| Autor: |
Xinyue Lian, Yan Ye, Jing Zou, Chunmei Wu, Shuang Ye, Qiang Guo, Sheng Chen, Liangjing Lu, Ran Wang, Qiong Fu, Chunde Bao |
| Rok vydání: |
2022 |
| Předmět: |
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| Zdroj: |
Rheumatology (Oxford, England). |
| ISSN: |
1462-0332 |
| Popis: |
Objectives To describe the 8-year longitudinal study and long-term prognosis of a large inception cohort of anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive (MDA5+) DM-interstitial lung disease (ILD) patients. Methods In total, 216 patients diagnosed with MDA5+ DM-ILD were enrolled and followed up to analyse long-term survival rate. Demographic and clinical variables were collected at baseline and each temporal end point. Seventy patients who survived the first year were analysed for the long-term serological and respiratory outcomes. Results A total of 85 patients (39.3%) died during the follow-up period up to 96 months, with 89% of the deaths occurring in the first year after diagnosis. Long-term outcome was reported in 70 patients. Serological markers including anti-MDA5 antibody showed significant improvement with time. Radiographic findings and pulmonary function also improved notably in the follow-up period, especially in rapidly progressive ILD group, as measured by high-resolution computed tomography imaging scores, the estimated forced vital capacity, estimated diffusing capacity of lung carbon monoxide and dyspnoea scores. Early application of anti-fibrosis therapy helped to improve long-term pulmonary function. Conclusions MDA5+ DM-ILD patients had a high mortality rate despite aggressive treatment. Patients who survived the first year usually showed a significant improvement in serological markers and pulmonary function during the long-term follow-up. |
| Databáze: |
OpenAIRE |
| Externí odkaz: |
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