Interstitial lung diseases (ILD) in children comprise a heterogeneous group of rare lung disorders which may lead to end stage lung disease. Little is known about the pre- and post-lung transplant status of this population. Previous studies use small numbers of patients with specific forms of ILD. Thus, our objective was to evaluate patients with all ILD diagnoses leading to lung transplantation at two tertiary care pediatric centers. We hypothesized that children with ILD are sicker at transplant evaluation but that outcomes are comparable to other lung transplant recipients.
All patients transplanted between October 1, 2002 and June 15, 2007 at Texas Children's Hospital and St Louis Children's Hospital were reviewed retrospectively. Pre-transplant morbidity parameters included pulmonary hypertension, compensated hypercapnia, six minute walk test (6MWT) z scores, need for positive pressure ventilation (PPV), and care in the intensive care unit (PICU). Post-transplant outcomes included number of infections and acute rejection episodes, time to post transplant lymphoproliferative disease (PTLD) and bronchiolitis obliterans (BO), and survival. Statistical analyses included Mann Whitney U Test, Fisher's Exact Test, Univariate and Logistic Regression, and Cox Proportional Hazards.
There were a total of 104 patients (ILD n=31, non-ILD n=73). ILD patients had significantly higher pre-transplant morbidity including presence of pulmonary hypertension (p=0.001), compensated hypercapnia (p=0.031), lower 6MWT z scores (p=0.001), need for PPV (p=0.006), and PICU care (p=0.001). After transplant, the only significantly different outcome was less grade A2 or higher acute rejection episodes in the ILD group (p=0.014). There were no differences in time to BO (p=0.840) and PTLD (p=0.902), incidence of respiratory (p=0.991,) and non respiratory infections (p=0.343). One year (p=0.499) and three year survival (p=0.657) were also similar.
Although ILD patients are sicker at time of transplant evaluation, their outcomes are comparable to other candidates. One notable finding is that ILD recipients experience significantly less acute allograft rejection episodes.
Lung transplantation is a reasonable therapeutic option to treat end stage childhood ILD.
Jennifer Rama, No Financial Disclosure Information; No Product/Research Disclosure Information