Organisation of diagnosis and treatment of idiopathic pulmonary fibrosis and other interstitial lung diseases in the Nordic countries

Introduction: Differences in the organisation of idiopathic pulmonary fibrosis (IPF) and interstitial lung diseases (ILDs) in the Nordic countries are not well described. Diagnostic setups, treatment modalities and followup plans may vary due to national, cultural and epidemiological features. The aim of the present study was to describe the different organisation of diagnostics and treatment of IPF and ILD in the Nordic countries. Methods: All university and regional hospitals with respiratory physicians were invited to respond to a questionnaire collecting data on the number of physicians, nurses, patients with ILD/IPF, the presence of and adherence to disease-specific national and international guidelines, diagnosis and treatment including ILD-specific palliation and rehabilitation programmes. Results: Twenty-four university and 22 regional hospitals returned the questionnaire. ILD and IPF incidence varied between 1.4 and 20/100,000 and 0.4 and 10/100,000, respectively. Denmark and Estonia have official national plans for the organisation of ILD. The majority of patients are managed at the university hospitals. The regional hospitals each manage 46 (5 - 200) patients with ILD and 10 (0 - 20) patients with IPF. There are from one to four ILD centres in each country with a median of two ILD specialists employed. Specialised ILD nurses are present in nine hospitals. None of the Nordic countries have national guidelines made by health authorities. The respiratory societies in Sweden, Norway and Denmark have developed national guidelines. All hospitals except two use the ATS/ERS/JRS/ALAT IPF guidelines from 2011. The limited number of ILD specialists, ILD-specialised radiologists and pathologists and the low volume of ILD centres were perceived as bottlenecks for implementation of guidelines. Twenty of the 24 university hospitals have multidisciplinary conferences (MDCs). Pulmonologists and radiologists take part in all MDCs while pathologists only participate at 17 hospitals. Prescription of pirfenidone is performed by all university hospitals except in Estonia. Triple therapy with steroid, azathioprine and N-acetylcysteine is not used. No hospitals have specific palliation programmes for patients with ILD/IPF, but 36 hospitals have the possibility of referring patients for palliative care, mostly based on existing oncology palliative care teams; seven hospitals have rehabilitation programmes for ILD. Conclusion: There are obvious differences between the organisations of ILD patients in the Nordic countries. We call for national plans that consider the challenge of cultural and geographical differences and suggest the establishment of national reference centres and satellite collaborative hospitals to enable development of common guidelines for diagnostics, therapy and palliation in this patient group.