Lung transplantation for end-stage pulmonary sarcoidosis

Background: Sarcoidosis is a multi-system granulomatous disease which can c ause significant pulmonary morbidity and occasionally be fatal. The long te rm benefits of lung transplantation for this disorder are unknown. Methods: A retrospective review was made of nine single lung transplant procedures performed at the University of Pittsburgh between March 1991 and March 1995 in patients with endstage lung disease secondary to sarcoidosis. Two conte mporaneous groups of recipients receiving transplants for COPD (n = 30) and inflammatory lung disease (n = 13) served as control groups. Surviving rec ipients underwent sequential surveillance bronchoscopy with transbronchial biopsy. Results: All recipients survived beyond post-operative day (POD) 30 , with 5 recipients currently alive. One year survival for this group was 6 /9 (67%). Eight of the 9 sarcoidosis recipients had sequential lung biopsy procedures. Five of these 8 recipients (62.5%) had recurrence of granulomat a in the lung allograft with the mean time to diagnosis of recurrent sarcoi dosis being POD 224.2 +/- 291.3 (range POD 21 - 719). None of these 5 recip ients had radiographic evidence or clinical symptoms related to granulomato us inflammation in the allograft. Preoperative and post-operative spirometr ic values were available on 8 recipients. Vital capacity significantly impr oved in all recipients from 1.54 +/- 0.43 litres to 2.55 +/-: 0.63 litres b y POD 180 and was maintained through the fourth postoperative year (p < 0.0 5 Wilcoxon Signed Rank). Spirometric values were also compared before and a fter transplantation in the 5 recipients with granulomata in the allograft. Vital capacity significantly improved in these 5 recipients from 1.53 +/- 0.48 litres to 2.71 +/- 0.71 litres by POD 180 and was maintained throughou t the first postoperative year (p < 0.05, Wilcoxon Signed Rank). The preval ence of high grade acute cellular rejection [ACR (histologic grades III and IV)I did not differ from that seen in a contemporaneous group of 30 single lung recipients who received allografts for COPD (p < 0.05 Mann-Whitney U) , nor when compared to a group of 13 single lung recipients who received al lografts for immunologically mediated lung disease (p < 0.05 Mann-Whitney U ). The prevalence of chronic rejection (histologic obliterative bronchiolit is [OBI) in the sarcoidosis recipients was 4/8 (50%). In the controls with COPD recipients the prevalence of OB was 10/30 (33.3%), and in the 13 contr ols with immunologic disease it was 6/13 (46.2%), There was no significant difference in the prevalence of OB between the sarcoidosis recipients and c ontrols. When analyzed to the fifth year after transplantation, freedom fro m the development of OB also failed to differ between these 3 groups (p = 0 .25, Logrank, Mantel-Cox). Conclusions: Although granulomatous inflammation in the lung allograft is common following transplantation for sarcoidosis, it is not clinically or radiographically relevant. In addition, the preval ence of high grade ACR and histologic OB is no different when compared to o ther single lung recipients. For these reasons lung transplantation is a vi able alternative for end-stage lung disease secondary to sarcoidosis.