Interventional therapy in sarcoidosis‐associated pulmonary arterial stenosis and pulmonary hypertension

Liu, Lingli; Xu, Jian; Zhang, Yuhai; Fang, Liying; Chai, Yaqin; Niu, Mengjie; Li, Shengqing

doi:10.1111/crj.12435

Cited by 18 publications

(9 citation statements)

References 29 publications

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“…Our systematic review identified 137 potentially relevant articles; the full text of 13 was reviewed and 9 were selected. None of the studies compared TTE to no testing; rather, all were nonrandomized studies that selected patients with sarcoidosis (most enrolled patients who had persistent respiratory symptoms despite treatment of their sarcoidosis) and reported the frequency of abnormal TTE results (180,184,(191)(192)(193)(194)(195)(196)(197). Some studies also reported the rate of confirmation by right heart catheterization.…”

Section: American Thoracic Society Documentsmentioning

confidence: 99%

Diagnosis and Detection of Sarcoidosis. An Official American Thoracic Society Clinical Practice Guideline

Crouser

Maier²,

Wilson

et al. 2020

Am J Respir Crit Care Med

559

507

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Background: The diagnosis of sarcoidosis is not standardized but is based on three major criteria: a compatible clinical presentation, finding nonnecrotizing granulomatous inflammation in one or more tissue samples, and the exclusion of alternative causes of granulomatous disease. There are no universally accepted measures to determine if each diagnostic criterion has been satisfied; therefore, the diagnosis of sarcoidosis is never fully secure. Methods: Systematic reviews and, when appropriate, meta-analyses were performed to summarize the best available evidence. The evidence was appraised using the Grading of Recommendations, Assessment, Development, and Evaluation approach and then discussed by a multidisciplinary panel. Recommendations for or against various diagnostic tests were formulated and graded after the expert panel weighed desirable and undesirable consequences, certainty of estimates, feasibility, and acceptability. Results: The clinical presentation, histopathology, and exclusion of alternative diagnoses were summarized. On the basis of the available evidence, the expert committee made 1 strong recommendation for baseline serum calcium testing, 13 conditional recommendations, and 1 best practice statement. All evidence was very low quality. Conclusions: The panel used systematic reviews of the evidence to inform clinical recommendations in favor of or against various diagnostic tests in patients with suspected or known sarcoidosis. The evidence and recommendations should be revisited as new evidence becomes available.

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Section: American Thoracic Society Documentsmentioning

confidence: 99%

Diagnosis and Detection of Sarcoidosis. An Official American Thoracic Society Clinical Practice Guideline

Crouser

Maier²,

Wilson

et al. 2020

Am J Respir Crit Care Med

559

507

View full text Add to dashboard Cite

show abstract

“…Sarcoidosis patients with PH should be evaluated for PA stenosis by CT angiography. 62 PA stenosis usually does not respond to anti-inflammatory therapy such as prednisone, but some proximal stenosis cases have reversed with stenting. 62,63 In the field of PH, the definition of response to therapy has varied.…”

Section: Therapymentioning

confidence: 99%

Sarcoidosis-Associated Pulmonary Hypertension

Shlobin

Baughman

2017

Semin Respir Crit Care Med

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Sarcoidosis-associated pulmonary hypertension (SAPH) is found in 5 to 20% of sarcoidosis patients. Elevated pulmonary artery pressure may be due to multiple factors, including vasculocentric, parenchymal, and mechanical, as well as comorbidities such as cardiac sarcoidosis and sleep apnea. Most SAPH patients have fibrotic lung disease, but SAPH may be present in those without advanced parenchymal lung disease. Several features have been shown to suggest SAPH, including reduced DLCO, shortened 6-minute walk distance, with or without desaturation, and the presence of increased pulmonary artery to aorta ratio on CT scanning. Echocardiography remains an important tool for the evaluation of SAPH but may both over- or underestimate the severity of pulmonary artery pressure. Right heart catheterization remains the definitive test to make the diagnosis. There have been several reports on the value of different modalities of treating SAPH. These include prospective clinical trials and one double-blind placebo-controlled randomized trial. Evidence-based guidelines for treatment of SAPH are discussed in this review.

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“…In a prospective study of 72 patients with SAPH from a single institution, eight patients were found to have vascular compression/distortion. In all eight patients, stenting was successful in reducing pulmonary artery pressure [35].…”

Section: Discussionmentioning

confidence: 85%

WASOG statement on the diagnosis and management of sarcoidosis-associated pulmonary hypertension

et al. 2022

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Sarcoidosis-associated pulmonary hypertension (SAPH) is an important complication of advanced sarcoidosis. Over the past few years, there have been several studies dealing with screening, diagnosis and treatment of SAPH. This includes the results of two large SAPH-specific registries. A task force was established by the World Association of Sarcoidosis and Other Granulomatous disease (WASOG) to summarise the current level of knowledge in the area and provide guidance for the management of patients. A group of sarcoidosis and pulmonary hypertension experts participated in this task force. The committee developed a consensus regarding initial screening including who should undergo more specific testing with echocardiogram. Based on the results, the committee agreed upon who should undergo right-heart catheterisation and how to interpret the results. The committee felt there was no specific phenotype of a SAPH patient in whom pulmonary hypertension-specific therapy could be definitively recommended. They recommended that treatment decisions be made jointly with a sarcoidosis and pulmonary hypertension expert. The committee recognised that there were significant defects in the current knowledge regarding SAPH, but felt the statement would be useful in directing future studies.

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Interventional therapy in sarcoidosis‐associated pulmonary arterial stenosis and pulmonary hypertension

Abstract: PAS caused by external compression in sarcoidosis is a significant reason for SAPH. IT is effective and safe in the treatment of PAS and SAPH.

Cited by 18 publications

References 29 publications

Diagnosis and Detection of Sarcoidosis. An Official American Thoracic Society Clinical Practice Guideline

Diagnosis and Detection of Sarcoidosis. An Official American Thoracic Society Clinical Practice Guideline

Sarcoidosis-Associated Pulmonary Hypertension

WASOG statement on the diagnosis and management of sarcoidosis-associated pulmonary hypertension

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