Pulmonary Hypertension in End-stage Pulmonary Sarcoidosis: Therapeutic Effect of Sildenafil?

Milman, Nils; Burton, Christopher M.; Iversen, Martin; Videbæk, R.; Jensen, C. E.; Carlsen, Jørn

doi:10.1016/j.healun.2007.11.576

Cited by 124 publications

(80 citation statements)

References 24 publications

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“…18 Whether these drugs are useful in PH that is associated with sarcoidosis or other lung diseases remains to be determined. 19 Small case series have described favorable outcomes with epoprostenol, 20 bosentan, 8 and sildenafil 21 therapy. We performed a retrospective analysis to describe our experience with PAH-specific therapies in patients with sarcoidosis-associated PH.…”

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confidence: 99%

Treatment of Sarcoidosis-Associated Pulmonary Hypertension

Barnett

Bonura

Nathan

et al. 2009

Chest

142

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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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confidence: 99%

Treatment of Sarcoidosis-Associated Pulmonary Hypertension

Barnett

Bonura

Nathan

et al. 2009

Chest

142

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“…In a series of 12 diseases, patients were treated with sildenafil, and mean pulmonary artery pressure declined while cardiac output increased. However, there was no significant difference in 6MWD (9) . Endothelin-1 is put forward to play an important role in the pathophysiology of PH associated sarcoidosis.…”

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confidence: 70%

We Perform Vasoreactivity Test in Pulmonary Hypertension, but Reproducibility?

Özkeçeci¹,

Akçi²,

Onrat³

et al. 2016

Erciyes Med J

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Sarcoidosis is a chronic, granulomatous disease that it can affect all organs. Pulmonary hypertension (PH) is one of the most important complications of sarcoidosis and indicates poor prognosis. PH is determined by right cardiac catheterization and mean pulmonary artery pressure is ≥25 mmHg at rest. Vasoreactivity test is recommended to this patients, to determine vasodilator drugs to response of pulmonary vascular bed. Calcium channel blockers are used in patients with a positive test vasoreactivity. We presented a case in this article whose vasoreactivity test was positive initially, but negative in follow-up.

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“…Such effect was noted during treatment with epoprostenol [17] and bosentan [20], but not with sildenafil [14]. Sildenafil did not cause a need for escalation of oxygen supplementation in the described patient either.…”

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confidence: 66%

“…A significant functional improvement was observed after three months of the therapy, with marked NT-proBNP serum concentration decrease. The publications on PAH-specific treatment in sarcoidosis are mostly case series, and the results are difficult to interpret [14][15][16][17][18]. Nevertheless, a haemodynamic improvement thanks to drugs such as epoprostenol, iloprost, sildenafil, and bosentan was reported in all of them.…”

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confidence: 99%

Pulmonary Function Tests Leading to the Diagnosis of Vascular Malformations in School-Aged Children

Kusak

Cichocka‐Jarosz

Jedynak-Wąsowicz

et al. 2017

Advances in Respiratory Medicine

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Development of sarcoidosis-associated pulmonary hypertension (SAPH) significantly worsens prognosis in sarcoidosis patients. Unfortunately, there is no treatment of proven benefit for this condition. Medications used for treatment of pulmonary arterial hypertension are of great interest in this respect. Here, we report a case of a patient with severe SAPH treated with sildenafil. A significant, but only temporary improvement in functional status was observed, and the patient died of gradually progressing heart and respiratory failure while awaiting for lung transplantation.

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Pulmonary Hypertension in End-stage Pulmonary Sarcoidosis: Therapeutic Effect of Sildenafil?

Cited by 124 publications

References 24 publications

Treatment of Sarcoidosis-Associated Pulmonary Hypertension

Treatment of Sarcoidosis-Associated Pulmonary Hypertension

We Perform Vasoreactivity Test in Pulmonary Hypertension, but Reproducibility?

Pulmonary Function Tests Leading to the Diagnosis of Vascular Malformations in School-Aged Children

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