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* From the Division of Hematology-Oncology and Bone Marrow Transplantation (Dr. Traynor), Department of Medicine, and Department of Cancer Biology (Dr. Liu), University of Massachusetts Memorial Medical Center and Medical School, Worcester, MA; and Divisions of Pulmonary Medicine (Dr. Corbridge), Immune Therapy (Ms. Eagan, and Drs. Oyama and Burt), and Rheumatology (Dr. Barr), Department of Medicine, Northwestern University School of Medicine, Chicago IL.
Correspondence to: Ann E. Traynor, MD, Bone Marrow Transplantation, Hematology-Oncology, Rm H8–525, 45 Lake Ave North, Worcester, MA 01655; e-mail: ann.traynor{at}umassmed.edu
Aim: To report the prevalence and reversibility of pulmonary function test (PFT) abnormalities among systemic lupus erythematosus (SLE) patients, refractory to therapy, undergoing hematopoietic stem cell transplantation (HSCT).
Methods: Thirty-four SLE patients received 200 mg/kg cyclophosphamide and 90 mg/kg equine antithymocyte globulin followed by HSCT. PFTs were performed prior to, at 6 months, and yearly following HSCT.
Results: The prevalence of significant PFT abnormalities was high (97%). Low FEV1 and FVC occurred in 26 of 34 patients (76%). A significant abnormality in diffusion capacity of the lung for carbon monoxide (DLCO) occurred in 26 of 32 individuals able to complete DLCO testing (81%). DLCO 
50% of predicted occurred in 18 of 32 patients (56%). Of these 18 patients, 4 had no thoracic diagnosis and 7 had no pulmonary diagnosis. For 3 of 11 patients with a DLCO 50% of predicted and a prior pulmonary diagnosis, the only diagnosis had been pleurisy. Ten of the 34 patients (29%) identified the lung as a target organ of the lupus and carried a pulmonary diagnosis, as indicated in Table 1
. Three patients had acute alveolar hemorrhage, four patients had acute lupus pneumonitis, two patients had shrinking lung syndrome (SLS), and one patient had SLE-related pulmonary hypertension. Of these 10 patients, 4 had received prior mechanical ventilation, and 7 had required home supplemental inspired oxygen. Patients have been monitored 77 months, and 28 patients have been monitored > 18 months after HSCT. Five of 28 patients had a normal entry FVC; for each, the FVC remains normal. Of the 23 patients with an abnormal baseline FVC, 18 have improved, 15 completely and 3 partially. Eight of these 18 patients also have improved DLCO. The two patients with a diagnosis of SLS and one patient with SLE-related pulmonary hypertension improved in both parameters. Only 5 of 23 patients with an abnormal FVC did not improve. Each of these five patients retained active lupus in spite of HSCT.
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Key Words: acute interstitial pneumonitis • alveolar hemorrhage • cyclophosphamide • hematopoietic stem-cell transplant • systemic lupus erythematosus
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