A syndrome resembling progressive systemic sclerosis after bone marrow transplantation. A model for scleroderma?
Six long term survivors of bone marrow transplants developed a syndrome similar to progressive systemic sclerosis (PSS). Cutaneous involvement (6/6), pulmonary disease (6/6), musculoskeletal involvement (4/6), keratoconjunctivitis/positive Schirmer's test (4/6), Raynaud's phenomenon (2/6), and renal and cardiac disease (1/6) were similar to findings in PSS patients. T and B lymphocyte counts and functions were also similar. This PSS-like syndrome, including visceral involvement, after bone marrow transplantation lends support to an immunologic hypothesis of the pathogenesis of progressive systemic sclerosis.
D000936:Antigen-Antibody Complex; D001402:B-Lymphocytes / Q000276:immunology; D016026:Bone Marrow Transplantation; D003404:Creatinine / Q000652:urine; D006087:Graft vs Host Reaction; D006801:Humans; D012595:Scleroderma, Systemic / Q000209:etiology*; D013601:T-Lymphocytes / Q000276:immunology; D014184:Transplantation, Homologous / Q000009:adverse effects*
D000936:Antigen-Antibody Complex; D003404:Creatinine