Case Report

Vol. 2 No. 2 (2015): European Journal of Rheumatology
DOI: https://doi.org/10.5152/eurjrheum.2015.0076

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Published: Sep 3, 2019
Keywords:
Amyopathic, dermatomyositis, interstitial pneumonitis, anti-MDA5 antibody

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Kazu Hamada-Ode
Department of Endocrinology, Metabolism, Nephrology and Rheumatology, Kochi University Medical School, Kochi, Japan
Yoshinori Taniguchi
Department of Endocrinology, Metabolism, Nephrology and Rheumatology, Kochi University Medical School, Kochi, Japan
Takahito Kimata
Department of Rheumatology, Misato Bay Side Medical Center, Kochi, Japan
Yasushi Kawaguchi
Department of Rheumatology, Tokyo Women’s Medical University, Tokyo, Japan
Yoshiko Shimamura
Department of Endocrinology, Metabolism, Nephrology and Rheumatology, Kochi University Medical School, Kochi, Japan
Masataka Kuwana
Department of Rheumatology, Nippon Medical School, Tokyo, Japan
Shimpei Fujimoto
Department of Endocrinology, Metabolism, Nephrology and Rheumatology, Kochi University Medical School, Kochi, Japan
Yoshio Terada
Department of Endocrinology, Metabolism, Nephrology and Rheumatology, Kochi University Medical School, Kochi, Japan

High-dose intravenous immunoglobulin therapy for rapidly progressive interstitial pneumonitis accompanied by anti-melanoma differentiation-associated gene 5 antibody-positive amyopathic dermatomyositis

Main Article Content

Kazu Hamada-Ode
Department of Endocrinology, Metabolism, Nephrology and Rheumatology, Kochi University Medical School, Kochi, Japan
Yoshinori Taniguchi
Department of Endocrinology, Metabolism, Nephrology and Rheumatology, Kochi University Medical School, Kochi, Japan
Takahito Kimata
Department of Rheumatology, Misato Bay Side Medical Center, Kochi, Japan
Yasushi Kawaguchi
Department of Rheumatology, Tokyo Women’s Medical University, Tokyo, Japan
Yoshiko Shimamura
Department of Endocrinology, Metabolism, Nephrology and Rheumatology, Kochi University Medical School, Kochi, Japan
Masataka Kuwana
Department of Rheumatology, Nippon Medical School, Tokyo, Japan
Shimpei Fujimoto
Department of Endocrinology, Metabolism, Nephrology and Rheumatology, Kochi University Medical School, Kochi, Japan
Yoshio Terada
Department of Endocrinology, Metabolism, Nephrology and Rheumatology, Kochi University Medical School, Kochi, Japan

Abstract

Abstract


Anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive amyopathic dermatomyositis (ADM) associated with rapidly progressive interstitial pneumonitis (RPIP) frequently has a poor prognosis and optimal treatment is not well defined. Here, we report a 62-year-old Japanese man with anti-MDA5 antibody-positive ADM associated with RPIP presented with progressive shortness of breath, Heliotrope rash, Gottron’s papules, arthralgia, and fatigue but no sign of muscle weakness. Laboratory investigation revealed serum levels of the following biomarkers: ferritin, 1393 ng/mL; Krebs von der Lungen-6, 1880 U/mL; and creatine kinase, 85 U/L. Computed tomography (CT) images showed diffuse ground-glass opacity in both lung fields. Because anti-MDA5 was positive, we made a diagnosis of ADM associated with RPIP and initiated treatment. Following five courses of combination therapy with prednisolone, cyclosporine A, and intravenous cyclophosphamide (IVCY), IVCY treatment was switched to high-dose intravenous immunoglobulin therapy (IVIg) because of the reactivation of interstitial pneumonia with an increased serum ferritin level. Additional treatment with IVIg improved RPIP, with normalization of anti-ADM antibody levels. Therefore, IVIg mayt be a new candidate treatment for anti-MDA5 antibody-positive ADM associated with RPIP.


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