title: Sarcoidosis-associated pulmonary hypertension and lung transplantation for sarcoidosis.
abstract: Pulmonary hypertension (PH) is a significant complication of sarcoidosis, occurring in approximately 6 to > 20% of cases, and markedly increases mortality among these patients. The clinician should exercise a high index of suspicion for sarcoidosis-associated PH (SAPH) given the nonspecific symptomatology and the limitations of echocardiography in this patient population. The pathophysiology of PH in sarcoidosis is complex and multifactorial. Importantly, there are inherent differences in the pathogenesis of SAPH compared with idiopathic pulmonary arterial hypertension, making the optimal management of SAPH controversial. In this article, we review the epidemiology, diagnosis, prognosis, and treatment considerations for SAPH. Lung transplantation (LT) is a viable therapeutic option for sarcoid patients with severe pulmonary fibrocystic sarcoidosis or SAPH refractory to medical therapy. We discuss the role for LT in patients with sarcoidosis, review the global experience with LT in this population, and discuss indications and contraindications to LT.
authors.affiliation: Division of Pulmonary and Critical Care Medicine, Department of Medicine, David Geffen School of Medicine at UCLA, Los Angeles, California.
authors.forename: Michael Y
authors.initials: MY
authors.lastname: Shino
authors.affiliation: Division of Pulmonary and Critical Care Medicine, Department of Medicine, David Geffen School of Medicine at UCLA, Los Angeles, California.
authors.forename: Joseph P
authors.initials: JP
authors.lastname: Lynch Iii
authors.affiliation: Department of Pathology, David Geffen School of Medicine at UCLA, Los Angeles, California.
authors.forename: Michael C
authors.initials: MC
authors.lastname: Fishbein
authors.affiliation: Department of Radiological Sciences, UCLA Medical Center, Los Angeles, California.
authors.forename: Charles
authors.initials: C
authors.lastname: McGraw
authors.affiliation: Division of Cardiology, Department of Medicine, David Geffen School of Medicine at UCLA, Los Angeles, California.
authors.forename: Jared
authors.initials: J
authors.lastname: Oyama
authors.affiliation: Division of Pulmonary and Critical Care Medicine, Department of Medicine, David Geffen School of Medicine at UCLA, Los Angeles, California.
authors.forename: John A
authors.initials: JA
authors.lastname: Belperio
authors.affiliation: Division of Pulmonary and Critical Care Medicine, Department of Medicine, David Geffen School of Medicine at UCLA, Los Angeles, California.
authors.forename: Rajan
authors.initials: R
authors.lastname: Saggar
mesh_terms: D004452:Echocardiography; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology* / Q000503:physiopathology / Q000628:therapy; D016040:Lung Transplantation; D011379:Prognosis; D012507:Sarcoidosis / Q000150:complications* / Q000503:physiopathology / Q000628:therapy; D017565:Sarcoidosis, Pulmonary / Q000150:complications / Q000503:physiopathology / Q000628:therapy