title: Intravenous immunoglobulin contributes to the control of antimelanoma differentiation-associated protein 5 antibody-associated dermatomyositis with palmar violaceous macules/papules.
abstract: Autoantibodies to melanoma differentiation-associated protein 5 (MDA5) are associated with a subset of patients with dermatomyositis (DM) who have rapidly progressive interstitial lung disease (RP-ILD) with poor prognosis. Intensive immunosuppressive therapy is initiated before irreversible lung damage can occur; however, there are few lines of evidence for the treatment of RP-ILD. Here, we report three cases of anti-MDA5 antibody-associated DM with RP-ILD in which the patients were treated with combined-modality therapy, including high-dose prednisolone, tacrolimus, intravenous cyclophosphamide and intravenous immunoglobulin (IVIG). In all three cases, serum ferritin levels, which are known to represent the disease activity of RP-ILD, were decreased after IVIG administration. IVIG might contribute to the control of the disease activity of anti-MDA5 antibody-positive DM. Moreover, palmar violaceous macules/papules around the interphalangeal joints, which was observed in all three cases in the incipient stage, might be a useful sign in suggesting a diagnosis of anti-MDA5 antibody-associated DM.
authors.affiliation: Department of Dermatology, Faculty of Medicine, University of Tsukuba, Ibaraki, Japan.
authors.forename: H
authors.initials: H
authors.lastname: Koguchi-Yoshioka
authors.affiliation: Department of Dermatology, Faculty of Medicine, University of Tsukuba, Ibaraki, Japan.
authors.forename: N
authors.identifier: http://orcid.org/0000-0002-5398-0773
authors.initials: N
authors.lastname: Okiyama
authors.affiliation: Department of Dermatology, Faculty of Medicine, University of Tsukuba, Ibaraki, Japan.
authors.forename: K
authors.initials: K
authors.lastname: Iwamoto
authors.affiliation: Department of Dermatology, Faculty of Medicine, University of Tsukuba, Ibaraki, Japan.
authors.forename: Y
authors.initials: Y
authors.lastname: Matsumura
authors.affiliation: Department of Dermatology, Faculty of Medicine, University of Tsukuba, Ibaraki, Japan.
authors.forename: T
authors.initials: T
authors.lastname: Ogawa
authors.affiliation: Department of Dermatology, Faculty of Medicine, University of Tsukuba, Ibaraki, Japan.
authors.forename: S
authors.initials: S
authors.lastname: Inoue
authors.affiliation: Department of Dermatology, Faculty of Medicine, University of Tsukuba, Ibaraki, Japan.
authors.forename: R
authors.initials: R
authors.lastname: Watanabe
authors.affiliation: Department of Dermatology, Faculty of Medicine, University of Tsukuba, Ibaraki, Japan.
authors.forename: M
authors.initials: M
authors.lastname: Fujimoto
mesh_terms: D000368:Aged; D000893:Anti-Inflammatory Agents / Q000008:administration & dosage; D001323:Autoantibodies / Q000097:blood; D003131:Combined Modality Therapy; D003520:Cyclophosphamide / Q000008:administration & dosage; D003882:Dermatomyositis / Q000517:prevention & control*; D005260:Female; D006229:Hand Dermatoses / Q000517:prevention & control*; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D007166:Immunosuppressive Agents / Q000008:administration & dosage; D000072640:Interferon-Induced Helicase, IFIH1 / Q000276:immunology*; D017563:Lung Diseases, Interstitial / Q000517:prevention & control*; D008297:Male; D008875:Middle Aged; D011239:Prednisolone / Q000008:administration & dosage; D016559:Tacrolimus / Q000008:administration & dosage; D016896:Treatment Outcome
chemical_list: D000893:Anti-Inflammatory Agents; D001323:Autoantibodies; D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D011239:Prednisolone; C452815:IFIH1 protein, human; D000072640:Interferon-Induced Helicase, IFIH1; D016559:Tacrolimus