Treatment of sarcoidosis-associated pulmonary hypertension: so close, and yet so far

Baughman, Robert P.; Shlobin, Oksana A.

doi:10.1183/13993003.01725-2017

Cited by 3 publications

(1 citation statement)

References 19 publications

(19 reference statements)

Supporting

Mentioning

Contrasting

Order By: Relevance

“…[5][6] Relevant studies have pointed out that the influencing factors of pathogenesis of pulmonary hypertension include hypoxia, genetic immunity, congenital, smoking and so on. Therefore, the treatment of pulmonary hypertension should also start from the above factors [7][8]. In recent years, the research on pulmonary hypertension in China has focused on the pathogenesis and diagnosis and treatment, and made significant progress [9][10].…”

Section: Introductionmentioning

confidence: 99%

Clinical Study on Pulmonary Hypertension Caused by Genetic Immunity and Environment

et al. 2020

View full text Add to dashboard Cite

Pulmonary hypertension is a chronic pulmonary vascular disease. In recent years, quite a number of studies suggest that the increase of pulmonary artery pressure is the result of multiple factors. A better understanding of the related factors is of great significance to the prevention, diagnosis, treatment and prognosis of pulmonary hypertension. Based on this, this paper studies the genetic immunity and environmental induced pulmonary hypertension. In this paper, 60 patients with pulmonary hypertension in our hospital from January 2020 to the first half of July 2020 were selected as the research objects. According to the pathogenic factors, the patients were divided into groups. Among them, pulmonary hypertension caused by genetic factors was group A, and pulmonary hypertension caused by environmental factors was group B. This study found that the incidence rate of family genetic history reached 6%, and the proportion of diseases caused by immune factors accounted for 2%. Because of the high altitude hypoxia environment, about 5% accounted for 5%. Smoking is the main factor leading to the morbidity of pulmonary hypertension, accounting for up to 70%. The results of this study provide theoretical basis for the treatment of pulmonary hypertension caused by genetic immunity and environment.

show abstract

Section: Introductionmentioning

confidence: 99%

Clinical Study on Pulmonary Hypertension Caused by Genetic Immunity and Environment

et al. 2020

View full text Add to dashboard Cite

show abstract

Clinical Features and Outcomes of Patients with Sarcoidosis-associated Pulmonary Hypertension

Parikh

Dahhan

Nicholl

et al. 2019

Sci Rep

View full text Add to dashboard Cite

The presence of pulmonary hypertension (PH) significantly worsens outcomes in patients with advanced sarcoidosis, but its optimal management is unknown. We aimed to characterize a large sarcoidosis-associated pulmonary hypertension (SAPH) cohort to better understand patient characteristics, clinical outcomes, and management strategies including treatment with PH therapies. Patients at Duke University Medical Center with biopsy-proven sarcoidosis and SAPH confirmed by right heart catheterization (RHC) were identified from 1990–2010. Subjects were followed for up to 11 years and assessed for differences by treatment strategy for their SAPH, including those who were not treated with PH-specific therapies. Our primary outcomes of interest were change in 6-minute walk distance (6MWD) and change in N-terminal pro-brain natriuretic peptide (NT-proBNP) by after therapy. We included 95 patients (76% women, 86% African American) with SAPH. Overall, 70% of patients had stage IV pulmonary sarcoidosis, and 77% had functional class III/IV symptoms. Median NT-proBNP value was elevated (910 pg/mL), and right ventricular dysfunction was moderate/severe in 55% of patients. Median values for mean pulmonary artery pressure (49 mmHg) and pulmonary vascular resistance (8.5 Woods units) were consistent with severe pulmonary hypertension. The mortality rate over median 3-year follow-up was 32%. Those who experienced a clinical event and those who did not had similar overall echocardiographic findings, hemodynamics, 6MWD and NT-proBNP at baseline, and unadjusted analysis showed that only follow-up NT-proBNP was associated with all-cause hospitalization or mortality. A sign test to evaluate the difference between NT-Pro-BNP before and after PH therapy produced evidence that a significant difference existed between the median pre- and post-NT-Pro-BNP (−387.0 (IQR: −1373.0-109), p = 0.0495). Use of PH-specific therapy may be helpful in selected patients with SAPH and pre-capillary pulmonary vascular disease. Prospective trials are needed to characterize responses to PH-specific therapy in this subset of patients with SAPH.

show abstract

Balloon pulmonary angioplasty – efficient therapy of chronic thromboembolic pulmonary hypertension in the patient with advanced sarcoidosis – a case report

et al. 2018

View full text Add to dashboard Cite

BackgroundApproximately a quarter of patients with advanced sarcoidosis develop pulmonary hypertension (PH), which affects their prognosis. We report unusual case of confirmed chronic thromboembolic pulmonary hypertension (CTEPH) in a patient with stage IV sarcoidosis successfully treated with balloon pulmonary angioplasty (BPA).Case presentationA 65 years old male with a history of colitis ulcerosa, and pulmonary sarcoidosis diagnosed in 10 years before, on long term oral steroids, with a history of deep vein thrombosis and acute pulmonary embolism chronically anticoagulated was referred to our center due to severe dyspnea. On admission he presented WHO functional class IV, mean pulmonary artery pressure (mPAP) in right heart catheterization (RHC) was elevated to 54 mmHg. Diagnosis of CTEPH was definitely confirmed with typical V/Q scan, and with selective pulmonary angiography (PAG) completes by intravascular imagining (intravascular ultrasound, optical coherent tomography). The patient was deemed inoperable by CTEPH team and two sessions of BPA with multimodal approach resulted in significant clinical and haemodynamical improvement to WHO class II and mPAP decrease to 27 mmHg.ConclusionsBalloon pulmonary angioplasty, rapidly developing method of treatment of inoperable CTEPH patients, is also extremely useful therapeutic tool in complex PH patients.

show abstract

Treatment of sarcoidosis-associated pulmonary hypertension: so close, and yet so far

Cited by 3 publications

References 19 publications

Clinical Study on Pulmonary Hypertension Caused by Genetic Immunity and Environment

Clinical Study on Pulmonary Hypertension Caused by Genetic Immunity and Environment

Clinical Features and Outcomes of Patients with Sarcoidosis-associated Pulmonary Hypertension

Balloon pulmonary angioplasty – efficient therapy of chronic thromboembolic pulmonary hypertension in the patient with advanced sarcoidosis – a case report

Contact Info

Product

Resources

About