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

Interstitial Lung Disease (ILD) Clinical Trial

— EXCITING  

Official title:

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

Clinical Trial Details — Status: Completed

Administrative data

• NCT number: NCT02645968
• Other study ID #: ILD Patient Registry
• Status: Completed
• Start date: May 2014
• Est. completion date: May 2020

Study information

• Verified date: December 2020
• Source: Heidelberg University
• Contact: n/a
• Is FDA regulated: No
• Study type: Observational [Patient Registry]

Clinical Trial Summary

The purpose of this study is 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.

Description:

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.

Following quality procedures are applicable for this registry:

• Quality assurance plan that addresses data validation and registry procedures, including any plans for site monitoring and auditing.

• Data checks to compare data entered into the registry against predefined rules for range or consistency with other data fields in the registry.

• Source data verification to assess the accuracy, completeness, or representativeness of registry data by comparing the data to external data sources (e.g., medical records, paper or electronic case report forms, or interactive voice response systems).

• Standard Operating Procedures to address registry operations and analysis activities, such as patient recruitment, data collection, data management, data analysis, reporting for adverse events, and change management.

• Sample size assessment to specify the number of participants or participant years necessary to demonstrate an effect.

• Plan for missing data to address situations where variables are reported as missing, unavailable, "non-reported," uninterpretable, or considered missing because of data inconsistency or out-of-range results

• Statistical analysis plan describing the analytical principles and statistical techniques to be employed in order to address the primary and secondary objectives, as specified in the study protocol or plan.

Recruitment information / eligibility

• Status: Completed
• Enrollment: 600
• Est. completion date: May 2020
• Est. primary completion date: April 2020
• Accepts healthy volunteers: No
• Gender: All
• Age group: 18 Years and older

Eligibility

Inclusion Criteria:

• Signed informed consent

• Able to read and understand the scope and nature of data collection

• Aged = 18 at time of informed consent

• Patients diagnosed with interstitial lung disease (ILD). Patients with suspicion on interstitial lung disease cannot be included into the registry, only after consens diagnosis of an ILD. This is also the case for patients with not classifiable ILDs.

Subjects with the following subtypes of ILD will be enrolled:

1. Idiopathic interstitial pneumonia (IIPs)

• Idiopathic pulmonary fibrosis (IPF)

• Non-specific interstitial pneumonia (NSIP)

• Desquamative interstitial pneumonia (DIP)

• Respiratory bronchiolitis-associated interstitial lung disease (RB-ILD)

• Cryptogenic organizing pneumonia (COP)

• Lymphocytic interstitial pneumonia (LIP)

• Acute interstitial pneumonia (AIP)

• Rare forms of IIPs (e.g. pleuropulmonary fibroelastosis)

• Not classifiable IIP

2. Granulomatous lung disease

• Sarcoidosis

• Berylliosis

• Other (e.g. involvement in chronic inflammatory liver and gut diseases, except EAA)

3. Hypersensitivity pneumonitis (exogen allergic alveolitis (EAA))

• Farmer's lung

• Bird keepers' lung disease

• Origin unknown

• Other

4. Rheumatic and connective tissue diseases with pulmonary involvement like:

• Connective tissue disease (subtype)

• Vasculitis

• Rheumatoid arthritis

5. Pneumoconiosis

• Asbestosis

• Silicosis

• Other

6. Other forms

• Pulmonary lymphangioleiomyomatosis

• Pulmonary Langerhans' cell histiocytosis

• Pulmonary alveolar proteinosis

• Eosinophilic pneumonia

• Post-ARDS Fibrosis

7. Drug-related

8. Radiotherapy associated

9. Fibrosis in emphysema patients without signs of other ILDs (i.e. smoking related interstitial fibrosis or "emphysemasclerosis")

10. Others

11. Not classifiable

Furthermore for each subtype it will be queried, if a diagnosis of concomitant emphysema in ILD was made.

Exclusion Criteria:

• Patients without interstitial lung disease

Related Conditions & MeSH terms

• Interstitial Lung Disease (ILD)
• Lung Diseases
• Lung Diseases, Interstitial

Locations

Country	Name	City	State
Germany	Universitätsklinikum Aachen, Medizinische Klinik I	Aachen	Nordrhein-Westfalen
Germany	Lungenärztliche Gemeinschaftspraxis Dr. Storz / Dr. Prettner	Böblingen	Baden-Württemberg
Germany	Medizinisches Versorgungszentrum Malteser Bonn gem. GmbH, Malteser Lungen- und Allergiezentrum Bonn	Bonn	Nordrhein-Westfalen
Germany	Johanniter-Krankenhaus Rheinhausen GmbH	Duisburg	Nordrhein-Westfalen
Germany	Ruhrlandklinik - Westdeutsches Lungenzentrum am Universitätsklinikum Essen gGmbH	Essen	Nordrhein-Westfalen
Germany	Klinikum Fulda gAG, Medizinische Klinik V - Pneumologie	Fulda	Hessen
Germany	Pneumologische Gemeinschaftspraxis Fulda Dres. Wiederhold, Schratz, Wissner	Fulda	Hessen
Germany	Universitätsklinikum Gießen und Marburg GmbH, Medizinische Klinik und Poliklinik II	Giessen	Hessen
Germany	Klinkum Gütersloh, Medizinische Klink III, Pneumologie, Schlaf- und Beatmungsmedizin, Infektiologie und Allgemeine Innere Medizin	Gütersloh	Nordrhein-Westfalen
Germany	Thoraxklinik Heidelberg, Abteilung Pneumologie und Beatmungsmedizin	Heidelberg	Baden-Württemberg
Germany	Lungenfachklinik Immenhausen	Immenhausen	Hessen
Germany	Klinikverbund Kempten-Oberallgäu, Klinik für Pneumologie, Thoraxonkologie, Schlaf- und Beatmungsmedizin	Immenstadt	Bayern
Germany	Malteser Krankenhaus St. Hildegardis, Klinik für Pneumologie und Beatmungsmedizin	Köln	Nordrhein-Westfalen
Germany	St. Marien-Hospital GmbH	Köln	Nordrhein-Westfalen
Germany	Gesundheitsverbund Landeskreis Konstanz, Krankenhausbetriebsgesellschaft Konstanz	Konstanz	Baden-Württemberg
Germany	Katholische Kranken- und Pflegeeinrichtung Leverkusen GmbH, St. Remigius Krankenhaus Opladen	Leverkusen	Nordrhein-Westfalen
Germany	framol-med GmbH, Pneumoglische Gemeinschaftspraxis Rheine	Rheine	Nordrhein-Westfalen
Germany	Krankenhaus Bethanien gGmbH, Klinik für Pneumologie und Allergologie, Zentrum für Schlaf- und Beatmungsmedizin	Solingen	Nordrhein-Westfalen
Germany	Klinikum Stuttgart, Klinik für Allgemeine Innere Medizin, Gastroenterologie, Hepatologie und Infektiologie, Pneumologie	Stuttgart	Baden-Württemberg
Germany	Pneumologische Praxis im Zentrum - PiZ, Dr. med. Hans-Paul Eulenbruch und Dr. med. Alexander Rupp	Stuttgart	Baden-Württemberg
Germany	Lungenpraxis Tübingen	Tübingen	Baden-Württemberg
Germany	Pneumologisches Zentrum	Wiesloch	Baden-Württemberg

Sponsors (11)

Lead Sponsor	Collaborator
Heidelberg University	Bundesverband der Pneumologen, German Center for Lung Research, German Respiratory Society, Helmholtz Zentrum München, Institut für Lungenforschung e.V., Lungenfibrose e.V., Sacura GmbH, Syneed Medidata GmbH, University of Giessen, WATL e.V.

Country where clinical trial is conducted

Germany, 

References & Publications (16)

Akgün KM, Crothers K, Pisani M. Epidemiology and management of common pulmonary diseases in older persons. J Gerontol A Biol Sci Med Sci. 2012 Mar;67(3):276-91. doi: 10.1093/gerona/glr251. Epub 2012 Feb 15. Review. — View Citation

Amar RK, Jick SS, Rosenberg D, Maher TM, Meier CR. Drug-/radiation-induced interstitial lung disease in the United Kingdom general population: incidence, all-cause mortality and characteristics at diagnosis. Respirology. 2012 Jul;17(5):861-8. doi: 10.1111/j.1440-1843.2012.02187.x. — View Citation

American Thoracic Society; European Respiratory Society. American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias. This joint statement of the American Thoracic Society (ATS), and the European Respiratory Society (ERS) was adopted by the ATS board of directors, June 2001 and by the ERS Executive Committee, June 2001. Am J Respir Crit Care Med. 2002 Jan 15;165(2):277-304. Review. Erratum in: Am J Respir Crit Care Med2002 Aug 1;166(3):426. — View Citation

Demedts M, Costabel U. ATS/ERS international multidisciplinary consensus classification of the idiopathic interstitial pneumonias. Eur Respir J. 2002 May;19(5):794-6. — View Citation

Demedts M, Wells AU, Antó JM, Costabel U, Hubbard R, Cullinan P, Slabbynck H, Rizzato G, Poletti V, Verbeken EK, Thomeer MJ, Kokkarinen J, Dalphin JC, Taylor AN. Interstitial lung diseases: an epidemiological overview. Eur Respir J Suppl. 2001 Sep;32:2s-16s. Review. — View Citation

Doyle TJ, Hunninghake GM, Rosas IO. Subclinical interstitial lung disease: why you should care. Am J Respir Crit Care Med. 2012 Jun 1;185(11):1147-53. doi: 10.1164/rccm.201108-1420PP. Epub 2012 Feb 23. Review. — View Citation

Ferreira JP, Almeida I, Marinho A, Cerveira C, Vasconcelos C. Anti-ro52 antibodies and interstitial lung disease in connective tissue diseases excluding scleroderma. ISRN Rheumatol. 2012;2012:415272. doi: 10.5402/2012/415272. Epub 2012 Mar 27. — View Citation

Gunnarsson R, Aaløkken TM, Molberg Ø, Lund MB, Mynarek GK, Lexberg AS, Time K, Dhainaut AS, Bertelsen LT, Palm O, Irgens K, Becker-Merok A, Nordeide JL, Johnsen V, Pedersen S, Prøven A, Garabet LS, Gran JT. Prevalence and severity of interstitial lung disease in mixed connective tissue disease: a nationwide, cross-sectional study. Ann Rheum Dis. 2012 Dec;71(12):1966-72. doi: 10.1136/annrheumdis-2011-201253. Epub 2012 May 1. — View Citation

Hallowell RW, Reed RM, Fraig M, Horton MR, Girgis RE. Severe pulmonary hypertension in idiopathic nonspecific interstitial pneumonia. Pulm Circ. 2012 Jan-Mar;2(1):101-6. doi: 10.4103/2045-8932.94842. — View Citation

King TE Jr. Smoking and subclinical interstitial lung disease. N Engl J Med. 2011 Mar 10;364(10):968-70. doi: 10.1056/NEJMe1013966. — View Citation

Kobayashi A, Okamoto H. Treatment of interstitial lung diseases associated with connective tissue diseases. Expert Rev Clin Pharmacol. 2012 Mar;5(2):219-27. doi: 10.1586/ecp.12.9. Review. — View Citation

Kreuter M, Herth FJ, Wacker M, Leidl R, Hellmann A, Pfeifer M, Behr J, Witt S, Kauschka D, Mall M, Günther A, Markart P. Exploring Clinical and Epidemiological Characteristics of Interstitial Lung Diseases: Rationale, Aims, and Design of a Nationwide Prospective Registry--The EXCITING-ILD Registry. Biomed Res Int. 2015;2015:123876. doi: 10.1155/2015/123876. Epub 2015 Nov 10. — View Citation

Lederer DJ, Enright PL, Kawut SM, Hoffman EA, Hunninghake G, van Beek EJ, Austin JH, Jiang R, Lovasi GS, Barr RG. Cigarette smoking is associated with subclinical parenchymal lung disease: the Multi-Ethnic Study of Atherosclerosis (MESA)-lung study. Am J Respir Crit Care Med. 2009 Sep 1;180(5):407-14. doi: 10.1164/rccm.200812-1966OC. Epub 2009 Jun 19. — View Citation

Lynge E. Implication for epidemiology of disease registers. Public Health Rev. 1993-1994;21(3-4):263-70. Review. — View Citation

Raghu G, Collard HR, Egan JJ, Martinez FJ, Behr J, Brown KK, Colby TV, Cordier JF, Flaherty KR, Lasky JA, Lynch DA, Ryu JH, Swigris JJ, Wells AU, Ancochea J, Bouros D, Carvalho C, Costabel U, Ebina M, Hansell DM, Johkoh T, Kim DS, King TE Jr, Kondoh Y, Myers J, Müller NL, Nicholson AG, Richeldi L, Selman M, Dudden RF, Griss BS, Protzko SL, Schünemann HJ; ATS/ERS/JRS/ALAT Committee on Idiopathic Pulmonary Fibrosis. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011 Mar 15;183(6):788-824. doi: 10.1164/rccm.2009-040GL. — View Citation

Tzouvelekis A, Galanopoulos N, Bouros E, Kolios G, Zacharis G, Ntolios P, Koulelidis A, Oikonomou A, Bouros D. Effect and safety of mycophenolate mofetil or sodium in systemic sclerosis-associated interstitial lung disease: a meta-analysis. Pulm Med. 2012;2012:143637. doi: 10.1155/2012/143637. Epub 2012 May 10. — View Citation

* Note: There are 16 references in all — Click here to view all references

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	Determination of characteristics, management and outcomes of patients with different ILDs under clinical practice conditions.	(disease-related) characteristics of ILD patients; diagnostic procedures for the identification of ILDs; the clinical management of different (sub) types of ILD under clinical practice; the outcome of different types of ILDs and of ILD related therapies from all forms of health care facilities	Six month interval over 5 years