Sarcoidosis, pulmonary hypertension, and acquired peripheral pulmonary artery stenosis

Mangla, Arun; Fisher, John F.; Libby, Daniel M.; Saddekni, Souheil

doi:10.1002/ccd.1810110110

Cited by 13 publications

(6 citation statements)

References 15 publications

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“…There are conflicting reports regarding the use of corticosteroids, with some describing improvement, 53–55 and others worsening of SAPH 13,22,28 . One study describes an improvement following corticosteroid therapy in patients with limited pulmonary fibrosis, but no treatment effect in patients with stage IV disease 16 .…”

Section: Management Of Sarcoidosis Associated Phmentioning

confidence: 99%

Pulmonary hypertension in sarcoidosis: A review

Corte¹,

Wells²,

Nicholson³

et al. 2010

Respirology

105

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Pulmonary hypertension (PH) is a well-recognized complication of sarcoidosis. Patients with sarcoidosisassociated PH (SAPH) have poorer functional status and greater supplemental oxygen requirements than sarcoidosis patients without PH, and are more likely to be listed for lung transplantation. PH is an independent risk factor for mortality in sarcoidosis patients awaiting lung transplantation. The pathophysiology of SAPH is complex, with multiple mechanisms contributing to pathogenesis, including the fibrous destruction of the pulmonary vascular bed, extrinsic compression of the central pulmonary vessels and an intrinsic vasculopathy. Recognition of SAPH may be delayed as it can be masked by the clinical picture of underlying pulmonary sarcoidosis, and right heart catheter remains the gold-standard for diagnosis. Management of SAPH is based on reversal of resting hypoxaemia, treatment of comorbidities and treatment of the underlying sarcoidosis. The use of corticosteroids in SAPH is controversial. Specific PH therapy is not routinely recommended in SAPH as there are no successful placebo-controlled trials, although there is limited data to suggest that endothelin receptor antagonists and phosphodiesterase-5 inhibitors may be useful.

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Section: Management Of Sarcoidosis Associated Phmentioning

confidence: 99%

Pulmonary hypertension in sarcoidosis: A review

Corte¹,

Wells²,

Nicholson³

et al. 2010

Respirology

105

View full text Add to dashboard Cite

show abstract

“…Less common causes of acquired branch pulmonary artery stenosis include aortic dissection [5,47], Takayasu arteritis [6,23], sarcoid [31], and lung carcinoma [56]. One case of idiopathic pulmonary artery branch stenosis has been associated with pericarditis; the stenosis may have been related to the inflammatory process, but it did require surgery [14].…”

Section: Etiologymentioning

confidence: 99%

Catheterization Treatment of Stenosis and Hypoplasia of Pulmonary Arteries

O’Laughlin

1998

Pediatr Cardiol

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Stenosis of pulmonary arteries is one of the most challenging problems requiring treatment in the care of patients with congenital and acquired cardiopulmonary disease. Surgical approaches have been met with difficulty over the years, and may themselves lead to further distortion of the treated arteries. Balloon dilation first came into use in the 1980s, and has proved moderately effective. Its use has been extended to proximal pulmonary valve stenosis in order to improve distal flow and artery growth in some variants of tetralogy of Fallot. More recently, the judicious application of stent implantation has improved the outlook for pulmonary artery stenosis. The etiology, treatment (with balloon dilation and stent placement), and prognosis of pulmonary arterial stenosis will be discussed.

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“…10,11 There are sparse data regarding the treatment of PH associated with sarcoidosis. Reports [12][13][14][15][16][17] of therapeutic responses to corticosteroids and other immunosuppressants are limited and have yielded variable results. Several efficacious pharmacologic agents are now available for the treatment of pulmonary arterial hypertension (PAH).…”

mentioning

confidence: 99%

Treatment of Sarcoidosis-Associated Pulmonary Hypertension

Barnett

Bonura

Nathan

et al. 2009

Chest

145

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Background: Pulmonary hypertension (PH) is a common complication of sarcoidosis that is associated with increased mortality. The pathogenesis of PH in sarcoidosis is uncertain, and the role of pulmonary arterial hypertension (PAH)-specific therapies remains to be determined. Methods: We conducted a retrospective study of patients with sarcoidosis and PH at two referral centers. New York Heart Association (NYHA) functional class, exercise capacity, hemodynamic data, pulmonary function tests, and survival were collected and analyzed. Results: Twenty-two sarcoidosis patients treated with PAH-specific therapies were identified. After a median of 11 months of follow-up, NYHA class was improved in nine subjects. Mean 6-min walk distance (n ‫؍‬ 18) increased by 59 m (p ‫؍‬ 0.032). Patients with a higher FVC experienced a greater increment in exercise capacity. Among 12 patients with follow-up hemodynamic data, mean pulmonary artery pressure was reduced from 48.5 ؎ 4.3 to 39.4 ؎ 2.8 mm Hg (p ‫؍‬ 0.008). The 1-and 3-year transplant-free survival rates were 90% and 74%, respectively. Conclusions: PAH-specific therapy may improve functional class, exercise capacity, and hemodynamics in PH associated with sarcoidosis. Prospective, controlled trials of PAH therapies for sarcoidosis are warranted to verify this apparent benefit. Mortality among the study population was high, highlighting the need for urgent evaluation at a lung transplant center.

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Sarcoidosis, pulmonary hypertension, and acquired peripheral pulmonary artery stenosis

Cited by 13 publications

References 15 publications

Pulmonary hypertension in sarcoidosis: A review

Pulmonary hypertension in sarcoidosis: A review

Catheterization Treatment of Stenosis and Hypoplasia of Pulmonary Arteries

Treatment of Sarcoidosis-Associated Pulmonary Hypertension

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