摘要:

Wegener granulomatosis (WG) is a necrotizing, granulomatous vasculitis that has a clinical predilection to involve the upper airways, lungs, and kidneys.1Although the first case was reported by Klinger in 1931, Friedrich Wegener in 1936 characterized the unique clinical and pathological features of this disease that subsequently came to bear his name. Vascular inflammation and occlusion leading to tissue ischemia is a hallmark of WG. Although strong evidence indicates that such blood vessel damage is immunologically mediated, the mechanisms that initiate this process are still largely unknown.2To date, there has been no clearly established association with genetic factors, specific infectious agents, or environmental irritants, although speculation has remained that these may play a role in triggering the onset of disease. Until the introduction of therapy with cyclophosphamide (CYC) and glucocorticoids, WG was uniformly fatal. Although drug toxicity and disease relapse remain of concern with this regimen, it has provided us with a successful means of treatment and the opportunity to better understand this disease through long-term patient follow-up.

ISSN:0002-9629    

出版商:OVID    

年代:2001

数据来源: OVID

摘要:

Pulmonary fibrosis is an extra-articular disorder that can occur in association with rheumatoid arthritis. The differential diagnosis of this disorder is similar to that of idiopathic pulmonary fibrosis, but specific entities such as atypical pulmonary infections and drug-induced interstitial lung disease must be considered as causes of pulmonary fibrosis in patients with rheumatoid arthritis. Although the cause of lung fibrosis in persons with rheumatoid arthritis is unknown, factors that can potentially contribute to the pathogenesis of this pulmonary disease include genetic susceptibility, development of an altered immunologic response, and/or aberrant host repair processes. The clinical course of patients with pulmonary fibrosis and rheumatoid arthritis is heterogeneous but is generally insidious, chronic, and progressive. These patients respond unpredictably to available empiric therapeutic agents and, overall, their prognosis is poor; limited data suggests that the median survival time can be less than 4 years.

ISSN:0002-9629    

出版商:OVID    

年代:2001

数据来源: OVID

摘要:

Berylliosis is an environmental chronic inflammatory disorder of the lung caused by inhalation of insoluble beryllium (Be) dusts and characterized by the accumulation of CD4+ T cells and macrophages in the lower respiratory tract. In response to Be inhalation, noncaseating granuloma formation and, eventually, fibrosis. The immunopathogenic process is maintained by Be-specific lung CD4+ T-lymphocytes. Consistent with the disease immunopathology, these Be-specific T cells have a T-helper 1 phenotype producing interleukin-2 and interferon-&ggr;, the macrophage-activating cytokine driving the granulomatous reaction. Previous studies have demonstrated that the glutamic acid in position 69 of the human leukocyte antigen class II b chain is strongly associated with increased susceptibility to Be in exposed workers, suggesting that human leukocyte antigen gene markers may be used as epidemiological probes to identify population groups at higher risk.

ISSN:0002-9629    

出版商:OVID    

年代:2001

数据来源: OVID

摘要:

More than a century has elapsed since the initial description of sarcoidosis, but critical aspects of the disorder remain poorly understood. Information obtained from epidemiologic observations and basic laboratory research suggests that the disease may represent an immunologic response to an exogenous agent in a genetically susceptible individual. However, a definitive etiologic role for any specific exogenous agent has never been proved, and a “candidate gene” underlying a predisposition to sarcoidosis has not yet been identified. This review presents an historical framework for considering available evidence regarding a transmissible agent in sarcoidosis and host susceptibility to the disease.

ISSN:0002-9629    

出版商:OVID    

年代:2001

数据来源: OVID