Clinical features of sarcoidosis associated pulmonary hypertension: Results of a multi-national registry

Baughman, Robert P.; Shlobin, Oksana A.; Wells, Athol U.; Alhamad, Esam H.; Culver, Daniel A.; Barney, Joseph B.; Cordova, Francis; Carmona, Eva M.; Scholand, Mary Beth; Wijsenbeek, Marlies; Ganesh, Sivagini; Birring, Surinder S.; Kouranos, Vasilis; O'Hare, Lanier; Baran, JoAnne; Cal, Joseph G; Lower, Elyse E.; Engel, Peter J.; Nathan, Steven D.

doi:10.1016/j.rmed.2018.04.015

Cited by 59 publications

(75 citation statements)

References 24 publications

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“…Forced vital capacity (FVC), forced expiratory volume in one second (FEV1), and DLCO are generally lower in SAPH compared with sarcoidosis patients without PH. 6,7,11,18,21,60 DLCO seems to be the strongest independent predictor. 18,21 As mentioned earlier, destruction of the alveoli and loss of pulmonary capillary volume due to fibrosis and muscle cell proliferation are believed to cause reduction of the DLCO.…”

Section: Pulmonary Function Testsmentioning

confidence: 87%

“…7,16 There is no consensus about female/male predominance in SAPH, and seems to vary in different ethnicities. 8,17,18…”

Section: Epidemiologymentioning

confidence: 99%

“…Most often, SAPH is attributed to parenchymal lung disease, generally as a manifestation of advanced fibrosis. However, a considerable number of sarcoidosis patients develop PH in absence of significant parenchymal lung disease 6,11,[18][19][20][21][22] or with near-normal lung function tests. 19,23 This suggests that other or multifactorial mechanisms might cause PH in sarcoidosis.…”

Section: Pathophysiologymentioning

confidence: 99%

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Sarcoidosis-Associated Pulmonary Hypertension

Huitema

Mathijssen

Mager

et al. 2020

Semin Respir Crit Care Med

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Pulmonary hypertension (PH) is a well-known complication of sarcoidosis, defined by a mean pulmonary artery pressure of ≥25 mm Hg. Since both PH and sarcoidosis are rare diseases, data on sarcoidosis-associated PH (SAPH) is retrieved mostly from small retrospective studies. Estimated prevalence of SAPH ranges from 3% in patients referred to a tertiary center up to 79% in patients awaiting lung transplant. Most patients with SAPH show advanced parenchymal disease as the underlying mechanism. However, some patients have disproportional elevated pulmonary artery pressure, and PH can occur in sarcoidosis patients without parenchymal disease. Other mechanisms such as vascular disease, pulmonary embolisms, postcapillary PH, extrinsic compression, and other sarcoidosis-related comorbidities might contribute to SAPH. The diagnosis of PH in sarcoidosis is challenging since symptoms and signs overlap. Suspicion can be raised based on symptoms or tests, such as pulmonary function tests, laboratory findings, electrocardiography, or chest CT. PH screening mainly relies on transthoracic echocardiography. Right heart catheterization should be considered on a case-by-case basis in patients with clinical suspicion of PH, taking into account clinical consequences. Treatment options are considered on patient level in a PH expert center, and might include oxygen therapy, immunosuppressive, or PH-specific therapy. However, qualitative evidence is scarce. Furthermore, in a subset of patients, interventional therapy or eventually lung transplant can be considered. SAPH is associated with high morbidity. Mortality is higher in sarcoidosis patients with PH compared with those without PH, and increases in patients with more advanced stages of sarcoidosis and/or PH.

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Section: Pulmonary Function Testsmentioning

confidence: 87%

“…7,16 There is no consensus about female/male predominance in SAPH, and seems to vary in different ethnicities. 8,17,18…”

Section: Epidemiologymentioning

confidence: 99%

Section: Pathophysiologymentioning

confidence: 99%

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Sarcoidosis-Associated Pulmonary Hypertension

Huitema

Mathijssen

Mager

et al. 2020

Semin Respir Crit Care Med

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“…103 Two recent large registries have reported on SAPH in France, the United States, Europe, and Middle East. 95,105 These two registries found that many patients had moderate-to-severe pulmonary hypertension. Most patients were treated with vasodilator therapy.…”

Section: Therapymentioning

confidence: 99%

Advanced Pulmonary Sarcoidosis

Gupta

Baughman

2020

Semin Respir Crit Care Med

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At least 5% of sarcoidosis patients die from their disease, usually from advanced pulmonary sarcoidosis. The three major problems encountered in advanced pulmonary sarcoidosis are pulmonary fibrosis, pulmonary hypertension, and respiratory infections. Pulmonary fibrosis is the result of chronic inflammation, but other factors including abnormal wound healing may be important. Sarcoidosis-associated pulmonary hypertension (SAPH) is multifactorial including parenchymal fibrosis, vascular granulomas, and hypoxia. Respiratory infections can be cause by structural changes in the lung and impaired immunity due to sarcoidosis or therapy. Anti-inflammatory therapy alone is not effective in most forms of advanced pulmonary sarcoidosis. New techniques, including high-resolution computer tomography and 18F-fluorodeoxyglucose positron emission tomography (PET) have proved helpful in identifying the cause of advanced disease and directing specific therapy.

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“…Pulmonary hypertension (PH) is a well-recognised complication of several ILDs, worsening prognosis and impairing exercise capacity [3][4][5][6][7][8]. Among patients with ILD, PH has been most often studied in IPF, sarcoidosis and hypersensitivity pneumonitis [9][10][11]. The prevalence of PH ranges from 8 up to 86% in patients with IPF, and from 6 up to 74% in patients with sarcoidosis-depending on the diagnostic algorithm, the PH definition, the stage and severity of the underlying disease [4,[12][13][14][15].…”

Section: Introductionmentioning

confidence: 99%

Can a New Scoring System Improve Prediction of Pulmonary Hypertension in Newly Recognised Interstitial Lung Diseases?

Sobiecka¹,

Lewandowska²,

Kober³

et al. 2020

Lung

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Introduction Pulmonary hypertension (PH) is a well-recognised complication of interstitial lung diseases (ILD), which worsens prognosis and impairs exercise capacity. Echocardiography is the most widely used, non-invasive method for PH assessment. The aim of our study was to identify the factors predictive for echocardiographic signs of PH in newly recognised ILD patients. Methods Ninety-three consecutive patients (28F/65M) with different ILD were prospectively evaluated from January 2009 to March 2014. Pulmonary function testing, 6-min walk distance (6MWD), initial and sixth minute room air oxygen saturation, NT-proBNP and echocardiography were assessed in each patient. Echocardiographic PH probability was determined according to the 2009 ESC/ERS guidelines. Results In 41 patients (Group B) increased PH possibility has been diagnosed on echocardiography, in 52 patients (Group A)-low PH probability. Most pronounced differences (p ≤ 0.0005) between groups B and A concerned: age, 6MWD, room air oxygen saturation at 6 min, DLCO and TLC/DLCO index (57.6 vs 43.8 years; 478 vs 583 m; 89.1% vs 93.4%; 54.8% predicted vs 70.5% predicted and 1.86 vs 1.44; respectively). Univariate analysis showed four-fold increased probability of PH when TLC/DLCO exceeded 1.67. A scoring system incorporating age, TLC/DLCO index, 6MWD and room air oxygen saturation at 6 min provided high diagnostic utility, AUC 0.867 (95% CI 0.792-0.867). Conclusion ILD patients with TLC/DLCO index > 1.67 have a high likelihood of PH and should undergo further evaluation. The composite model of PH prediction, including age, 6-min walk test and TLC/DLCO was highly specific for recognition of PH on echocardiography.

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Clinical features of sarcoidosis associated pulmonary hypertension: Results of a multi-national registry

Abstract: The clinical features of SAPH were similar across multiple centers in the US, Europe, and the Middle East. The severity of SAPH was related to reduced DLCO. There were treatment differences between the US and non-US centers.

Cited by 59 publications

References 24 publications

Sarcoidosis-Associated Pulmonary Hypertension

Sarcoidosis-Associated Pulmonary Hypertension

Advanced Pulmonary Sarcoidosis

Can a New Scoring System Improve Prediction of Pulmonary Hypertension in Newly Recognised Interstitial Lung Diseases?

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