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Enhanced immune complex formation in the lungs of patients with dermatomyositis

  1. Author:
    Zaizen, Yoshiaki
    Okamoto, Masaki
    Azuma, Koichi
    Fukuoka, Junya
    Hozumi, Hironao
    Sakamoto, Noriho
    Suda, Takafumi
    Mukae, Hiroshi
    Hoshino,Tomoaki

  2. Author Address

    Division of Respirology, Neurology, and Rheumatology, Department of Medicine, Kurume University School of Medicine, 67 Asahi-Machi, Kurume, Fukuoka, 830-0011, Japan., Department of Pathology, Nagasaki University Graduate School of Biomedical Sciences, 1-7-1 Sakamoto, Nagasaki, 852-8501, Japan., Department of Respirology and Clinical Research Center, National Hospital Organization Kyushu Medical Center, 1-8-1 Jigyouhama, Chuo-Ku, Fukuoka, 810-8563, Japan., Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama, Higashi-Ku, Hamamatsu, Sizuoka, 431-3192, Japan., Department of Respiratory Medicine, Nagasaki University Graduate School of Biomedical Sciences, 1-7-1 Sakamoto, Nagasaki, 82-8501, Japan., Division of Respirology, Neurology, and Rheumatology, Department of Medicine, Kurume University School of Medicine, 67 Asahi-Machi, Kurume, Fukuoka, 830-0011, Japan. hoshino@med.kurume-u.ac.jp., Cancer Innovation Laboratory (CIL), Center for Cancer Research (CCR), National Cancer Institute (NCI)-Frederick, 1050 Boyles St, MD, 21702-1201, Frederick, USA. hoshino@med.kurume-u.ac.jp.,
    1. Year: 2023
    2. Date: Mar 19
    3. Epub Date: 2023 03 19
  2. Journal: Respiratory Research
    1. 24
    2. 1
    3. Pages: 86
  4. Type of Article: Article
  5. Article Number: 86
  1. 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. 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. 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. 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. Keywords: Acute exacerbation; Anti-MDA5 antibody; Dermatomyositis; Idiopathic pulmonary fibrosis; Immune complex; Immunoglobulin; Interstitial pneumonia; Rapidly progressive interstitial lung disease; Type III hypersensitivity.

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External Sources

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  2. DOI: 10.1186/s12931-023-02362-0
  3. PMID: 36934274
  4. PMCID: PMC10024827
  5. PII : 10.1186/s12931-023-02362-0

Library Notes

  1. Fiscal Year: FY2022-2023
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