S2K-Leitlinie zur Diagnostik und Therapie der idiopathischen Lungenfibrose

Abstract

Idiopathic pulmonary fibrosis is a fatal lung disease with a variable and unpredictable natural history and limited treatment options. Since publication of the ATS-ERS statement on IPF in the year 2000 diagnostic standards have improved and a considerable number of randomized controlled treatment trials have been published necessitating a revision. In the years 2006 - 2010 an international panel of IPF experts produced an evidence-based guideline on diagnosis and treatment of IPF, which was published in 2011. In order to implement this evidence-based guideline into the German Health System a group of German IPF experts translated and commented the international guideline, also including new publications in the field. A consensus conference was held in Bochum on December 3rd 2011 under the protectorate of the "Deutsche Gesellschaft für Pneumologie und Beatmungsmedizin (DGP)" and supervised by the "Arbeitsgemeinschaft der Wissenschaftlichen Medizinischen Fachgesellschaften" (AWMF). Most recommendations of the international guideline were found to be appropriate for the german situation. Based on recent clinical studies "weak negative" treatment recommendations for pirfenidone and anticoagulation were changed into "weak positive" for pirfenidone and "strong negative" for anticoagulation. Based on negative results from the PANTHER-trial the recommendation for the combination therapy of prednisone plus azathiorpine plus N-acetlycsteine was also changed into strong negative für patients with definite IPF. This document summarizes essential parts of the international IPF guideline and the comments and recommendations of the German IPF consensus conference.

Real-World Experience with Nintedanib in Patients with Idiopathic Pulmonary Fibrosis

“…To be eligible for treatment and inclusions in our analyses, patients were required to have a confirmed multidisciplinary team diagnosis of IPF in accordance with the ATS/ERS/JRS/ALAT consensus guidelines [18] and the German guideline for diagnosis and treatment of IPF [19]. …”

Pirfenidone in Idiopathic Pulmonary Fibrosis: Real-Life Experience from a German Tertiary Referral Center for Interstitial Lung Diseases

“…A Cochrane meta-analysis on the cumulative data of these phase II and III trials involving a total of 1,155 patients showed that pirfenidone slows down the decline of FVC and reduces the risk of disease progression by 30% [12]. Consequently, pirfenidone was given a weak positive treatment recommendation in the German guideline for the diagnosis and management of IPF [13]. In addition, the British National Institute for Health and Care Excellence (NICE) approved pirfenidone as a treatment option for mild-to-moderate IPF in a recent technology appraisal guidance [14].…”

Guidelines of the Polish Respiratory Society for Diagnosis and Treatment of Idiopathic Pulmonary Fibrosis

“…A multidisciplinary team involved in IPF diagnosis should always include a clinician (pulmonologist) and radiologist as well as pathologist (in case of lung biopsy has been performed)all should be experienced in ILD diagnosis, and if needed, also a rheumatologist (possibility of CTD), occupational medicine specialist (potential environmental exposure), specialist in respiratory physiology, cardiologist (both helpful, for instance, in assessing the risk associated with invasive diagnosis) or another specialist whose assessment can be helpful in making diagnostic or therapeutic decisions [29,87,[201][202][203]. A MDD in IPF diagnosis is of greatest importance when there is no unequivocal radiographic diagnosis of UIP (in HRCT) [29,31,204].…”

“…The involvement of a MDD is considered the gold standard in the diagnostic process for IPF, especially in differentiation from other ILDs associated with fibrosis [1,29,31,87,201]. An appropriate assessment of the HRCT image forms the basis of IPF diagnosis.…”