S2K Guideline for Diagnosis of Idiopathic Pulmonary Fibrosis

Abstract

Idiopathic pulmonary fibrosis (IPF) is a severe and often fatal disease. Diagnosis of IPF requires considerable expertise and experience. Since the publication of the international IPF guideline in the year 2011 and the update 2018 several studies and technical advances have occurred, which made a new assessment of the diagnostic process mandatory. The goal of this guideline is to foster early, confident, and effective diagnosis of IPF. The guideline focusses on the typical clinical context of an IPF patient and provides tools to exclude known causes of interstitial lung disease including standardized questionnaires, serologic testing, and cellular analysis of bronchoalveolar lavage. High-resolution computed tomography remains crucial in the diagnostic workup. If it is necessary to obtain specimens for histology, transbronchial lung cryobiopsy is the primary approach, while surgical lung biopsy is reserved for patients who are fit for it and in whom a bronchoscopic diagnosis did not provide the information needed. After all, IPF is a diagnosis of exclusion and multidisciplinary discussion remains the golden standard of diagnosis.

Epidemiology, healthcare utilization, and related costs among patients with IPF: results from a German claims database analysis

“…In a review of 34 studies from 21 countries published between 1968 and 2012, Hutchinson et al [ 28 ] reported incidence estimates of 3–9 cases per 100,000 individuals per year in Europe and North America. The German Guideline for Diagnosis and Management of IPF from 2013 reported prevalence estimates of 2–29 cases per 100,000 individuals [ 29 ]. Compared with these estimates, our data suggest a relatively high incidence and prevalence of IPF in Germany.…”

Serum profiles of metabolomic and GDF-15 differentiate idiopathic pulmonary fibrosis from other interstital lung diseases: a preliminary study

“…The 5-year survival rate of IPF is 20%-40%, which is second only to many malignant tumors. In acute exacerbation, the hospitalization rate is more than 50%, and the median survival time is 3-4 months [20, 21]. Patients without systemic anti-fibrosis treatment or lung transplantation have a survival time of about 3.5-4.5 years [22], and the prognosis is extremely poor.…”

“…Even if the imaging is consistent with typical interstitial pneumonia (UIP), other types of ILD that can cause UIP still need to be excluded for differentiation. In addition, the diagnosis of IPF is often delayed clinically, and it takes 1.5 years from symptoms to diagnosis of IPF, which is not conducive to early intervention and prognosis of patients [20]. Therefore, exploring the diagnosis and differential diagnosis markers of ILD and IPF is of great significance for patients to get timely diagnosis, make effective and scientific treatment decisions, and improve the prognosis of patients.…”

Biomarkers in Idiopathic Pulmonary Fibrosis

“…Similar to the previous guidelines, current existing guidelines such as 2021 German Respiratory Society (DGP), 2018 American Thoracic Society (ATS), European Respiratory Society (ERS), Japanese Respiratory Society (JRS), American Latin Thoracic Association (ALAT) guidelines strongly recommend not to measure any serum biomarker for IPF diagnosis and distinguishing IPF from other interstitial lung diseases in patients with newly detected ILD of apparently unknown cause who are clinically suspected of having IPF. Also, no guidelines on prognostic biomarkers are available [12,[17][18][19][20].…”

“…MMP7 indicates a correct IPF diagnosis in more than half of the patients, suggesting an incorrect classification in about one-third of patients. Based on these data, the diagnostic value of these serum biomarkers is currently considered insufficient to support clinical use [17].…”