Chest
Volume 120, Issue 3, September 2001, Pages 873-880
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Clinical Investigations
Sarcoidosis
Characteristics and Outcomes of Patients With Sarcoidosis Listed for Lung Transplantation

https://doi.org/10.1378/chest.120.3.873Get rights and content

Abstract

Study objectives

To characterize the course of patients with advanced sarcoidosis who have been listed for lung transplantation and to identify prognostic factors for death while they are on the waiting list.

Setting

Tertiary-care university hospital.

Patients

Forty-three patients with sarcoidosis who have been listed for lung transplantation at the University of Pennsylvania Medical Center.

Methods

A multivariable explanatory analysis using a Cox proportional hazards model was performed to determine risk factors that are independently associated with mortality while patients await transplantation.

Results

Twenty-three of the 43 patients (53%) died while awaiting transplantation. The survival rate of listed patients (as determined by the Kaplan-Meier method) was 66% at 1 year, 40% at 2 years, and 31% at 3 years. In a univariate analysis, the following factors were significantly associated with death on the waiting list: Pao2 ≤ 60 mm Hg (relative risk [RR], 3.4; 95% confidence interval [CI], 1.2 to 9.3); mean pulmonary artery pressure ≥ 35 mm Hg (RR, 3.2; 95% CI, 1.1 to 9.5); cardiac index≤ 2 L/min/m2 (RR, 2.8; 95% CI, 1.2 to 6.6), and right atrial pressure (RAP) ≥ 15 mm Hg (RR, 7.6; 95% CI, 3.0 to 19.3). Multivariable analysis revealed that RAP ≥ 15 mm Hg was the only independent prognostic variable (RR, 5.2; 95% CI, 1.6 to 16.7; p = 0.006). Twelve patients underwent lung transplantation. Survival after transplantation determined by the Kaplan-Meier method was 62% at both 1 and 2 years, and 50% at 3 years.

Conclusions

Patients with advanced sarcoidosis awaiting lung transplantation have a high mortality rate with a median survival of < 2 years. Mortality is most closely linked to elevated RAP. While earlier referral may diminish the mortality rate of patients on the waiting list for transplantation, further improvements in posttransplantation outcomes will be necessary to ensure that this procedure truly bestows a survival benefit.

Section snippets

Study Population

Between January 1991 and May 2000, 56 patients with sarcoidosis were evaluated in the University of Pennsylvania Lung Transplant Program. All patients underwent a standard battery of tests that included pulmonary function testing (ie, spirometry, lung volume measurements by body plethysmography, and single-breath diffusing capacity) (MedGraphics System 1085; Medical Graphics Inc; St. Paul, MN), measurement of room air arterial blood gas, 6-min walk testing, chest radiography, echocardiography,

Characteristics of Listed Patients

The demographic, clinical, and physiologic characteristics of the 43 patients who were listed are summarized in Table 1. The mean (± SD) age of patients was 45 ± 8 years. There were 34 black and 9 white patients in the group; 15 patients were men and 28 were women. The diagnosis of sarcoidosis was established 13 ± 8 years prior to listing, and respiratory symptoms had been present for 10 ± 7 years. Forty-seven percent of patients had extrapulmonary involvement, 77% were receiving prednisone,

Discussion

In this study, we have examined the clinical course of 43 patients with advanced sarcoidosis listed for lung transplantation in an attempt to identify prognostic indexes. Our study demonstrates that the mortality rate in this group is very high, with approximately half of listed patients dying prior to transplantation and a median survival time for the group of < 2 years. Notably, we have shown that hypoxemia, pulmonary hypertension, diminished cardiac index, and elevated RAP are associated

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    This research was supported by the Craig and Elaine Dobbin Pulmonary Research Fund.

    This article was presented in part at the American Thoracic Society Meeting, Toronto, Canada, in May 2000.

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