Dynamic change of lymphocytes associated with short-term prognosis in anti-MDA5-positive dermatomyositis with interstitial lung disease: a multicenter retrospective study.

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  • Additional Information
    • Source:
      Publisher: Springer Country of Publication: Germany NLM ID: 8211469 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1434-9949 (Electronic) Linking ISSN: 07703198 NLM ISO Abbreviation: Clin Rheumatol Subsets: MEDLINE
    • Publication Information:
      Publication: <2008->: Heidelberg : Springer
      Original Publication: Brussels : Acta Medica Belgica, [1982-
    • Subject Terms:
      Lung Diseases, Interstitial*/immunology ; Lung Diseases, Interstitial*/blood ; Lung Diseases, Interstitial*/mortality ; Lung Diseases, Interstitial*/complications ; Dermatomyositis*/complications ; Dermatomyositis*/blood ; Dermatomyositis*/immunology ; Interferon-Induced Helicase, IFIH1*/immunology ; Lymphocytes*; Humans ; Male ; Female ; Retrospective Studies ; Middle Aged ; Prognosis ; Adult ; Lymphocyte Count ; Aged ; Lymphopenia/blood ; Lymphopenia/complications ; Lymphopenia/immunology ; Proportional Hazards Models ; Autoantibodies/blood
    • Abstract:
      Lymphopenia is a unique manifestation of anti-MDA5 positive dermatomyositis with interstitial lung disease (MDA5 + DM-ILD). This study aimed to investigate the relationship between dynamic changes in peripheral lymphocytes and short-term prognosis in patients of MDA5 + DM-ILD. Two hundred sixty-three MDA5 + DM-ILD patients were divided into different groups according to lymphocyte count and death or survival within 1 month, then the differences in clinical features and outcomes were compared. Associations between lymphocytes and risk of death within 1 month were also investigated in different lymphocyte groups using Cox proportional hazard models. A generalized additive mixed model (GAMM) was established to analyze the dynamic changes of lymphocytes in the death 1-month group. Lymphocytes of the patients who died within 1 month were significantly lower than survivors by different lymphocyte grouping methods, and the total lymphocytes showed a gradually decreasing trend in non-survivors. And the difference between survivors and non-survivors was more obvious over time. The lowest tertile of baseline lymphocytes as a reference, the hazard ratios for death within 1 month in the highest tertile were 0.497 (95% CI 0.26-0.949, P for trend = 0.033) after adjustment for potential confounders. GAMM analysis found a mean daily decrease of lymphocytes (0.034 × 10^9/L) after admission in death 1-month patients. Low baseline lymphocytes and gradually declined lymphocytes are both associated with a high risk of death within 1 month. However dynamic changes in lymphocytes can better reflect the disease status and better predict the short-term prognosis than baseline lymphocytes in MDA5 + DM-ILD patients. Key points •Low baseline lymphocytes and gradually decreased trend along time correlated with poor short-term prognosis in MDA5 + DM-ILD patients. •Dynamic changes of lymphocytes can better reflect the disease status and better predict the 1-month prognosis than baseline lymphocytes in MDA5 + DM-ILD patients. •Generalized additive mixed model (GAMM) analysis found that in 1-month non-survivors, peripheral blood lymphocytes decreased by 0.034 × 10^9/L per day, while the lymphocytes in survivors gradually increased.
      (© 2024. The Author(s).)
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    • Grant Information:
      No. BE2016611 Jiangsu Provincial Key Research and Development Program; 81970063 National Natural Science Foundation of China; 81900061 National Natural Science Foundation of China
    • Contributed Indexing:
      Keywords: Dermatomyositis; Interstitial lung disease; MDA5; Peripheral lymphocyte count
    • Accession Number:
      EC 3.6.4.13 (Interferon-Induced Helicase, IFIH1)
      EC 3.6.1.- (IFIH1 protein, human)
      0 (Autoantibodies)
    • Publication Date:
      Date Created: 20240918 Date Completed: 20241018 Latest Revision: 20241114
    • Publication Date:
      20241114
    • Accession Number:
      PMC11489275
    • Accession Number:
      10.1007/s10067-024-07110-3
    • Accession Number:
      39292419