FAMILIAL IDIOPATHIC PULMONARY FIBROSIS - EVIDENCE OF LUNG INFLAMMATION IN UNAFFECTED FAMILY MEMBERS
FAMILIAL IDIOPATHIC PULMONARY FIBROSIS - EVIDENCE OF LUNG INFLAMMATION IN UNAFFECTED FAMILY MEMBERS
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DOI：
10.1056/nejm198605223142103
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发表时间：
1986-05-22
影响因子：
158.5
通讯作者：
CRYSTAL, RG
中科院分区：
文献类型：
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作者：
BITTERMAN, PB;RENNARD, SI;CRYSTAL, RG
我们评估了三个常染色体显性遗传型特发性肺纤维化家族的17名临床未受影响的成员的肺泡炎症证据。这项研究中的每个人都通过镓-67扫描进行了肺部炎症的一般评估，并通过支气管肺泡灌洗对回收细胞的类型及其激活状态进行了表征。17名受试者中有8名在灌洗研究中有肺泡炎症的证据。支持性数据包括中性粒细胞和激活的巨噬细胞数量增加，释放一种或多种中性粒细胞趋化因子和肺成纤维细胞生长因子-与明显特发性肺纤维化患者中观察到的结果相似。这八个中的四个也有一个积极的镓扫描;在所有其他临床上未受影响的受试者的扫描是正常的。在对8名有炎症证据的受试者中的7名进行2至4年的随访期间，没有出现肺纤维化的临床证据。这些结果表明，肺泡炎症发生在大约一半的临床上未受影响的家庭成员在遗传常染色体显性遗传性特发性肺纤维化的风险。这些有肺部炎症证据但无纤维化的人是否会继续具有临床上明显的肺纤维化尚不清楚。
We evaluated 17 clinically unaffected members of three families with an autosomal dominant form of idiopathic pulmonary fibrosis for evidence of alveolar inflammation. Each person in this study was examined by gallium-67 scanning for a general estimate of pulmonary inflammation, and by bronchoalveolar lavage for characterization of the types of recovered cells and their state of activation. Eight of the 17 subjects had evidence of alveolar inflammation of the lavage studies. Supporting data included increased numbers of neutrophils and activated macrophages that released one or more neutrophil chemoattractants, and growth factors for lung fibroblasts-findings similar to those observed in patients with overt idiopathic pulmonary fibrosis. Four of these eight also had a positive gallium scan; in all the other clinically unaffected subjects the scan was normal. During a follow-up of two to four years in seven of the eight subjects who had evidence of inflammation, no clinical evidence of pulmonary fibrosis has appeared. These results indicate that alveolar inflammation occurs in approximately half the clinically unaffected family members at risk of inheriting autosomal dominant idiopathic pulmonary fibrosis. Whether these persons with evidence of pulmonary inflammation but no fibrosis will proceed to have clinically evident pulmonary fibrosis is not yet known.