Registry for Exploring Clinical and Epidemiological Characteristics of Interstitial Lung Diseases - EXCITING

Interstitial lung diseases (ILD) are a heterogeneous group of more than 200 entities that can be idiopathic or as a result of other diseases and/or treatments. All forms of ILD are considered to be seldom or very seldom (and most of them are so-called "orphan" diseases), in their entirety they seem to represent an epidemiological burden, which should not be underestimated, e.g. as "normal" symptoms of the elderly.

The knowledge about the epidemiology of ILDs is very limited, in general and in Germany in particular. For Germany no current, valid and comprehensive population-based data on ILDs exist because no appropriate ILD classification was available for a long time. The epidemiological data, which were collected before the introduction of the current classification of ILD according to the ATS/ERS consensus and before HRCT was considered the diagnostic gold standard, are not very reliable. Furthermore systemic investigations relating to the development of an ILD of risk groups, such as patients undergoing radio- or chemotherapy or patients with connective tissue disease, are restricted.

Thus, only little is known about costs associated with ILDs. Idiopathic Pulmonary Fibrosis (IPF), as one of the disease entities, was shown to be associated with high healthcare utilization and costs in England and the USA, as in many cases lung transplantation is the only therapeutic option and also new pharmaceutical treatments are costly. But there is a lack of data on healthcare utilization and financial consequences of other ILD than IPF in Germany. Therefore, this registry will provide an important database for health economics and health services research questions.

Against this background, this multi-centre, non interventional prospective observational disease and outcomes registry for ILD aims to collect valid data from all forms of health care on the incidence, prevalence, the regional distribution, characteristics, management and outcomes of different ILDs under clinical practice conditions. In detail, demographic data such as gender, age, country of birth, place of residence; disease related data such as subtype of ILD, treatment center, diagnostic procedures (e.g. HRCT, lung biopsy, pulmonary function tests); risk factors (e.g. smoking status, profession, familial ILD); co-morbidities; ILD management as analysed by the kind and frequency of pharmacological and non-pharmacological therapies; outcome; and consumption of resources will be recorded. Therefore, this registry will allow obtaining a comprehensive and current epidemiologic report for ILDs in Germany and can be particularly helpful in uncovering correlations between risk exposure and disease development.

This study, with up to 100 sites, initially focused on the federal states Baden-Wuerttemberg and Hesse, is planned for a minimum of 5 years and 600 patients should be enrolled as part of the study a screening/baseline visit, on which the Informed Consent Form will be signed, and every 6 months regularly updates/phone contacts will be performed.