ISSN:0883-0185    

DOI:10.3109/08830189509056715

出版商:Taylor&Francis    

年代:1995

数据来源: Taylor

摘要:

A number of humoral and cellular immune abnormalities are present in patients with early scleroderma (systemic sclerosis). Most of these abnormalities reflect ongoing autoimmune reactions of the cellular and humoral types, resulting in a variety of autoantibodies to cellular and tissue constituents. Evidence exists for a defect(s) in immunoregulation favoring excessive helper T cell activity. The presence of circulating cytokines and shed interleukin-2 receptors suggest ongoing cellular immune reactions are occurring, generating cytokines and lymphokines that are capable of effecting the vascular and fibrotic lesions that are hallmarks of the disease. Future directions for research are suggested that would focus on determining if, and at what point, flbroblasts might function autonomously to generate excessive matrix components and on determining the nature of the original antigenic stimulus that starts the scleroderma process.

ISSN:0883-0185    

DOI:10.3109/08830189509056716

出版商:Taylor&Francis    

年代:1995

数据来源: Taylor

ISSN:0883-0185    

DOI:10.3109/08830189509056717

出版商:Taylor&Francis    

年代:1995

数据来源: Taylor

摘要:

Fibrosis of the pulmonary parenchyma is a frequent and serious complication of scleroderma (systemic sclerosis, SSc), resulting in significant morbidity and mortality. During the past decade data have accumulated in support of an inflammatory process affecting the alveoli and distal airways that culminates in irreversible fibrosis in many SSc patients. Recent findings indicate the presence of lung fibroblasts with altered phenotype and biologic activity (myofibroblasts), perhaps arising from the influence of cytokines on resident lung fibroblasts. Acute-phase inflammatory cytokines such as IL-1α, TNF-α, MlP-1α, IL-8 and RANTES are increased in SSc bronchoalveolar lavage (BAL) fluid, as is thrombin, a potent mitogen for lung fibroblasts. Chronic-phase inflammatory and fibrogenic cytokines such as PDGF and TGF-βare also present in increased amounts in SSc BAL fluid. The inciting event(s) and the process(es) leading to the perpetuation of fibrosis in SSc are unknown. Treatment of SSc lung disease has been empiric and generally disappointing, and it is likely that effective treatment awaits a better understanding of the biological events that regulate collagen and other extracellular matrix synthesis.

ISSN:0883-0185    

DOI:10.3109/08830189509056718

出版商:Taylor&Francis    

年代:1995

数据来源: Taylor

ISSN:0883-0185    

DOI:10.3109/08830189509056704

出版商:Taylor&Francis    

年代:1995

数据来源: Taylor