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Scleroderma lung disease: Evolving understanding in light of newer studies
Current Opinion in Rheumatology, 10/27/08
Antoniou KM et al. - Treatment benefits consist of the prevention of progression and are largely confined to patients with extensive pulmonary fibrosis in systemic sclerosis (SSc). Accurate prognostic evaluation by staging the severity of lung disease remains central to management and will be a major focus of future studies.
Methods- Aim was to review therapeutic goals in pulmonary fibrosis in SSc with particular attention to importance of identification of alveolitis
- Immunological/inflammatory activation remains the primary therapeutic target
- In most cases, lung disease is predominantly fibrotic and prevention of progression is the only practicable therapeutic goal
- A granulocytosis on bronchoalveolar lavage and ground-glass attenuation on CT, are usually indicative of fibrotic disease
- Recent staging system, integrating CT and pulmonary function data, might, with refinement, identify pts likely to benefit from treatment
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