Abstract
Introduction
Sarcoidosis is a rare inflammatory disease with unclear natural history. Using a large,
retrospective, longitudinal, population-based cohort, we sought to define its natural
history in order to guide future clinical studies.
Methods
We identified 722 newly diagnosed cases of sarcoidosis within Kaiser Permanente Northwest
health care records between 1995 and 2015. We investigated immunosuppressive medication
use in the two years following diagnosis, analyzed demographic and clinical characteristics,
and quantified chest imaging and pulmonary function testing (PFTs) across the clinical
course.
Results
Over two years of follow-up, 41% of patients were treated with prednisone. Of those,
75% tapered off their first course within 100 days, although half of those patients
required recurrent therapy. Five percent of the entire cohort remained on prednisone
for longer than one year, with an average daily dose of 10–20 mg. Chest imaging was
associated with early prednisone use, and chest CT was associated with changes in
prednisone dose. PFTs or demographics were not associated with prednisone use. Cumulative
prednisone doses were significantly higher in African Americans (1,845 mg additional)
and those who had a chest CT (2,015 mg additional). Overall, PFTs were less frequently
obtained than chest imaging and had no significant change over disease course.
Discussion
The natural history of sarcoidosis varies greatly. For those requiring therapy, corticosteroid
burden is high. Chest imaging drives medication dose changes as compared to PFTs,
but neither outcome fully captures the entire history of disease. Prospective cohorts
are needed with purposefully collected, repeated measures that include objective clinical
assessments and symptoms.
Keywords
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References
- From scientific discovery to treatments for rare diseases - the view from the national center for advancing translational sciences - office of rare diseases research.Orphanet J. Rare Dis. 2018; 13 (Epub 2018/11/08, PubMed PMID: 30400963; PMCID: PMC6219030): 196https://doi.org/10.1186/s13023-018-0936-x
- Diagnosis and detection of sarcoidosis. An official American thoracic society clinical practice guideline.Am. J. Respir. Crit. Care Med. 2020; 201 (Epub 2020/04/16, PubMed PMID: 32293205; PMCID: PMC7159433): e26-e51https://doi.org/10.1164/rccm.202002-0251ST
- Outcome of sarcoidosis.Clin. Chest Med. 2008; 29 (x. Epub 2008/06/10, PubMed PMID: 18539245): 565-574https://doi.org/10.1016/j.ccm.2008.03.006
- Sarcoidosis of the lung. Natural history and effects of treatment.Sarcoidosis. 1987; 4 (Epub 1987/03/01. PubMed PMID: 3589194): 64-67
FDA. Rare Diseases: Natural History Studies for Drug Development 2019.
- Endpoints for clinical trials of sarcoidosis.Sarcoidosis Vasc. Diffuse Lung Dis. 2012; 29 (Epub 2013/03/07. PubMed PMID: 23461070): 90-98
- Negative clinical trials in sarcoidosis: failed therapies or flawed study design?.Eur. Respir. J. 2014; 44 (Epub 2014/11/02, PubMed PMID: 25362122): 1123-1126https://doi.org/10.1183/09031936.00156314
- Sarcoidosis from bench to bedside: a state-of-the-art series for the clinician.Eur. Respir. J. 2012; 40 (Epub 2012/07/04, PubMed PMID: 22753834): 14-16https://doi.org/10.1183/09031936.00070712
- Excessive thoracic computed tomographic scanning in sarcoidosis.Thorax. 1995; 50 (Epub 1995/12/01, PubMed PMID: 8553298; PMCID: PMC1021348): 1264-1266https://doi.org/10.1136/thx.50.12.1264
- Changes in chest roentgenogram of sarcoidosis patients during a clinical trial of infliximab therapy: comparison of different methods of evaluation.Chest. 2009; 136 (Epub 2009/04/28, PubMed PMID: 19395578): 526-535https://doi.org/10.1378/chest.08-1876
- The utility of the chest radiograph in diagnosing exacerbations of pulmonary sarcoidosis.Respirology. 2008; 13 (Epub 2008/01/17, PubMed PMID: 18197917): 97-102https://doi.org/10.1111/j.1440-1843.2007.01206.x
- Radiomic measures from chest high-resolution computed tomography associated with lung function in sarcoidosis.Eur. Respir. J. 2019; 54 (Epub 2019/06/15, PubMed PMID: 31196947; PMCID: PMC7261239)https://doi.org/10.1183/13993003.00371-2019
- Recurrent CT, cumulative radiation exposure, and associated radiation-induced cancer risks from CT of adults.Radiology. 2009; 251 (Epub 2009/04/01, PubMed PMID: 19332852): 175-184https://doi.org/10.1148/radiol.2511081296
- Toxicity risk from glucocorticoids in sarcoidosis patients.Respir. Med. 2017; 132 (Epub 2017/12/13, PubMed PMID: 29229111): 9-14https://doi.org/10.1016/j.rmed.2017.09.003
- Bone health issues in sarcoidosis.Curr. Rheumatol. Rep. 2011; 13 (Epub 2011/02/18, PubMed PMID: 21327743; PMCID: PMC3311464): 265-272https://doi.org/10.1007/s11926-011-0170-1
- Adverse outcomes from initiation of systemic corticosteroids for asthma: long-term observational study.J. Asthma Allergy. 2018; 11 (Epub 2018/09/15, PubMed PMID: 30214247; PMCID: PMC6121746): 193-204https://doi.org/10.2147/JAA.S176026
- Risk of cardiovascular disease among patients with sarcoidosis: a population-based retrospective cohort study, 1976-2013.Eur. Respir. J. 2017; 49 (Epub 2017/02/10, PubMed PMID: 28182567; PMCID: PMC5564441)https://doi.org/10.1183/13993003.01290-2016
- The effect of corticosteroids on quality of life in a sarcoidosis clinic: the results of a propensity analysis.Respir. Med. 2015; 109 (Epub 2015/02/24, PubMed PMID: 25698652; PMCID: PMC4447298): 526-531https://doi.org/10.1016/j.rmed.2015.01.019
- Presenting characteristics as predictors of duration of treatment in sarcoidosis.QJM. 2006; 99 (Epub 2006/04/06, PubMed PMID: 16595563): 307-315https://doi.org/10.1093/qjmed/hcl038
- An approach to the treatment of pulmonary sarcoidosis with corticosteroids: the six phases of treatment.Chest. 1999; 115 (Epub 1999/04/20, PubMed PMID: 10208222): 1158-1165https://doi.org/10.1378/chest.115.4.1158
- Outcome in sarcoidosis. The relationship of relapse to corticosteroid therapy.Chest. 1997; 111 (Epub 1997/03/01, PubMed PMID: 9118698): 623-631https://doi.org/10.1378/chest.111.3.623
- Relapses of sarcoidosis: what are they and can we predict who will get them?.Eur. Respir. J. 2014; 43 (Epub 2014/02/04, PubMed PMID: 24488991): 337-339https://doi.org/10.1183/09031936.00138913
- Daily home spirometry to detect early steroid treatment effects in newly treated pulmonary sarcoidosis.Eur. Respir. J. 2018; 51
- New advances in the management of pulmonary sarcoidosis.bmj. 2019; : 367
- ERS clinical practice guidelines on treatment of sarcoidosis.Eur. Respir. J. 2021; (In press)2004079https://doi.org/10.1183/13993003.04079-2020
Article info
Publication history
Published online: September 24, 2021
Accepted:
September 14,
2021
Received in revised form:
August 31,
2021
Received:
May 11,
2021
Identification
Copyright
© 2021 Elsevier Ltd. All rights reserved.
