Furst D E, Clements P J, Graze P, Gale R, Roberts N
Arthritis Rheum. 1979 Aug;22(8):904-10. doi: 10.1002/art.1780220815.
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.
6名骨髓移植长期存活者出现了一种类似于进行性系统性硬化症(PSS)的综合征。皮肤受累(6/6)、肺部疾病(6/6)、肌肉骨骼受累(4/6)、角膜结膜炎/施密特试验阳性(4/6)、雷诺现象(2/6)以及肾脏和心脏疾病(1/6)与PSS患者的表现相似。T和B淋巴细胞计数及功能也相似。这种骨髓移植后出现的包括内脏受累的类PSS综合征支持了进行性系统性硬化症发病机制的免疫假说。