title: Antimelanoma differentiation-associated protein 5 antibody level is a novel tool for monitoring disease activity in rapidly progressive interstitial lung disease with dermatomyositis.
abstract: BACKGROUND
  Antimelanoma differentiation-associated protein (anti-MDA)5 antibodies are associated with rapidly progressive interstitial lung disease (RP-ILD) in patients with clinically amyopathic dermatomyositis (CADM) or dermatomyositis (DM).
  
  
  OBJECTIVE
  We aimed to evaluate the relevance of monitoring anti-MDA5 antibody levels for the management of RP-ILD in patients with CADM or DM.
  
  
  METHODS
  Twelve patients with CADM (n = 10) or DM (n = 2) accompanied by RP-ILD were included. Baseline characteristics and outcomes were recorded. Serial measurements of anti-MDA5 antibody levels were measured. All patients were treated with corticosteroids, tacrolimus and intravenous cyclophosphamide.
  
  
  RESULTS
  All patients achieved RP-ILD remission after combined immunosuppressive therapy for a mean of 6·8 months, with significant decreases noted in the mean anti-MDA5 antibody levels at remission. Six (50%) patients became anti-MDA5 antibody negative after therapy. After a mean follow-up of 31 months, RP-ILD relapse was observed in four (33%) patients in both the anti-MDA5 antibody sustained positive group and the negative conversion group. However, relapsed patients in the sustained positive group relapsed earlier than those in the negative conversion group. Thus, a decrease in anti-MDA5 antibody levels during remission was associated with longer remission. Relapses were associated with a reincrease of anti-MDA5 antibody levels in four of four (100%) patients. In contrast, none of the patients without reincrease in anti-MDA5 antibody exhibited symptoms of relapse during follow-up. Therefore, reincrease in anti-MDA5 antibody levels was associated with relapse.
  
  
  CONCLUSIONS
  The anti-MDA5 antibody level is a novel parameter for monitoring and a good predictor of RP-ILD relapse in patients with CADM or DM.
authors.affiliation: Department of Dermatology, Faculty of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, 920-8641, Japan.
authors.forename: T
authors.initials: T
authors.lastname: Matsushita
authors.affiliation: Department of Dermatology, Faculty of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, 920-8641, Japan.
authors.forename: K
authors.initials: K
authors.lastname: Mizumaki
authors.affiliation: Department of Dermatology, Faculty of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, 920-8641, Japan.
authors.forename: M
authors.initials: M
authors.lastname: Kano
authors.affiliation: Department of Dermatology, Faculty of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, 920-8641, Japan.
authors.forename: N
authors.initials: N
authors.lastname: Yagi
authors.affiliation: Department of Dermatology, Faculty of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, 920-8641, Japan.
authors.forename: M
authors.initials: M
authors.lastname: Tennichi
authors.affiliation: Department of Dermatology, Faculty of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, 920-8641, Japan.
authors.forename: A
authors.initials: A
authors.lastname: Takeuchi
authors.affiliation: Department of Dermatology, Faculty of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, 920-8641, Japan.
authors.forename: Y
authors.initials: Y
authors.lastname: Okamoto
authors.affiliation: Department of Dermatology, Faculty of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, 920-8641, Japan.
authors.forename: Y
authors.initials: Y
authors.lastname: Hamaguchi
authors.affiliation: Medical and Biological Laboratories Co., Ltd, Nagoya, 460-0008, Japan.
authors.forename: A
authors.initials: A
authors.lastname: Murakami
authors.affiliation: Department of Dermatology, University of Fukui, Fukui, 910-1193, Japan.
authors.forename: M
authors.initials: M
authors.lastname: Hasegawa
authors.affiliation: Department of Allergy and Rheumatology, Nippon Medical School, Graduate School of Medicine, Tokyo, 113-8603, Japan.
authors.forename: M
authors.initials: M
authors.lastname: Kuwana
authors.affiliation: Department of Dermatology, Faculty of Medicine, University of Tsukuba, Tennodai, Tsukuba, 305-8575, Japan.
authors.forename: M
authors.initials: M
authors.lastname: Fujimoto
authors.affiliation: Department of Dermatology, Faculty of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, 920-8641, Japan.
authors.forename: K
authors.initials: K
authors.lastname: Takehara
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D001323:Autoantibodies / Q000378:metabolism; D003520:Cyclophosphamide / Q000627:therapeutic use; D003879:Dermatologic Agents / Q000627:therapeutic use; D003882:Dermatomyositis / Q000188:drug therapy / Q000276:immunology*; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D000072640:Interferon-Induced Helicase, IFIH1 / Q000276:immunology; D017563:Lung Diseases, Interstitial / Q000188:drug therapy / Q000276:immunology*; D008297:Male; D008875:Middle Aged; D012008:Recurrence; D016896:Treatment Outcome
chemical_list: D000305:Adrenal Cortex Hormones; D001323:Autoantibodies; D003879:Dermatologic Agents; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; C452815:IFIH1 protein, human; D000072640:Interferon-Induced Helicase, IFIH1