Prevalence of sarcoidosis-associated pulmonary hypertension: cumulative analysis of two PULSAR studies

Abstract

We have read with great interest the study by HUITEMA et al. [1] showing a pulmonary hypertension (PH) prevalence of around 3% in a large cohort of predominantly Caucasian sarcoidosis patients in a Dutch tertiary centre. According to the authors, a European Caucasian cohort has never been studied before this PULmonary hypertension in pulmonary SARcoidosis (PULSAR) study. In this context, in 2013 we reported another PULSAR study (PULmonary hypertension in SARcoidosis) in an albeit smaller cohort from a German tertiary centre [2]. Of interest, we showed very similar results, with a prevalence of precapillary PH of 3.6% with a similar study design in Caucasian sarcoidosis patients. Taken together, we think that it is very remarkable that both PULSAR studies confirm consistent prevalences and a cumulative analysis of both PULSAR trials would demonstrate sarcoidosis-associated pulmonary hypertension (SAPH) proven by right heart catheter in 15/510 patients and precapillary PH in 13/510 patients (table 1). The strength of both PULSAR studies is the prospective design and the invasive measurement of haemodynamics in most patients with suspicion of PH in transthoracic echocardiography; therefore, we would propose to use these algorithms to evaluate PH in sarcoidosis patients with unexplained dyspnoea. Echocardiography can show different signs of PH, before the definitive diagnosis for PH should be made by right heart catheterisation. Regarding the advanced stages with fibrosis in a relevant part of SAPH patients in both studies, we think that in these Caucasian cohorts the obliteration of the vascular bed by parenchymal fibrosis is most often the cause; however, the role of different factors, including pulmonary vascular compression due to adenopathy, granulomatous arteritis, pulmonary veno-occlusive disease, portopulmonary hypertension and postcapillary PH due to cardiac sarcoidosis, remains open. As we know that PH is a complication of advanced pulmonary sarcoidosis and it is associated with increasing morbidity and mortality, it is important to increase the awareness of SAPH and also to identify these different phenotypes.

Prevalence of Sarcoidosis-Associated Pulmonary Hypertension: A Systematic Review and Meta-Analysis

“…Of these, 49 studies were subsequently excluded for a lack of usable data. Three studies were removed for duplicated data used in another study ( 13 , 20 , 21 ). Finally, the analysis included 25 studies ( 7 , 11 , 12 , 22 – 43 ).…”

“…However, the results are somewhat different for the limited sample sizes and these studies are individually underpowered to effectively address this issue. Although there are relevant literatures describing and discussing this topic ( 13 , 14 ), there are no meta-analysis reporting the epidemiology of SAPH globally. Therefore, we performed a systematic review and meta-analysis to comprehensively calculate the prevalence of SAPH in general and advanced sarcoidosis populations.…”

Healthcare resource utilization and quality of life in patients with sarcoidosis‐associated pulmonary hypertension

“… 2 , 4 , 6 Estimates from prospective screening studies on the prevalence of PH in patients with sarcoidosis range from 3% to 6% when PH is confirmed by right heart catheterization (RHC) and from 6% to 21% for PH suspected by echocardiography. 2 , 7 , 8 , 9 , 10 , 11 , 12 …”

“…2,4,6 Estimates from prospective screening studies on the prevalence of PH in patients with sarcoidosis range from 3% to 6% when PH is confirmed by right heart catheterization (RHC) and from 6% to 21% for PH suspected by echocardiography. 2,[7][8][9][10][11][12] Patients with sarcoidosis-associated pulmonary hypertension (SAPH) most frequently present with persistent or unexplained dyspnea, [13][14][15][16] which has a negative impact on patients' health-related quality of life (HRQoL). 2,17,18 Moreover, the development of SAPH is independently associated with a 7-to 10-fold increase in the risk of death compared to patients who have sarcoidosis without PH.…”

“…Pulmonary hypertension (PH) is a well‐recognized and serious but poorly defined complication of sarcoidosis 2,4,6 . Estimates from prospective screening studies on the prevalence of PH in patients with sarcoidosis range from 3% to 6% when PH is confirmed by right heart catheterization (RHC) and from 6% to 21% for PH suspected by echocardiography 2,7–12 …”