de Carvalho E F, Parra E R, de Souza R, A'b Saber A M, Machado J de Carvalho, Capelozzi V L
Department of Pathology, University of Sao Paulo Medical School, Sao Paulo, Brazil.
Histopathology. 2008 Aug;53(2):195-204. doi: 10.1111/j.1365-2559.2008.03072.x.
To compare septal and vascular matrix remodelling, vascular occlusion, pulmonary function tests and survival between two groups: one with idiopathic non-specific interstitial pneumonia (NSIP) and one with NSIP associated with systemic sclerosis (SSc).
Pulmonary biopsy specimens were examined from 40 patients, 22 with NSIP and 18 with NSIP associated with SSc. The content of septal collagen and elastic fibres, as well as the elastic fibres in the vascular interstitium, were higher in the SSc group (P = 0.01, P = 0.001 and P < 0.0001, respectively). Among pulmonary function tests, the diffusing capacity for carbon monoxide/alveolar volume was affected to a greater extent in the SSc group (59% of the predicted value in SSc and 97% in the idiopathic group). There were no differences in collagen content of the vascular interstitium, arterial occlusion, or survival between the two groups.
Although the fibrotic process is more intense in the SSc group, it does not affect the prognosis of these patients. Because the elastotic process is higher in the SSc group, this might suggest that autoimmune inflammatory mechanisms affecting the elastic fibre system play a greater role in the pathogenesis and pulmonary remodelling process of SSc NSIP than in idiopathic NSIP.
比较两组患者的间隔和血管基质重塑、血管闭塞、肺功能测试及生存率，一组为特发性非特异性间质性肺炎（NSIP）患者，另一组为与系统性硬化症（SSc）相关的NSIP患者。
对40例患者的肺活检标本进行检查，其中22例为NSIP患者，18例为与SSc相关的NSIP患者。SSc组的间隔胶原和弹性纤维含量以及血管间质中的弹性纤维含量更高（分别为P = 0.01、P = 0.001和P < 0.0001）。在肺功能测试中，一氧化碳弥散量/肺泡容积在SSc组受影响程度更大（SSc组为预测值的59%，特发性组为97%）。两组在血管间质胶原含量、动脉闭塞或生存率方面无差异。
尽管SSc组的纤维化过程更强烈，但并不影响这些患者的预后。由于SSc组的弹性化过程更严重，这可能表明影响弹性纤维系统的自身免疫炎症机制在SSc-NSIP的发病机制和肺重塑过程中比在特发性NSIP中起更大作用。
