Successful Rescue Therapy With Daratumumab in Rapidly Progressive Interstitial Lung Disease Caused by MDA5-Positive Dermatomyositis

Article Clinical Neurology

Daratumumab for treatment-refractory antibody-mediated diseases in neurology

Franziska Scheibe et al.

Summary: This study retrospectively examined the efficacy of daratumumab in patients with antibody-mediated autoimmune diseases. The results showed that daratumumab treatment led to significant clinical improvement and reduction in autoantibody production.

EUROPEAN JOURNAL OF NEUROLOGY (2022)

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Article Rheumatology

British Society for Rheumatology guideline on management of paediatric, adolescent and adult patients with idiopathic inflammatory myopathy

Alexander G. S. Oldroyd et al.

RHEUMATOLOGY (2022)

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Review Rheumatology

Management of MDA-5 antibody positive clinically amyopathic dermatomyositis associated interstitial lung disease: A systematic review

Mark McPherson et al.

Summary: Anti-MDA5 antibody-positive clinically amyopathic dermatomyositis (CADM) is often associated with rapidly progressive interstitial lung disease (RP-ILD) and high mortality rates. The management of this condition is challenging due to a lack of sufficient data. Early initiation of a combined immunosuppressive therapeutic regimen, including high-dose systemic glucocorticoids and other immunosuppressive agents, appears to improve survival rates in RP-ILD patients. Additional therapies such as plasma exchange, tofacitinib, and rituximab may be considered for refractory cases. Early detection and treatment are crucial for improving outcomes in this subset of patients.

SEMINARS IN ARTHRITIS AND RHEUMATISM (2022)

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Review Immunology

Dermatomyositis With Anti-MDA5 Antibodies: Bioclinical Features, Pathogenesis and Emerging Therapies

Anais Nombel et al.

Summary: Anti-MDA5 dermatomyositis is a rare systemic autoimmune disease with a diverse clinical spectrum, with three distinct clinical phenotypes defined by different prognosis. Specific antibodies targeting MDA5 are the only known molecular component shared by the three entities. Current knowledge on the pathogenic mechanisms of the disease is still in its infancy, but evidence supports a central role of interferon-mediated vasculopathy in lesion development.

FRONTIERS IN IMMUNOLOGY (2021)

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Letter Hematology