Highlights
- •er4-year survival in patients with pulmonary sarcoidosis patients referred to a tertiary centre was 94.6%.
- •Signs of PH on echo, severe fibrotic disease and decline in pulmonary function test are important factors for mortality.
- •The cause of mortality in pulmonary sarcoidosis is heterogeneous including pulmonary, cardiovascular and neurologic causes.
Abstract
Background
Sarcoidosis is a systemic disease of unknown aetiology with significant morbidity
and mortality. The PULSAR study prospectively performed cardiac analysis including
systematic pulmonary hypertension screening in sarcoidosis patients newly referred
to a tertiary sarcoidosis center. In this manuscript we studied the four-year mortality
of this population.
Methods and main findings
Between august 2015 and October 2017, 399 patients (58% male, mean age 49.4 years,
90.5% Caucasian) were included and followed for a mean period of 4.3±0.7 years. In
total, 10 patients had died at the time of analysis. 1-, 2-, 3- and 4-year survival rate was 100% (n=399), 99.0% (n=399), 98.2% (n=399)
and 94.6% (n=276). Most patients died of respiratory failure, other causes were heterogeneous
including cardiac, neurological and non-sarcoidosis origin. A low CPI score or modified
Walsh score was associated with higher mortality, similar for high PH probability
on echocardiography and elevated right ventricular systolic pressure.
Conclusion
This study highlights that elevated RVSP and presence of PH on echocardiography and
progression of fibrotic disease with subsequent decline in pulmonary function test
are important factors for mortality in sarcoidosis patients.
To read this article in full you will need to make a payment
Purchase one-time access:
Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online accessOne-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:
Subscribe to Respiratory MedicineAlready a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
References
- Sarcoidosis mortality in Sweden: a population-based cohort study.Eur. Respir. J. European Respiratory Society. 2018; : 51
- Sarcoidosis-related mortality in the United States from 1988 to 2007.Am. J. Respir. Crit. Care Med. 2011; 183: 1524-1530
- Predictors of Mortality in Pulmonary Sarcoidosis. vol. 153. Chest Elsevier Inc, 2018: 105-113
- Survival in sarcoidosis-associated pulmonary hypertension: the importance of hemodynamic evaluation.Chest American College of Chest Physicians. 2010; 138: 1078-1085
- Predictors of Mortality in Fibrosing Pulmonary Sarcoidosis.Respir. Med. W.B. Saunders Ltd, 2020: 169
- Stage IV sarcoidosis: comparison of survival with the general population and causes of death.Eur. Respir. J. 2011; 38: 1368-1373
- Management and long-term outcomes of sarcoidosis-associated pulmonary hypertension.Eur. Respir. J. 2017; 50: 1700465
- Prevalence of pulmonary hypertension in pulmonary sarcoidosis; the first large European prospective study.Eur. Respir. J. 2019; : 1900897
- ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension.Eur. Heart J. 2015. 2015; 37: 67-119
- HRS expert consensus statement on the diagnosis and management of arrhythmias associated with cardiac sarcoidosis.Heart Rhythm United States. 2014; 11: 1305-1323
- An integrated clinicoradiological staging system for pulmonary sarcoidosis: a case-cohort study.Lancet Respir. Med. 2014; 2: 123-130
- Physiological predictors of survival in patients with sarcoidosis-associated pulmonary hypertension: results from an international registry.Eur. Respir. J. 2020; 55: 1901747
Article info
Publication history
Published online: February 20, 2022
Accepted:
February 6,
2022
Received in revised form:
January 16,
2022
Received:
October 1,
2021
Identification
Copyright
© 2022 Elsevier Ltd. All rights reserved.
