Lymphocytic alveolitis: a surprising index of poor prognosis in patients with primary Sjogren's syndrome

Lymphocytic alveolitis: a surprising index of poor prognosis in patients with primary Sjogren's syndrome
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DOI:
10.1007/s00296-005-0092-1
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发表时间:
2006-07-01
影响因子:
4
通讯作者:
Constantopoulos, S. H.
Constantopoulos, S. H.
中科院分区:
医学3区
文献类型:
--
作者:
Dalavanga, Y. A.;Voulgari, P. V.;Constantopoulos, S. H.

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12年前,我们报道了淋巴细胞性肺泡炎[或支气管肺泡灌洗(BAL)淋巴细胞增多症]与原发性干燥综合征(PSS)的临床肺损害相关。我们的论文是基于奥米克龙和细微的临床和功能证据的间质性肺疾病(ILD)的PSS患者的“高淋巴细胞性肺泡炎”(>15%的淋巴细胞在BAL)。这份报告是对这些患者的随访研究。对1991年研究的22例PSS患者的基本临床和功能重新评估,强调了有肺泡炎和无肺泡炎的患者之间的差异。没有明显的功能下降。然而,两组之间有两个统计学上的显著差异:(1)只有BAL淋巴细胞增多症患者必须接受类固醇治疗(5/12比0/10,P<0.05);(2)只有BAL淋巴细胞增多症患者死亡(6/12比0/10,P<0.01)。死因多种多样。只有两次与呼吸道感染有关,而没有人死于ILD继发的呼吸衰竭。BAL淋巴细胞增多似乎是PSS患者预后不良的一个令人惊讶的严重指标。我们没有提供统一的病理生理机制,因此,我们所建议的是尽可能多的PSS患者及早实施BAL。然后对这些患者进行系统的随访,以评估BAL淋巴细胞增多症在临床严重PSS的发生中是否具有任何病理生理学意义,严重到足以导致死亡。
Twelve years ago we reported that lymphocytic alveolitis [or bronchoalveolar lavage (BAL) lymphocytosis] correlates with clinical pulmonary involvement in primary Sjogren's syndrome (pSS). Our thesis was based omicron n subtle clinical and functional evidence of interstitial lung disease (ILD) in pSS patients with "high lymphocytic alveolitis" (> 15% lymphocytes in BAL). This report is a follow-up study of these patients. Basic clinical and functional re-evaluation of the 22 patients with pSS, studied in 1991, emphasized the differences between those with alveolitis and those without alveolitis. There was no significant functional decline. There were, however, two statistically significant differences between the two groups: (1) only patients with BAL lymphocytosis had to be treated with steroids (5/12 vs. 0/10, P < 0.05) and (2) only patients with BAL lymphocytosis had died in the mean time (6/12 vs. 0/10, P < 0.01). The causes of death were various. On only two occasions were they related to respiratory infections while there were no deaths from respiratory failure secondary to ILD. BAL lymphocytosis appears to be a surprisingly serious index of dismal prognosis in patients with pSS. We offer no unifying pathophysiologic mechanism for it and, therefore, all we propose is that BAL is performed early, in as many patients with pSS as possible. These patients should then be followed up systematically, in order to evaluate if BAL lymphocytosis has any pathophysiologic importance in the development of clinically serious pSS, which is serious enough to lead to death.