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Enhanced immune complex formation in the lungs of patients with dermatomyositis.
- Source :
-
Respiratory research [Respir Res] 2023 Mar 19; Vol. 24 (1), pp. 86. Date of Electronic Publication: 2023 Mar 19. - Publication Year :
- 2023
-
Abstract
- Background: Interstitial lung disease is frequently comorbid with dermatomyositis and has a poor prognosis, especially in patients with the anti-melanoma differentiation-associated gene 5 (MDA5) autoantibody. However, the pathogenesis of dermatomyositis-related interstitial lung disease remains unclear.<br />Methods: We examined 18 and 19 patients with dermatomyositis-related interstitial lung disease and idiopathic pulmonary fibrosis (control), respectively. Lung tissues obtained from these patients were semi-quantitatively evaluated by immunohistochemical staining with in-house anti-human MDA5 monoclonal antibodies, as well as anti-human immunoglobulin (Ig) G, IgM, IgA, and complement component 3(C3) antibodies. We established human MDA5 transgenic mice and treated them with rabbit anti-human MDA5 polyclonal antibodies, and evaluated lung injury and Ig and C3 expression.<br />Results: MDA5 was moderately or strongly expressed in the lungs of patients in both groups, with no significant differences between the groups. However, patients with dermatomyositis-related interstitial lung disease showed significantly stronger expression of C3 (p < 0.001), IgG (p < 0.001), and IgM (p = 0.001) in the lungs than control. Moreover, lung C3, but IgG, IgA, nor IgM expression was significantly stronger in MDA5 autoantibody-positive dermatomyositis-related interstitial lung disease (n = 9) than in MDA5 autoantibody-negative dermatomyositis-related interstitial lung disease (n = 9; p = 0.022). Treatment with anti-MDA5 antibodies induced lung injury in MDA5 transgenic mice, and strong immunoglobulin and C3 expression was observed in the lungs of the mice.<br />Conclusion: Strong immunoglobulin and C3 expression in the lungs involve lung injury related to dermatomyositis-related interstitial lung disease. Enhanced immune complex formation in the lungs may contribute to the poor prognosis of MDA5 autoantibody-positive dermatomyositis-related interstitial lung disease.<br /> (© 2023. The Author(s).)
- Subjects :
- Animals
Humans
Mice
Antigen-Antibody Complex
Autoantibodies
Disease Progression
Immunoglobulin A
Immunoglobulin M
Interferon-Induced Helicase, IFIH1 genetics
Lung
Prognosis
Retrospective Studies
Dermatomyositis genetics
Dermatomyositis complications
Lung Diseases, Interstitial etiology
Lung Injury
Subjects
Details
- Language :
- English
- ISSN :
- 1465-993X
- Volume :
- 24
- Issue :
- 1
- Database :
- MEDLINE
- Journal :
- Respiratory research
- Publication Type :
- Academic Journal
- Accession number :
- 36934274
- Full Text :
- https://doi.org/10.1186/s12931-023-02362-0