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European MultiPartner ILD Registry

About the project

EMPIRE-ILD is a prospective, international, multicentre, observational, non-interventional, open-label, non-randomised registry of adult patients with ILDs specified below. Patients treated by all available treatment modalities will are included in the registry.

The project builds on the previous 8-year EMPIRE project, which was focused on idiopathic pulmonary fibrosis and resulted in an expert collaboration of several dozens of centres from 11 countries in Central and South-Eastern Europe and Asia. The aim of EMPIRE-ILD is to extend the collection of real-life data to other diagnoses, taking advantage of the unique community and linkages established in previous years. ILDs epidemiology, clinical practice and response to treatment is monitored. Other objectives include describing real-life approach to ILDs in various countries regarding diagnostic algorithms, treatment patterns and management, and treatment outcomes.

Interstitial lung disease

Interstitial lung diseases (ILDs) are a heterogeneous collection of distinctive lung disorders classified on the grounds of shared clinical, radiographic, physiologic or pathologic factors. It concerns alveolar epithelium, pulmonary capillary endothelium, basement membrane, and perivascular and perilymphatic tissues. It may occur when an injury to the lungs triggers an abnormal healing response. The disease presents itself with the following symptoms: shortness of breath, non-productive coughing, fatigue, and weight loss, which tend to develop slowly, over several months.

ILD occurs either as a primary (idiopathic) disease, or as a result of external factors (environmental and occupational exposure, drugs) or other disease (connective tissue diseases and vasculitides).

Treatment for ILDs was for a long time only empirical, based on anti-inflammatory and immunosuppressive drugs with no chance to influence fibroproliferative healing in the lungs. The potential to treat ILDs with progressive fibrosis appeared only in the last decade when 2 antifibrotic drugs for idiopathic pulmonary fibrosis appeared and only 5 years ago the antifibrotic effect of nintedanib was approved also in progressive pulmonary fibrosis of other origin. However, the randomized clinical trial showed only a short-term effect of nintedanib and pirfenidone and only real-world experience could show the long-term effect of antifibrotics on the survival of IPF patients and also offer insight on epidemiology and clinical diversity of this disease. 

All the facts described above illustrate that clinical research on ILDs is needed at all levels: from basic research through randomised clinical trials to follow-up in clinical practice, e.g., through clinical registries. Despite the limitations resulting from non-interventional observation of patients in real-life settings, registries can provide answers to many questions that would be difficult to answer in clinical trials, such as the determination of risk and prognostic factors, the effect of treatment in less numerous patient groups, or the monitoring of different treatment sequences.

Progressive pulmonary fibroses

Progressive pulmonary fibrosis (PPF) is a consequence of many ILDs with fibroproliferation as a pathogenetic feature of the disease. The main representative is IPF however there is in fact a big group of other fibrotic ILDs of different aetiology and clinical presentation which can have under some circumstances the same outcome as IPF, i.e. relentlessly progressive fibrosis. Antifibrotic treatment with nintedanib can have an effect on slowing down progression and prolonging survival in these ILDs as well, however there is not enough data on long term effect of this treatment from randomised controlled trials. The registries are thus a source of valuable data for longitudinal clinical and epidemiological studies in PPFs,

Idiopathic and familial pulmonary fibrosis

Idiopathic pulmonary fibrosis (IPF) is a progressive, irreversible, and ultimately fatal disease of unknown aetiology. It is characterised by diffuse progressive fibrosis of the lung, decreasing lung volumes and respiratory insufficiency and failure. The outcome of IPF is relatively uniform, but the course of progression varies from patient to patient. The classic clinical phenotype of IPF is a slowly progressive decline in lung function and worsening dyspnoea leading to death within 2–5 years of diagnosis. The introduction of antifibrotic (AF) treatment has led to significant improvements in IPF patients’ outcomes.

Idiopathic interstitial pneumonias other than IPF

Other idiopathic interstitial pneumonias (IIPs) include various diseases with different clinical courses, such as acute interstitial pneumonia (AIP), nonspecific interstitial pneumonia (NSIP), smoking-related IIPs (desquamative interstitial pneumonia, DIP, and respiratory bronchiolitis-associated interstitial lung disease, RBILD) and pleuroparenchymal fibroelastosis (PPFE). They can have also progressive course characterized by fibroproliferative healing, especially fibrotic NSIP and PPFE where only antifibrotics may be of help.

Hypersensitivity pneumonitis

Hypersensitivity pneumonitis (HP) classified as an interstitial lung disease is characterized by a complex immunological reaction of the lung parenchyma in response to repetitive inhalation of a sensitized allergen, such as microorganisms, plant and animal proteins or chemicals. More than 300 antigens that span over a wide range of occupations have been described to cause HP. According to biologic behaviour of the disease we recognize two types, fibrosing and non-fibrosing HP. While diagnosis and treatment of nonfibrotic HP poses usually not a problem, the diagnosis of fibrotic one is more complicated, and treatment and prognosis resembles that of IPF. Presence of pulmonary fibrosis portends a poor prognosis with a median survival of 3 to 5 years and the treatment with antifibrotics is usually the only chance of these patients for al longer survival. For an advanced disease, lung transplant may be the only viable option.

Connective tissue disease-associated interstitial lung disease

Connective tissue disease (CTD) related interstitial lung disease (CTD-ILD) is one of the leading causes of morbidity and mortality of CTD. Clinically, CTD-ILD is highly heterogenous and involves rheumatic immunity and multiple manifestations of respiratory complications affecting the airways, vessels, lung parenchyma, pleura, and respiratory muscles. Currently, the diagnosis of CTD-ILD is primarily based on distinct pathology subtype(s), imaging, as well as related CTD and autoantibodies profiles. Compared with idiopathic interstitial pneumonias, CTD-ILD is associated with a more favourable prognosis and, in some cases, may respond to immunosuppressive therapy. However, the fibrotic CTD-ILDs are not influenced by anti-inflammatory drugs and the only chance in case of progressive fibrosis is treatment with antifibrotics, namely nintedanib.

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