ABSTRACT
Lung (L) and heart-lung (HL) transplantation (Tx) is the ultimate therapeutic
option for carefully selected patients with end-stage heart-lung or lung disease,
such as Eisenmenger’s complex, emphysema, cystic fibrosis, and interstitial lung
diseases. Although the procedure is mainly performed to alleviate symptoms
and to improve quality of life, most patients experience an improved survival
compared to non-transplanted patients, with a mean actuarial five-year survival of
50% for lung and 44% for heart-lung transplants according to the International
Society for Heart and lung Transplantation (ISHLT) database (1). In the author’s
lung transplant program, the five-year survival increased from 50% in the initial
experience to about 75% in more recent years (p < 0.0001, Fig. 1). This improved survival is mainly due to better operative and perioperative outcomes,
thanks to improved surgical techniques, perioperative anaesthetic and intensive
care management and better understanding, and availability of immunosuppressive
drugs. Obliterative bronchiolitis (OB) or bronchiolitis obliterans syndrome (BOS),
the clinical correlate of OB, remains the leading cause of morbidity and late
mortality after HL or LTx, accounting for about 30% of late mortality (1). The
prevalence of OB/BOS after LTx has not changed significantly, remaining at 40%
to 50% five years after transplantation (1). However, some patients with BOS
may survive for prolonged periods, which in part may reflect introduction of
azithromycin therapy (discussed in detail later). In this chapter, we review the
incidence, risk factors, clinical features, diagnosis and treatment of OB/BOS,
with emphasis on the potential role of newer macrolide antibiotics.