Schnabel A, Reuter M
Sana Gelenk- und Rheumazentrum Baden-Württemberg, Klinik für Rheumatologie und Klinische Immunologie, Bad Wildbad.
Z Rheumatol. 2009 Oct;68(8):650-4, 656-7. doi: 10.1007/s00393-009-0482-y.
Interstitial lung disease (ILD) is among the most common organ manifestations in collagen vascular diseases and can be a major determinant of the long-term prognosis. Early diagnosis rests mainly on spirometry, including measurement of the CO diffusion capacity, and high-resolution computed tomography (HRCT). The most common histopathology of ILD in collagen vascular diseases is the non-specific interstitial pneumonia (NSIP) pattern. It is distinguished from the usual interstitial pneumonia (UIP) pattern, which is most common in idiopathic lung fibrosis, by a significant inflammatory component, a distinct HRCT pattern, with often prominent ground-glass opacities, and a distinct cell pattern on bronchoalveolar lavage (BAL) with an often prominent lymphocyte component. Therapeutic experience is heterogeneous but evidence is accumulating suggesting that NSIP in the collagen vascular diseases is amenable to immunosuppression. Rapid clinical and/or radiological deterioration, reflecting inflammatory activity, appears to mark those patients who are most likely to benefit from cyclophosphamide therapy.
间质性肺疾病（ILD）是胶原血管病最常见的器官表现之一，并且可能是长期预后的主要决定因素。早期诊断主要依靠肺量计，包括一氧化碳弥散能力的测量，以及高分辨率计算机断层扫描（HRCT）。胶原血管病中ILD最常见的组织病理学表现是非特异性间质性肺炎（NSIP）模式。它与特发性肺纤维化中最常见的寻常型间质性肺炎（UIP）模式不同，其具有显著的炎症成分、独特的HRCT模式（常伴有明显的磨玻璃影），以及支气管肺泡灌洗（BAL）中独特的细胞模式（常伴有明显的淋巴细胞成分）。治疗经验参差不齐，但越来越多的证据表明，胶原血管病中的NSIP适合免疫抑制治疗。反映炎症活动的快速临床和/或放射学恶化似乎表明这些患者最有可能从环磷酰胺治疗中获益。
