title: Progressive interstitial fibrosis of the lung in sclerodermoid chronic graft-versus-host disease.
abstract: 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.
authors.affiliation: Division of Hematology and Oncology, Department of Internal Medicine, University of Rostock, Germany.
authors.forename: D
authors.initials: D
authors.lastname: Wolff
authors.forename: F
authors.initials: F
authors.lastname: Reichenberger
authors.forename: B
authors.initials: B
authors.lastname: Steiner
authors.forename: C
authors.initials: C
authors.lastname: Kahl
authors.forename: M
authors.initials: M
authors.lastname: Leithäuser
authors.forename: T
authors.initials: T
authors.lastname: Skibbe
authors.forename: T
authors.initials: T
authors.lastname: Friedrich
authors.forename: H
authors.initials: H
authors.lastname: Terpe
authors.forename: W
authors.initials: W
authors.lastname: Helbig
authors.forename: M
authors.initials: M
authors.lastname: Freund
mesh_terms: D000328:Adult; D016026:Bone Marrow Transplantation / Q000009:adverse effects; D018549:Cryptogenic Organizing Pneumonia / Q000209:etiology; D005260:Female; D006086:Graft vs Host Disease / Q000209:etiology* / Q000473:pathology; D006801:Humans; D007165:Immunosuppression Therapy / Q000009:adverse effects; D015470:Leukemia, Myeloid, Acute / Q000628:therapy; D015459:Leukemia-Lymphoma, Adult T-Cell / Q000628:therapy; D008297:Male; D011658:Pulmonary Fibrosis / Q000209:etiology* / Q000473:pathology; D012595:Scleroderma, Systemic / Q000209:etiology* / Q000473:pathology; D012871:Skin Diseases / Q000209:etiology / Q000473:pathology; D019172:Transplantation Conditioning / Q000009:adverse effects