Progressive interstitial fibrosis of the lung in sclerodermoid chronic graft-versus-host disease

• Published in: Bone marrow transplantation (Basingstoke) Vol. 29; no. 4; pp. 357 - 360
• Main Authors: WOLFF, D, REICHENBERGER, F, STEINER, B, KAHL, C, LEITHÄUSER, M, SKIBBE, T, FRIEDRICH, T, TERPE, H, HELBIG, W, FREUND, M
• Format: Journal Article
• Language: English
• Published: Basingstoke Nature Publishing Group 01.02.2002
• Subjects: Adult; Anesthesia. Intensive care medicine. Transfusions. Cell therapy and gene therapy; Biological and medical sciences; Bone marrow; Bone marrow transplantation; Bone Marrow Transplantation - adverse effects; Bone marrow, stem cells transplantation. Graft versus host reaction; Bronchiolitis obliterans; Bronchopneumonia; Chronic obstructive pulmonary disease, asthma; Complications; Cryptogenic Organizing Pneumonia - etiology; Female; Fibrosis; Graft versus host disease; Graft vs Host Disease - etiology; Graft vs Host Disease - pathology; Graft-versus-host reaction; Hematopoietic stem cells; Humans; Immunosuppression; Immunosuppression - adverse effects; Irradiation; Leukemia, Myeloid, Acute - therapy; Leukemia-Lymphoma, Adult T-Cell - therapy; Lung diseases; Lungs; Male; Medical sciences; Patients; Pneumology; Pulmonary fibrosis; Pulmonary Fibrosis - etiology; Pulmonary Fibrosis - pathology; Radiation; Scleroderma; Scleroderma, Systemic - etiology; Scleroderma, Systemic - pathology; Signs and symptoms; Skin Diseases - etiology; Skin Diseases - pathology; Stem cell transplantation; Stem cells; Toxicity; Transfusions. Complications. Transfusion reactions. Cell and gene therapy; Transplantation; Transplantation Conditioning - adverse effects; Human; Immunopathology; Lung disease; Connective tissue disease; Skin disease; Respiratory disease; Stem cell; Lung; Hematopoietic cell; Homograft; Autoimmune disease; Graft versus host reaction; Long term; Case study; Chronic; Systemic disease; Fibrosis; Bronchus disease; Graft; Complication; Bronchiolitis obliterans; Scleroderma
• ISSN: 0268-3369; 1476-5365
• DOI: 10.1038/sj.bmt.1703386

Sclerodermoid chronic graft-versus-host disease (sGVHD) is a well-known complication in patients with a long history of chronic GVHD. Pulmonary involvement in chronic GVHD presents typically as bronchiolitis obliterans (BO). Pulmonary fibrosis after allogeneic hematopoietic stem cell transplantation (HSCT) is presumed to be caused by the long-term toxicity of the conditioning regimen or the result of lung injury elicited predominantly by viral infections or GVHD. We present two patients with late onset pulmonary fibrosis associated with moderate sGVHD of the skin after HSCT. At the initial diagnosis of chronic GVHD both patients presented with symptoms of interstitial pneumonia. Years later both patients developed moderate to severe interstitial pulmonary fibrosis in association with sGVHD. One patient showed additional clinical and histological signs of BO. While one patient responded to increased immunosuppression including total nodal irradiation (1 Gy), the other patient died due to complications related to pulmonary fibrosis.