Clinical and imaging characteristics of antisynthetase syndrome and anti-melanoma differentiation-associated gene 5 antibody positive dermatomyositis associated interstitial lung disease
WANG Pengfei
GUO Qian
JIANG Xiaopeng
DUAN Wei
Abstract:Objective:To explore the clinical features and imaging characteristics of the antisynthetase syndrome(ASS)and anti-melanoma differentiation-associated gene 5(MDA5)antibody-positive dermatomyositis(DM)as-sociated interstitial lung disease(ILD).Methods:Clinical case data(116 cases)of patients with ASS and anti-MDA5 antibody-positive DM admitted to the Shanxi Bethune Hospital from January 2018 to May 2023 were collected,and patients associated with ILD were further screened and divided into the ASS-ILD and MDA5+DM-ILD groups re-spectively.The clinical symptoms,imaging manifestations,treatment regimens,and prognosis of patients between the different groups were retrospectively analyzed.Results:The MDA5+DM group had a higher incidence of ILD compared to the ASS group.The prevalence of Gottron's sign,dyspnea symptoms,and rapidly progressive ILD was higher in the MDA5+DM-ILD group than in the ASS-ILD group.Muscle weakness symptoms were more common in the ASS-ILD group than in the MDA5+DM-ILD group,with a statistically significant difference(all P<0.05).Ground glass opacities and reticular opacities were commonly observed in both groups.Ground glass opacities and mediastinal emphy-sema were more common in the MDA5+DM-ILD group than in the ASS-ILD group,with a statistically significant difference(both P<0.05).The morbidity and mortality rates were significantly higher in the MDA54 DM-ILD group than in the ASS-ILD group,with a statistically significant difference(P<0.05).MDA5+DM-ILD patients treated with a triple-drug therapy of glucocorticoids+cyclophosphamide and another immunosuppressant(tacrolimus or to-facitinib)showed a decrease in the morbidity and mortality rate compared to the dual-drug therapy.Conclusion:The MDA5+DM group had a higher incidence of ILD compared to the ASS group.Patients with MDA5+DM-ILD had se-vere cutaneous lesions and dyspnea compared to patients with ASS-ILD,while myositis symptoms were milder than those of patients with ASS-ILD.Glass ground opacities and mediastinal emphysema were important imaging findings in the MDA5+DM-ILD group.
Keywords:interstitial lung diseaseantisynthetase antibody syndromeanti-melanoma differentiation-associated gene 5 antibodiesclinical featuresimaging characteristics
Publication Date:2024-04-10
Online Publishing Date:2025-08-15(First online date of this platform, not the publication date of the document)
Pages:6( 283-288 )
Proceeding of Clinical Medicine

Proceeding of Clinical Medicine

ISSN:1671-8631
Year, Vol.(Issue):2024,33(4)