SARCOIDOSIS

Pulmonary hypertension associated with sarcoidosis: mechanisms, haemodynamics and prognosis

H Nunes^1,2, M Humbert², F Capron², M Brauner¹, O Sitbon², J-P Battesti¹, G Simonneau², D Valeyre¹

¹ UPRES EA 2363, Service de Pneumologie, Service de Radiologie, Hôpital Avicenne, Assistance Publique-Hôpitaux de Paris, Université Paris 13, Bobigny, France
² UPRES EA 2705, Centre des Maladies Vasculaires Pulmonaires, Service de Pneumologie et Réanimation Respiratoire, Service d’Anatomie Pathologique, Hôpital Antoine Béclère, Assistance Publique-Hôpitaux de Paris, Université Paris Sud, Clamart, France

Correspondence to:
Dr H Nunes
Service de Pneumologie, Hôpital Avicenne, 125 rue de Stalingrad, 93009 Bobigny, France; hilario.nunes{at}avc.ap-hop-paris.fr

Background: Pulmonary hypertension (PH) is a rare complication of sarcoidosis, although it is not uncommon in advanced disease.

Methods: A retrospective series of 22 sarcoidosis patients (16 men) of mean (SD) age 46 (13) years with PH was divided into two groups depending on the absence (stage 0: n = 2, stage II: n = 4, stage III: n = 1) or presence (n = 15) of radiographic pulmonary fibrosis at the time of PH diagnosis.

Results: In both groups PH was moderate to severe and there was no response to acute vasodilator challenge. In non-fibrotic cases no other cause of PH was found, suggesting a specific sarcoidosis vasculopathy, although no histological specimens were available. In cases with fibrosis there was no correlation between haemodynamics and lung volumes or arterial oxygen tensions, suggesting other mechanisms for PH in addition to pulmonary destruction and hypoxaemia. These included extrinsic arterial compression by lymphadenopathies in three cases and histologically proven pulmonary veno-occlusive disease in the five patients who underwent lung transplantation. Ten patients received high doses of oral prednisone for PH (stage 0: n = 1, stage II: n = 4 and stage IV: n = 5); three patients without pulmonary fibrosis experienced a sustained haemodynamic response. Survival of the overall population was poor (59% at 5 years). Mortality was associated with NYHA functional class IV but not with haemodynamic parameters or with lung function.

Conclusion: Two very different phenotypes of sarcoidosis combined with PH are observed depending on the presence or absence of pulmonary fibrosis. PH is a severe complication of sarcoidosis.

Abbreviations: CI, cardiac index; FEV₁, forced expiratory volume in 1 second; FVC, forced vital capacity; mRAP, mean right atrial pressure; KCO, carbon monoxide transfer coefficient; mPAP, mean pulmonary artery pressure; PaO₂, arterial oxygen tension at room air; PH, pulmonary hypertension; PVOD, pulmonary veno-occlusive disease; PVRI, pulmonary vascular resistance index; PWP, pulmonary wedge pressure; SACE, serum angiotensin converting enzyme; SvO₂, mixed venous oxygen saturation. TLC, total lung capacity; TLCO, carbon monoxide transfer factor

Keywords: sarcoidosis; pulmonary hypertension; pulmonary veno-occlusive disease; pulmonary artery compression

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