Intravenous immunoglobulin for interstitial lung diseases of anti-melanoma differentiation-associated gene 5-positive dermatomyositis

Abstract

Objective Rapidly progressive interstitial lung disease (RP-ILD) in dermatomyositis (DM) patients positive for anti–melanoma differentiation–associated gene 5 (anti–MDA5) autoantibody (MDA5-DM) often have poor prognosis, frequently fatal. As there is a scarcity of data regarding the effect of intravenous immunoglobulin (IVIG) on RP-ILD in MDA5-DM patients (MDA5-RPILD), we conducted this study to determine the efficacy of a IVIG add-on initial treatment. Methods Patients with newly-onset MDA5-RPILD from September 2018 to June 2020 were retrospectively reviewed for 6 months in the First Affiliated Hospital of Zhengzhou University. They were divided into two groups: IVIG and non-IVIG groups. The major measurement of treatment outcome was the difference in the mortality in 3-month and 6-month between two group patients. Other relevant indicators were also recorded, including the incidence of infection, the dosages of GCs, the remission rate and the variables in laboratory data. Results The IVIG group (n = 31) showed significantly lower 6-month mortality rate than the non-IVIG group (n = 17) (22.6% vs 52.9%; p= 0.033). The IVIG group patients had a higher remission rate at 3 months (71.0% vs 41.2%; p= 0.044). Gradual reduction was observed in the first 3 months with regards to the titer of anti–MDA5 autoantibody, the serum level of ferritin, and the GGO scores. Conclusion IVIG adjunct therapy is a very effective first-line treatment for patients with MDA5-RPILD. IVIG may increase the survival and remission rate by lowering ferritin concentration, anti-MDA5 titer and GGO score.

The clinical features and prognoses of anti-MDA5 and anti-aminoacyl-tRNA synthetase antibody double-positive dermatomyositis patients

“…In our cohort, IVIG was more commonly used in anti-MDA5+/ARS+ DM compared to anti-MDA5-/ARS+ DM. Its value arises from the inhibition of cytokine production and enhancement of the clearance of pathogenic autoantibodies ( 32 ). Thus, IVIG can also be considered in these patients.…”

Novel Therapeutic Approaches in Connective Tissue Disease-Associated Interstitial Lung Disease

“…106,107 There are case reports and case series in both ASS and anti-MDA5-positive ILD suggesting benefit in some patients. [107][108][109][110][111][112][113] A retrospective review of 11 patients treated with IVIG for refractory ASS-ILD was performed at a single center. Patients who were treated with IVIG for nonpulmonary indications or who had rituximab initiation within 6 months prior to IVIG were excluded.…”

“…There was also higher rate of remission at 3 months in the IVIG group (71 vs. 41.2%, p ¼ 0.044). 112…”

Targeting intracellular pathways in idiopathic inflammatory myopathies: A narrative review

“…Furthermore, MDA5 is physiologically involved in the IFN-mediated response to viral infections and anti-MDA5 autoantibodies are thought to be directly pathogenic in ADM (90). Accordingly, plasma exchange and intravenous immunoglobulins showed efficacy in the treatment of both COVID-19 and anti-MDA5 ILD (91)(92)(93).…”