[肺朗格汉斯细胞组织细胞增生症的诊断与治疗经验]

[Own experience in diagnosis and therapy in pulmonary Langerhans cell granulomatosis].

作者信息

Sobiecka M, Wesołowski S, Kuś J

机构信息

I Klinika Gruźlicy i Chorób Płuc Instytutu Gruźlicy i Chorób Płuc w Warszawie.

出版信息

Pol Merkur Lekarski. 2001 Jun;10(60):416-20.

PMID:11503253

Abstract

UNLABELLED

We have assessed 12 patients (2 females, 10 males) aged between 19 and 53 years (mean 38.3 +/- 10.3) diagnosed with LCG during 14-year period (1985-1998). All patients were smokers. Follow-up was from 6 to 132 months (mean 47.5 +/- 44.4). LCG diagnosis was confirmed by histology in 10 cases (9 lung, and 1 bone biopsy). In 2 patients the diagnosis was made on clinical grounds, including characteristic appearance on HRCT scans. Mean FVC was 78.9 +/- 15.9% of predicted, DLCO 64.1 +/- 22% of predicted. In 8 patients (67%) FVC or DLCO were below 80% of predicted. In 2 patients with histologically proven diagnosis, HRCT was not characteristic for LCG. The treatment was introduced in 8 patients (67%). Only 3 out of 8 patients initially treated with steroids responded to this treatment. In the rest treated patients, therapy was changed to cytotoxic agents. 3 patients died (1 after 2 years, and 2 after 11 years) 2 due to LCG and 1 due to pneumonia.

CONCLUSION

HRCT appearance is not always characteristic in patients with histological diagnosis of LCG. Pulmonary involvement in LCG is frequently connected with lung function derangements. Response to steroids is poor, and switching to cytotoxic agents is often necessary.

摘要

未标注

我们评估了12例患者（2例女性，10例男性），年龄在19至53岁之间（平均38.3±10.3岁），在14年期间（1985 - 1998年）被诊断为淋巴细胞间质性肺炎（LCG）。所有患者均为吸烟者。随访时间为6至132个月（平均47.5±44.4个月）。10例患者（9例肺部活检，1例骨活检）经组织学确诊为LCG。2例患者根据临床依据诊断，包括高分辨率CT（HRCT）扫描的特征性表现。平均用力肺活量（FVC）为预测值的78.9±15.9%，一氧化碳弥散量（DLCO）为预测值的64.1±22%。8例患者（67%）的FVC或DLCO低于预测值的80%。2例经组织学确诊的患者，HRCT表现不具有LCG的特征性。8例患者（67%）接受了治疗。最初接受类固醇治疗的8例患者中只有3例对此治疗有反应。其余接受治疗的患者，治疗改为细胞毒性药物。3例患者死亡（1例在2年后，2例在11年后），2例死于LCG，1例死于肺炎。