Lung transplantation in telomerase mutation carriers with pulmonary fibrosis.

Lung transplantation is the only intervention that prolongs survival in idiopathic pulmonary fibrosis (IPF). Telomerase mutations are the most common identifiable genetic cause of IPF, and at times, the telomere defect manifests in extrapulmonary disease such as bone marrow failure. The relevance of this genetic diagnosis for lung transplant management has not been examined. We gathered an international series of telomerase mutation carriers who underwent lung transplant in the U.S.A., Australia and Sweden. The median age at transplant was 52 years. Seven recipients are alive with a median follow-up of 1.9 years (range 6 months to 9 years); one died at 10 months. The most common complications were haematological, with recipients requiring platelet transfusion support (88%) and adjustment of immunosuppressives (100%). Four recipients (50%) required dialysis for tubular injury and calcineurin inhibitor toxicity. These complications occurred at significantly higher rates relative to historic series (p<0.0001). Our observations support the feasibility of lung transplantation in telomerase mutation carriers; however, severe post-transplant complications reflecting the syndromic nature of their disease appear to occur at higher rates. While these findings need to be expanded to other cohorts, caution should be exercised when approaching the transplant evaluation and management of this subset of pulmonary fibrosis patients.

Human Immunodeficiency Virus and Allergic Bronchopulmonary Aspergillosis: Case Report and Review of Literature.

Allergic bronchopulmonary aspergillosis (ABPA) results from a hypersensitivity response to airways colonization with Aspergillus fumigatus, and it occurs most often in individuals with asthma or cystic fibrosis. Allergic bronchopulmonary aspergillosis is an indolent, but potentially progressive, disease in patients. In patients infected with human immunodeficiency virus (HIV), ABPA is rare, and its description in the literature is limited to case reports. We describe the occurrence of ABPA in a 37-year-old woman with well controlled HIV infection. This represents the first documented case of ABPA in an HIV-infected patient whose only pulmonary comorbidity included the ramifications of prior acute respiratory distress syndrome due to Pneumocystis jirovecii pneumonia. We also review prior case reports of ABPA in HIV-infected patients and consider risk factors for its development.

A novel risk score that incorporates recipient and donor variables to predict 1-year mortality in the current era of lung transplantation.

BACKGROUND: In this study we sought to construct a novel scoring system to pre-operatively stratify a patient's risk of 1-year mortality after lung transplantation (LTx) based on recipient- and donor-specific characteristics. METHODS: The UNOS database was queried for adult (≥18 years) patients undergoing LTx between May 1, 2005 and December 31, 2012. The population was randomly divided in a 4:1 fashion into derivation and validation cohorts. A multivariable logistic regression model for 1-year mortality was constructed within the derivation cohort. Points were then assigned to independent predictors (p < 0.05) based on relative odds ratios. Risk groups were established based on score ranges. RESULTS: During the study period, 9,185 patients underwent LTx and the 1-year mortality was 18.0% (n = 1,654). There was a similar distribution of variables between the derivation (n = 7,336) and validation (n = 1,849) cohorts. Of the 14 covariates included in the final model, 9 were ultimately allotted point values (maximum score = 70). The model exhibited good predictive strength (c = 0.65) in the derivation cohort and demonstrated a strong correlation between the observed and expected rates of 1-year mortality in the validation cohort (r = 0.87). The low-risk (score 0 to 11), intermediate-risk (score 12 to 21) and high-risk (score ≥22) groups had a 10.8%, 17.1% and 32.0% risk of mortality (p < 0.001), respectively. CONCLUSIONS: This is the first scoring system that incorporates both recipient- and donor-related factors to predict 1-year mortality after LTx. Its use could assist providers in the identification of patients at highest risk for poor post-transplant outcomes.