Registries are a useful way to learn about the clinical spectrum of interstitial lung diseases (ILDs) in different parts of the world. Furthermore, they offer data about the worldwide differences in diagnostic approaches and clinical management. Registries have become increasingly accepted as a valid source of data by the medical community.
The incidence and prevalence of ILDs show varying data among epidemiological studies and registries. Disease registries can provide better estimates of the epidemiology as well as offer new insights in the etiology, associated risks, natural history and outcomes of these diseases.
Motivation for centers and pharma
There are a number of incentives for medical centers to participate in registries, namely scientific interest, high standard of these projects, project/team dynamics, authorship (all study centers are named as authors and contributors) and honoraria. For pharmaceutical companies, there are other incentives to motivate a contribution to registries. First there is an altruistic scientific interest to support the IPF community. Furthermore, real-life and long-term data on their own products is needed, such as drug utilisation (dosing, titration, combination, switch, patient groups, etc.), feasibility testing of new protocols, drug safety, and economic analyses.
There are different types of ILD registries in Germany with different goals and approaches. Juergen Behr (Munich, Germany) discussed some aspects of a registry in Germany, called the INSIGHTS-IPF registry, which contains only IPF patients. There is a very high consistency and completeness of the data.
He found a positive association between change in lung physiology (e.g. FVC) and changes in patient-reported outcomes (PROs), such as health-realted quality of life (HRQoL). HRQoL in IPF is measured using different instruments, including generic instruments, such as the St George Respiratory Questionnaire (SGRQ) and SF-36, and disease specific instruments, such as ATAQ-IPF, SGRQ-IPF, and K-BILD.
Longitudinal data examining the effects of disease behaviour on HRQoL is limited. Based on an Australian registry, Ian Glaspole (Footscray, Australia) described some contributing factors to HRQoL at baseline and contributors to longitudinal change in HRQoL. Symptoms, especially dyspnoea, cough and depression, are major contributors ot HRQoL at baseline. The implication according to Glaspole is that therapeutic strategies to improve or maintain HRQoL, should target symptom control. However, there are very limited options to improve symptoms in IPF.
Worsening of symptoms predicts the longitudinal decline in HRQoL. Furthermore, physiological decline is an independent predictor of worsening HRQoL and occurs with increasing disease duration.
ILD registries to date are primarily performed in western countries, in which IPF and ILD associated with connective tissue disease (CTD-ILD) are among the most common forms of ILD. Data regarding ILD burden and demographics for patients in India and South Asia are scarce and may differ substantially due to confounders such as environmental factors, cultural differences, infections and tuberculosis. Ganesh Raghu (Seattle, United States of America) is one of the main initiators of the ILD-India initiative, a prospective registry with well-defined cases of ILD in India. All new onset ILD in patients of Indian origin, who were raised and are living in India, are included.
The data of more than 1,000 consecutive cases of ILD, who were evaluated by participating site investigators at their clinics/sites, provide a snap shot of the categories of ILD in India. The most common pattern of ILD is hypersensitivity pneumonitis (HP), which occured in 47% of patients with ILD. The majority (71.5%) of patients with HP live in urban areas. The demographic profile of patients diagnosed with IPF was found to be similar to that of patients of European and Asian ascent living western and other eastern hemispheres of the world. IPF and CTD-ILD each account for approximately 14% of new onset ILD diagnoses in India, with UIP on HRCT in 89% of IPF cases and 30% of CTD-ILD cases. Rheumatoid arthritis was the most common CTD-ILD, followed by scleroderma.
Finally, Monica Spiteri (Stoke-on-Trent, United Kingdom) discussed the BTS ILD registry, which was launched in February 2013. This registry, as with the other registries, provides an easily accessible, secure system for the national collection of longitudinal demographic and clinical information on two ILD conditions: IPF and sarcoidosis.
Source: Session 415: Interstitial lung disease registry. September 5th, 13:00-14:30, Prince Regent Room Oral Presentation.