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Therapeutic plasma exchange for clinically amyopathic dermatomyositis (CADM) associated with rapidly progressive

Akira Yamagata1, Machiko Arita1, Ayaka Tanaka1

  • 1Department of Respiratory Medicine, Ohara Healthcare Foundation, Kurashiki Central Hospital, Okayama, Japan.

Journal of Clinical Apheresis
|August 19, 2020
PubMed
Abstract

Insights

Therapeutic plasma exchange (TPE) effectively treated rapidly progressive interstitial pneumonia (RPIP) in patients with clinically amyopathic dermatomyositis (CADM) and anti-MDA5 antibodies. TPE reduced anti-MDA5 antibody levels, suggesting a novel therapeutic strategy for refractory cases.

Area of Science:

  • Rheumatology
  • Immunology
  • Pulmonology

Background:

  • Clinically amyopathic dermatomyositis (CADM) with anti-melanoma differentiation-associated gene 5 antibody (anti-MDA5 Ab) often leads to fatal rapidly progressive interstitial pneumonia (RPIP).
  • Therapeutic plasma exchange (TPE) shows promise for CADM-RPIP refractory to standard immunosuppressive therapies, but its mechanisms remain unclear.

Observation:

  • This retrospective study analyzed three patients with CADM-RPIP treated with TPE between 2017 and 2018.
  • All patients tolerated TPE well and showed clinical improvement.

Findings:

  • TPE treatment led to a significant decrease in anti-MDA5 Ab titers.
  • Laboratory findings indicated improvement in disease activity following TPE.

Implications:

  • TPE is a viable and effective treatment option for CADM-RPIP unresponsive to conventional therapies.
  • The removal of anti-MDA5 Ab and other pathogenic factors via TPE may contribute to favorable clinical outcomes.
Keywords:
anti-melanoma differentiation-associated gene 5 antibodyapheresisclinically amyopathic dermatomyositisrapidly progressive interstitial pneumoniatherapeutic plasma exchange

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