Management of end-stage sarcoidosis: pulmonary hypertension and lung transplantation

Abstract

Sarcoidosis is not only a multisystem, but also a multinational disease that is prevalent throughout the world, including Europe, the USA and Japan. Lung involvement in sarcoidosis is seemingly invariable, with up to 95% of patients manifesting some form of pulmonary disease during the course of their lifetime. The natural history of sarcoidosis in the lung is quite variable and spans the spectrum from spontaneous resolution to advanced fibrocystic disease in ,5% of cases. Advanced sarcoidosis will be the subject of this review with a special focus on pulmonary hypertension and lung transplantation as a last-resort treatment option for some patients with end-stage disease.

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

“…The study by BOUCLY et al [11] confirms data from prior studies about the reduced survival of sarcoidosis patients who develop pulmonary hypertension [22]. In this report, the 3-and 5-year survival rates were 74% and 55% respectively, similar to previously published papers [10,18].…”

Pulmonary hypertension in chronic interstitial lung diseases

“…In the absence of pulmonary fibrosis, corticosteroid therapy should be considered to treat PH complicating sarcoidosis, since it may sometime be efficacious. In patients with fibrotic disease, corticosteroids seem to be inactive and, when appropriate, these patients should have early referral for lung transplant evaluation [79,80]. Caution should be used with vasodilator therapies in patients with PH in sarcoidosis due to the potential risk of developing pulmonary oedema [79,80].…”

Sarcoidosis from bench to bedside: a state-of-the-art series for the clinician

“…Seven case series and one clinical trial [20][21][22][23][24][25][26][27] using therapy specific for pulmonary arterial hypertension collectively suggest a possible benefit of these agents in specific patients with sarcoidosis-associated pulmonary hypertension, although this requires further study. SCHLOBIN and NATHAN [19] also discussed the specificities of lung transplantation in the setting of sarcoidosis, with the main particularities being the multi-organ involvement, the risk of infection (fungal infections developing in cavities) and surgical difficulties due to bulky hilar adenopathy and perihilar fibrosis, pleural thickening or pulmonary hypertension.…”

“…In a recent issue of the ERJ, SCHLOBIN and NATHAN [19] comprehensively reviewed the evidence regarding pulmonary hypertension associated with sarcoidosis. Seven case series and one clinical trial [20][21][22][23][24][25][26][27] using therapy specific for pulmonary arterial hypertension collectively suggest a possible benefit of these agents in specific patients with sarcoidosis-associated pulmonary hypertension, although this requires further study.…”