Design and rationale of ProSar, the first Danish sarcoidosis registry

Main Article Content

Janne Møller
Ole Hilberg https://orcid.org/0000-0002-3075-3463
Elisabeth Bendstrup https://orcid.org/0000-0002-4238-6963

Keywords

Sarcoidosis, registry, epidemiology

Abstract

Background: The clinical presentation and disease course in sarcoidosis are highly heterogenic and varies among ethnic groups. Incidence and prevalence of sarcoidosis in Scandinavian countries are high. Though sarcoidosis is generally a benign disease, the overall risk of death in individuals with sarcoidosis is higher compared with the general population. Information on epidemiology, health status, treatment, distinct phenotypes, genotypes and course of sarcoidosis is limited. Systematic data registration is important to characterize patients with sarcoidosis and to enhance the understanding of the disease. To address this, we developed the first Danish sarcoidosis registry, ProSar.


Objective: To describe the design and rationale of the noninterventional, prospective, and observational Danish sarcoidosis registry.


Methods: The registry was designed by respiratory physicians in collaboration with specialist representatives from the fields of extrapulmonary organ involvement. The registry includes individual data on patient demographics, family history, referral patterns, symptoms, pulmonary and extra-pulmonary organ involvement, comorbidities, treatment and diagnostic investigations as well as patient reported outcome measures relevant to sarcoidosis. Eligible patients are Danish adults diagnosed with sarcoidosis able to read and understand Danish.


Conclusion: The first Danish sarcoidosis registry, ProSar, will collect prospective epidemiological data including clinical characteristics, referral patterns, disease trajectories and treatment pathways in Danish patients with sarcoidosis. The registry is expected to facilitate improved medical management and outcome for patients and future sarcoidosis research.

Downloads

Download data is not yet available.
Abstract 11 |

References

1. Byg KE, Milman N HS. Sarcoidosis in Denmark 1980-1994. A registry-based incidence study comprising 5536 patients. Sarcoidosis Vasc Diffus Lung Dis. 2003;20:46–52.
2. Arkema E V., Grunewald J, Kullberg S, Eklund A, Askling J. Sarcoidosis incidence and prevalence: A nationwide register-based assessment in Sweden. Eur Respir J [Internet]. 2016;48(6):1690–9. Available from: http://dx.doi.org/10.1183/13993003.00477-2016
3. Gade M, Løkke A, Hilberg O. Mortality Among Patients with Sarcoidosis: A Nationwide Population-Based Study. 2020. 7780–7780 p.
4. Kearney GD, Obi ON, Maddipati V, Mohan A, Malur A, Carter JC, et al. Sarcoidosis deaths in the United States: 1999–2016. Respir Med. 2019;149(November 2018):30–5.
5. Noble PW, Albera C, Bradford WZ, Costabel U, Glassberg MK, Kardatzke D, et al. Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): Two randomised trials. Lancet [Internet]. 2011;377(9779):1760–9. Available from: http://dx.doi.org/10.1016/S0140-6736(11)60405-4
6. Richeldi L, Du Bois RM, Raghu G, Azuma A, Brown KK, Costabel U, et al. Efficacy and Safety of Nintedanib in Idiopathic Pulmonary Fibrosis. n engl j med. 2014;22:2071–82.
7. Behr J, Kreuter M, Hoeper MM, Wirtz H, Klotsche J, Koschel D, et al. Management of patients with idiopathic pulmonary fibrosis in clinical practice: the INSIGHTS-IPF registry. Available from: http://ow.ly/JbWRn
8. Hansen S, Hoffmann-Petersen B, Sverrild A, Bräuner E V., Lykkegaard J, Bodtger U, et al. The Danish National Database for Asthma: establishing clinical quality indicators. Eur Clin Respir J. 2016;3(1):33903.
9. Culver DA, Urgen Behr J¨, Belperio JA, Corte TJ, De Andrade JA, Flaherty KR, et al. CONCISE CLINICAL REVIEW Patient Registries in Idiopathic Pulmonary Fibrosis. 2019; Available from: www.clinicaltrials.gov
10. Kreuter M, Herth FJF, Wacker M, Leidl R, Hellmann A, Pfeifer M, et al. 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 [Internet]. 2015;2015. Available from: http://dx.doi.org/10.1155/2015/123876
11. Moore I, Wrobel J, Rhodes J, Lin Q, Webster S, Jo H, et al. Australasian interstitial lung disease registry (AILDR): Objectives, design and rationale of a bi-national prospective database. BMC Pulm Med. 2020;20(1):1–10.
12. Thillai M, Chang W, Chaudhuri N, Forrest I, Ho L-P, Lines S, et al. Sarcoidosis in the UK: insights from British Thoracic Society registry data. BMJ Open Resp Res [Internet]. 2019;6:357. Available from: http://dx.doi.org/10.1136/bmjresp-2018-000357
13. Bendstrup E, Yde AK, Hyldgaard C, Shaker S, Davidsen JR. DANILDA – The First Danish National Interstitial Lung Disease Registry. Eur Respir J [Internet]. 2019 Sep 28;54(suppl 63):PA4723. Available from: http://erj.ersjournals.com/content/54/suppl_63/PA4723.abstract
14. Hyldgaard C, Hilberg O, Muller A, Bendstrup E. A cohort study of interstitial lung diseases in central Denmark. Respir Med [Internet]. 2014;108(5):793–9. Available from: http://dx.doi.org/10.1016/j.rmed.2013.09.002
15. Crouser ED, Maier LA, Wilson KC, Bonham CA, Morgenthau AS, Patterson KC, et al. AMERICAN THORACIC SOCIETY Diagnosis and Detection of Sarcoidosis An Of fi cial American Thoracic Society Clinical Practice Guideline. 2020;201(8).
16. Patel AS, Siegert RJ, Creamer D, Larkin G, Maher TM, Renzoni EA, et al. The development and validation of the King’s Sarcoidosis Questionnaire for the assessment of health status. Thorax. 2013;68(1):57–65.
17. De Vries J, Michielsen H, Van Heck GL, Drent M. Measuring fatigue in sarcoidosis: the Fatigue Assessment Scale (FAS). Br J Health Psychol. 2004 Sep;9(Pt 3):279–91.
18. Hoitsma E, De Vries J, Drent M. The small fiber neuropathy screening list: Construction and cross-validation in sarcoidosis. 2011;

Most read articles by the same author(s)