interstitial lung disease in connective tissue diseases evolving concepts of pathogenesis and management间质性肺疾病的发病机制和管理的结缔组织疾病发展的概念.pdfVIP

Castelino and Varga Arthritis Research Therapy 2010, 12:213 /content/12/4/213 R E V I E W Interstitial lung disease in connective tissue diseases: evolving concepts of pathogenesis and management 1 2 Flavia V Castelino * and John Varga one subset, and diff use parenchymal lung diseases, which Abstract may be secondary to a variety of occupational or Interstitial lung disease (ILD) is a challenging clinical environmental exposures, or – as discussed in the present entity associated with multiple connective tissue review – can complicate multiple rheumatic or connec- diseases, and is a signii cant cause of morbidity and tive tissue diseases (CTDs). h ese diseases include sys- mortality. Ef ective therapies for connective tissue temic sclerosis (SSc), where ILD occurs in a majority of disease-associated interstitial lung disease (CTD-ILD) patients, and rheumatoid arthritis (RA), polymyositis/ are still lacking. Multidisciplinary clinics dedicated to dermato myositis (PM/DM), Sjögren’s syndrome, sys temic the early diagnosis and improved management of lupus erythematosus (SLE), undiff erentiated CTD, and patients with CTD-ILD are now being established. mixed CTD, where ILD is a less frequent complication There is rapid progress in understanding and (Table 1). In addition to ILD, other forms of lung damage identifying the ef ector cells, the proinl ammatory and involving the pleura, vasculature, airways, and lymphatic proi brotic mediators, and the pathwa