Sarcoidosis-Associated Pulmonary Hypertension and Lung Transplantation for Sarcoidosis

Michael Y. Shino, MD; Joseph P. Lynch III, MD; Michael C. Fishbein, MD; Charles McGraw, MD; Jared Oyama, MD; John A. Belperio, MD; Rajan Saggar, MD

Disclosures

Semin Respir Crit Care Med. 2014;35(3):362-371. 

In This Article

Abstract and Introduction

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.

Introduction

Pulmonary hypertension (PH) is a significant complication of sarcoidosis, occurring in 6 to 74% of patients.[1–9] The presence of PH markedly increases mortality among these patients; this effect is independent of pulmonary function.[1,10,11] As will be discussed in detail later, lung transplantation (LT) is a viable therapeutic option for severe pulmonary parenchymal or vascular disease refractory to medical therapy in patients with sarcoidosis,[12] but has significant morbidity and mortality. In this article, we first discuss in depth sarcoidosis-associated PH (SAPH), including pathogenesis, prevalence, clinical features, and therapy. Finally, we discuss LT (results, indications, and identifying appropriate candidates) for sarcoid patients with end-stage fibrocystic disease or SAPH.

Comments

3090D553-9492-4563-8681-AD288FA52ACE

processing....