Allard P, Kermarec J, L'Her P, Natali F, Lebarbu M, Le Vagueresse R
Ann Med Interne (Paris). 1984;135(6):431-4.
The authors report the case of a 70 year old woman with a 15 year history of the Gougerot-Sjogren syndrome, Raynaud's phenomenon and sclerodactyly. Progressive respiratory symptoms in this case were not due to pulmonary fibrosis secondary to scleroderma but to diffuse interstitial lymphocytic pneumonia (ILP) secondary to the Gougerot-Sjogren syndrome. The authors emphasize the diagnostic value of the significant, permanent lymphocytosis in the aspirate after broncho-alveolar lavage. The differential diagnosis is difficult because the radiological and clinical signs of pulmonary complications of the Gougerot-Sjogren syndrome and of scleroderma are very similar. Reports of the triple association of ILP-Gougerot-Sjogren syndrome-scleroderma are uncommon in the medical literature. However, prospective studies suggest that paraclinical stigmata of the Sjogren syndrome are relatively common in patients with scleroderma. These observations suggest that some patients with clinical "pulmonary fibrosis" may in fact have ILP secondary to slowly progressive Sjogren's syndrome.
作者报告了一例70岁女性病例,该患者患有古热罗-舍格伦综合征、雷诺现象和指硬皮病达15年之久。该病例中进行性呼吸症状并非由硬皮病继发的肺纤维化所致,而是由古热罗-舍格伦综合征继发的弥漫性间质性淋巴细胞肺炎(ILP)引起。作者强调支气管肺泡灌洗后吸出物中显著、持续性淋巴细胞增多的诊断价值。鉴别诊断困难,因为古热罗-舍格伦综合征和硬皮病肺部并发症的放射学和临床体征非常相似。ILP-古热罗-舍格伦综合征-硬皮病三联征的报道在医学文献中并不常见。然而,前瞻性研究表明,舍格伦综合征的副临床体征在硬皮病患者中相对常见。这些观察结果表明,一些临床诊断为“肺纤维化”的患者实际上可能患有继发于缓慢进展性舍格伦综合征的ILP。