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The natural course of progressive systemic sclerosis patients with interstitial lung involvement

Clinical Rheumatologyvolume26，，，，pages349–354（2007）引用本文

抽象的

Objective

该研究的目的是评估间质性肺部受累患者的全身性硬化症（SSC）的自然过程，并评估治疗的作用。

材料和方法

Sixty SSc patients with interstitial lung involvement were included in the study and history and retrospective data records of the patients were reviewed.

结果

It was observed that 47 patients (78.3%) had Raynaud’s phenomenon, 7 patients (11.7%) had skin involvement, 5 patients (8.3%) had joint involvement, and 1 patient (1.7%) had gastrointestinal system involvement as the first manifestation of the disease. Lung involvement had developed on an average of 113±106 months after the first manifestation of the disease and was apparent within the first year in 10 patients (16.7%), between 1 and 2 years in 3 patients (5%), between 2 and 3 years in 2 patients (3.3%), between 3 and 4 years in 5 patients (8.3%), between 4 and 10 years in 21 patients (35%), and after 10 years in 19 patients (31.7%). When the symptoms of lung involvement appeared, 37 patients (61.7%) were not receiving treatment while 23 (38.3%) were using an immunosuppressive agent. The time interval of lung involvement for the treated patients was 131±95 months while it was 101±112 months in untreated patients (p>0.05).

Conclusion

In SSc patients with intersititial pulmonary involvement, the disease frequently starts with Raynaud’s phenomenon and pulmonary symptoms tend to appear at a mean of 7 years after the onset of disease. The first sign of the disease, the probability of interstitial pulmonary involvement, is highest during the first 15 years after. Although this probability decreases after 15 years, in up to 10% of the patients, interstitial pulmonary involvement can still occur even up to 40 years. Immunosuppressive treatment is not effective in preventing the development of pulmonary involvement. However, it delays the manifestation of pulmonary symptoms for nearly 4 years.

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  1. Ikitimur Hande

    目前的地址：Adnan KahveciBulvarýRontgenSokak，第34/23号Bahcelievler，34180，伊斯坦布尔，土耳其

Affiliations

  1. Department of Pulmonary Diseases, Cerrahpasa Medical Faculty, Istanbul University, Istanbul, Turkey

    Musellim Benan, Ikitimur Hande & Ongen Gul

作者
  1. Musellim Benan
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  2. Ikitimur Hande
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  3. Ongen Gul
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Benan，M.，Hande，I。＆Gul，O。进行间质肺参与的进行性全身性硬化症患者的自然过程。Clin Rheumatol26，，349–354（2007). https://doi.org/10.1007/s10067-006-0302-6

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  • doi：https://doi.org/10.1007/s10067-006-0302-6

Keywords

  • Interstitial lung involvement
  • Natural course
  • Systemic sclerosis