Clinical features and prognostic factors of anti‐melanoma differentiation‐associated gene 5 antibody‐positive dermatomyositis with rapidly progressive interstitial lung disease in Chinese patients

Li, Lian; Tong, Jing-Jing; Xu, Shuoyu

doi:10.1002/iid3.882

Cited by 2 publications

References 25 publications

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Elevated serum gamma-glutamyl transferase level as a predictor of mortality in patients with anti-MDA5 antibody-positive dermatomyositis

Hu,

Zhang,

Ding

et al. 2024

Preprint

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Background Gamma-glutamyl transferase (GGT) has been identified to correlate with systemic inflammation in autoimmune diseases, while the role of GGT in anti-melanoma differentiation-associated protein 5 antibody-positive dermatomyositis (MDA5 + DM) remains unknown. This study aimed to investigate the clinical and prognostic significance of serum GGT in MDA5 + DM patients. Methods Patients with MDA5 + DM admitted to the First Affiliated Hospital of Zhengzhou University between February 2019 and May 2023 were retrospectively analyzed. We compared the clinical features and prognosis between MDA5 + DM patients with elevated serum GGT levels and those with normal serum GGT levels. Cox regression analysis was performed to identify independent factors associated with mortality. Results A total of 299 MDA5 + DM patients were enrolled in this study. During the median follow-up time of 13.1(4.4–28.1) months, 153(51.2%) patients developed rapidly progressive interstitial lung disease (RP-ILD) and 75(25.1%) patients died within 6 months after disease onset. Serum GGT levels were significantly higher in the death group compared to the survival group [95(56–165) vs 45(26–90) U/L, p<0.001]. Based on the serum GGT levels at the time of diagnosis, we divided all the patients into two groups: normal GGT group (GGT ≤ 58U/L, n = 155) and elevated GGT group (GGT>58U/L, n = 144). Compared with the normal GGT group, patients in the elevated GGT group had increased incidences of skin ulcer and RP-ILD, higher levels of lactate dehydrogenase (LDH), Krebs Von den Lungen-6 (KL-6), ferritin and C-reactive protein (CRP), while lower levels of albumin and lymphocyte counts. Moreover, the Kaplan–Meier survival analysis demonstrated that the cumulative survival rate was significantly lower in the elevated GGT group than that in the normal GGT group (log-rank p < 0.001). Multivariate Cox regression analysis revealed that RP-ILD, GGT>58U/L, LDH>345U/L, CRP>5mg/L and anti-Ro52 antibody positivity were independent risk factors of mortality in MDA5 + DM patients. Conclusions Elevated serum GGT level was an independent risk factor for mortality in MDA5 + DM patients. As a novel and readily available predictor, serum GGT level may help clinicians in guiding prognostic stratification and personalized treatment.

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Elevated serum gamma-glutamyl transferase level as a predictor of mortality in patients with anti-MDA5 antibody-positive dermatomyositis

Hu,

Zhang,

Ding

et al. 2024

Preprint

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Baricitinib and Pulse Steroids Combination Treatment in Hyperinflammatory COVID-19: A Rheumatological Approach in the Intensive Care Unit

Ferro,

La Rocca,

Elefante

et al. 2024

IJMS

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Hyperinflammatory Coronavirus disease 2019 (COVID-19) and rapidly-progressive interstitial lung diseases (RP-ILD) secondary to inflammatory myopathies (IIM) present important similarities. These data support the use of anti-rheumatic drugs for the treatment of COVID-19. The aim of this study was to compare the efficacy of combining baricitinib and pulse steroids with the Standard of Care (SoC) for the treatment of critically ill COVID-19 patients. We retrospectively enrolled consecutive patients admitted to the Intensive Care Unit (ICU) with COVID-19-pneumonia. Patients treated with SoC (dexamethasone plus remdesivir) were compared to patients treated with baricitinib plus 6-methylprednisolone pulses (Rheuma-group). We enrolled 246 patients: 104/246 in the SoC and 142/246 in the Rheuma-group. All patients presented laboratory findings suggestive of hyperinflammatory response. Sixty-four patients (26.1%) died during ICU hospitalization. The mortality rate in the Rheuma-group was significantly lower than in the SoC-group (15.5 vs. 40.4%, p < 0.001). Compared to the SoC-group, patients in the Rheuma-group presented significantly lower inflammatory biomarker levels after one week of treatment. Higher ferritin levels after one week of treatment were strongly associated with mortality (p < 0.001). In this large real-life COVID-19 cohort, baricitinib and pulse steroids led to a significant reduction in mortality, paralleled by a prompt reduction in inflammatory biomarkers. Our experience supports the similarities between hyperinflammatory COVID-19 and the IIM-associated RP-ILD.

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Clinical features and prognostic factors of anti‐melanoma differentiation‐associated gene 5 antibody‐positive dermatomyositis with rapidly progressive interstitial lung disease in Chinese patients

Cited by 2 publications

References 25 publications

Elevated serum gamma-glutamyl transferase level as a predictor of mortality in patients with anti-MDA5 antibody-positive dermatomyositis

Elevated serum gamma-glutamyl transferase level as a predictor of mortality in patients with anti-MDA5 antibody-positive dermatomyositis

Baricitinib and Pulse Steroids Combination Treatment in Hyperinflammatory COVID-19: A Rheumatological Approach in the Intensive Care Unit

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