory symptoms in RA.…”

“…ILD may easily be mistaken for COPD and vice versa which has previously been pointed out for other ILD subtypes as, for example, IPF. 64 Currently, there are no studies on patients with RA with unexplained respiratory symptoms, nor any studies on screening for respiratory symptoms in RA. This cohort of patients with RA will access the diagnostic accuracy of TUS in detecting ILD in a cohort of patients with RA with unexplained respiratory symptoms.…”

The diagnostic trajectories of Danish patients with autoimmune rheumatologic disease associated interstitial lung disease: an interview-based study

“…Many patients report to be treated repeatedly for respiratory infections or more common lung diseases like COPD, heart failure and asthma even though they report no improvement, a tendency that previously has been observed also in patients with idiopathic pulmonary fibrosis (IPF) based on prescription data and patient interviews [ 15 , 30 ]. Similar reasons for delay were also reported in an IPF cohort, where many patients experienced multiple erroneous differential diagnoses like asthma, emphysema and COPD, resulting in a diagnostic delay of more than one year for the majority of patients before they were diagnosed with IPF [ 15 , 31 ].…”

Idiopathic pulmonary fibrosis: Current and future treatment

“…Early diagnosis of IPF is difficult because initial symptoms, most commonly dyspnea and cough, are mild and nonspecific and overlap many other common conditions. 17 , 18 , 19 Once symptoms become more pronounced, the diagnosis can be made and antifibrotic therapy options may be initiated. 20 Upon physical examination, respiratory auscultation may detect Velcro crackles and clubbing of fingers may be apparent.…”