Clinical features of macrophage activation syndrome in adult dermatomyositis: A single‐center retrospective case‐control study
Abstract Background Little is known about the features of macrophage activation syndrome (MAS) in dermatomyositis, especially the association between rapidly progressive interstitial lung disease (RP‐ILD) and MAS. Objective To determine the characteristics of MAS in patients with dermatomyositis and...
| Main Authors: | , , , , , , , |
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| Format: | Article |
| Language: | English |
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Wiley
2024-01-01
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| Series: | Immunity, Inflammation and Disease |
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| Online Access: | https://doi.org/10.1002/iid3.1141 |
| _version_ | 1797346798516305920 |
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| author | Dingxian Zhu Shuni Ying Changyi Yang Sheng Li Shunli Tang Chuanyin Sun Hong Fang Jianjun Qiao |
| author_facet | Dingxian Zhu Shuni Ying Changyi Yang Sheng Li Shunli Tang Chuanyin Sun Hong Fang Jianjun Qiao |
| author_sort | Dingxian Zhu |
| collection | DOAJ |
| description | Abstract Background Little is known about the features of macrophage activation syndrome (MAS) in dermatomyositis, especially the association between rapidly progressive interstitial lung disease (RP‐ILD) and MAS. Objective To determine the characteristics of MAS in patients with dermatomyositis and their association with RP‐ILD. Methods This was a retrospective cohort study of 201 dermatomyositis patients at the First Affiliated Hospital of Zhejiang University over a 10‐year period. Results A total of 22 (10.9%) patients were diagnosed with MAS. The rate of RP‐ILD was significantly higher in patients with MAS than in those without MAS (81.8% vs. 17.4%, respectively, p < .001). Multivariate analysis indicated that RP‐ILD (p = .019), ferritin level > 1685 ng/mL (p = .007) and hemoglobin < 100 g/L (p = .001) were independent risk factors for MAS. Furthermore, RP‐ILD patients with MAS presented more cardiac injury (50.0% vs. 13.3%, respectively, p < .009), central nervous system dysfunction (42.8% vs. 3.4%, respectively, p < .001) and hemorrhage (38.9% vs. 3.3%, respectively, p = .003) than RP‐ILD patients without MAS. The 90‐day cumulative survival rate for patients with MAS was significantly lower than for those without MAS (18.2% vs. 82.1%, respectively, p < .001). Conclusion MAS was a common and fatal complication of dermatomyositis in our cohort. MAS is closely related to RP‐ILD in patients with dermatomyositis. When RP‐ILD is present in dermatomyositis patients with abnormal laboratory findings, such as cytopenia and hyperferritinemia, the presence of MAS should be considered. |
| first_indexed | 2024-03-08T11:38:13Z |
| format | Article |
| id | doaj.art-559cee9575864cd19b4741bde4848a7b |
| institution | Directory Open Access Journal |
| issn | 2050-4527 |
| language | English |
| last_indexed | 2024-03-08T11:38:13Z |
| publishDate | 2024-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Immunity, Inflammation and Disease |
| spelling | doaj.art-559cee9575864cd19b4741bde4848a7b2024-01-25T11:02:19ZengWileyImmunity, Inflammation and Disease2050-45272024-01-01121n/an/a10.1002/iid3.1141Clinical features of macrophage activation syndrome in adult dermatomyositis: A single‐center retrospective case‐control studyDingxian Zhu0Shuni Ying1Changyi Yang2Sheng Li3Shunli Tang4Chuanyin Sun5Hong Fang6Jianjun Qiao7Department of Dermatology, The First Affiliated Hospital Zhejiang University School of Medicine Hangzhou ChinaDepartment of Dermatology, The First Affiliated Hospital Zhejiang University School of Medicine Hangzhou ChinaDepartment of Dermatology, The First Affiliated Hospital Zhejiang University School of Medicine Hangzhou ChinaDepartment of Dermatology, The First Affiliated Hospital Zhejiang University School of Medicine Hangzhou ChinaDepartment of Dermatology, The First Affiliated Hospital Zhejiang University School of Medicine Hangzhou ChinaDepartment of Rheumatology, The First Affiliated Hospital Zhejiang University School of Medicine Hangzhou ChinaDepartment of Dermatology, The First Affiliated Hospital Zhejiang University School of Medicine Hangzhou ChinaDepartment of Dermatology, The First Affiliated Hospital Zhejiang University School of Medicine Hangzhou ChinaAbstract Background Little is known about the features of macrophage activation syndrome (MAS) in dermatomyositis, especially the association between rapidly progressive interstitial lung disease (RP‐ILD) and MAS. Objective To determine the characteristics of MAS in patients with dermatomyositis and their association with RP‐ILD. Methods This was a retrospective cohort study of 201 dermatomyositis patients at the First Affiliated Hospital of Zhejiang University over a 10‐year period. Results A total of 22 (10.9%) patients were diagnosed with MAS. The rate of RP‐ILD was significantly higher in patients with MAS than in those without MAS (81.8% vs. 17.4%, respectively, p < .001). Multivariate analysis indicated that RP‐ILD (p = .019), ferritin level > 1685 ng/mL (p = .007) and hemoglobin < 100 g/L (p = .001) were independent risk factors for MAS. Furthermore, RP‐ILD patients with MAS presented more cardiac injury (50.0% vs. 13.3%, respectively, p < .009), central nervous system dysfunction (42.8% vs. 3.4%, respectively, p < .001) and hemorrhage (38.9% vs. 3.3%, respectively, p = .003) than RP‐ILD patients without MAS. The 90‐day cumulative survival rate for patients with MAS was significantly lower than for those without MAS (18.2% vs. 82.1%, respectively, p < .001). Conclusion MAS was a common and fatal complication of dermatomyositis in our cohort. MAS is closely related to RP‐ILD in patients with dermatomyositis. When RP‐ILD is present in dermatomyositis patients with abnormal laboratory findings, such as cytopenia and hyperferritinemia, the presence of MAS should be considered.https://doi.org/10.1002/iid3.1141complicationdermatomyositismacrophage activation syndromerapidly progressive interstitial lung disease |
| spellingShingle | Dingxian Zhu Shuni Ying Changyi Yang Sheng Li Shunli Tang Chuanyin Sun Hong Fang Jianjun Qiao Clinical features of macrophage activation syndrome in adult dermatomyositis: A single‐center retrospective case‐control study Immunity, Inflammation and Disease complication dermatomyositis macrophage activation syndrome rapidly progressive interstitial lung disease |
| title | Clinical features of macrophage activation syndrome in adult dermatomyositis: A single‐center retrospective case‐control study |
| title_full | Clinical features of macrophage activation syndrome in adult dermatomyositis: A single‐center retrospective case‐control study |
| title_fullStr | Clinical features of macrophage activation syndrome in adult dermatomyositis: A single‐center retrospective case‐control study |
| title_full_unstemmed | Clinical features of macrophage activation syndrome in adult dermatomyositis: A single‐center retrospective case‐control study |
| title_short | Clinical features of macrophage activation syndrome in adult dermatomyositis: A single‐center retrospective case‐control study |
| title_sort | clinical features of macrophage activation syndrome in adult dermatomyositis a single center retrospective case control study |
| topic | complication dermatomyositis macrophage activation syndrome rapidly progressive interstitial lung disease |
| url | https://doi.org/10.1002/iid3.1141 |
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