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Deficiency of Interleukin-1 Receptor Antagonist: A Case with Late Onset Severe Inflammatory Arthritis, Nail Psoriasis with Onychomycosis and Well Responsive to Adalimumab Therapy

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  • معلومة اضافية
    • Publication Information:
      Hindawi Limited, 2019.
    • Publication Date:
      2019
    • Collection:
      LCC:Immunologic diseases. Allergy
    • Abstract:
      DIRA (deficiency of the IL-1Ra) is a rare condition that usually presents in the neonatal period. Patients with DIRA present with systemic inflammation, respiratory distress, joint swelling, pustular rash, multifocal osteomyelitis, and periostitis. Previously, we reported a patient with a novel mutation in IL1RN with a healthy neonatal period, a late-onset of pustular dermatosis, inflammatory arthritis, and excellent response to canakinumab treatment. Herein, we are presenting a new case of late-onset DIRA syndrome, carrying a different mutation and showing different clinical findings. This patient is the first one in the literature with the inflammatory arthritis, nail psoriasis, and onychomycosis and with her remarkable response to monoclonal antibodies. The case responded well and fully recovered after treatment with adalimumab, but not with canakinumab. The DIRA disease can lead to death from multiple organ failures and if recognized early, the treatment with replacement of the deficient protein with biologic agents induces rapid and complete remission. Therefore, clinical symptoms should be learned exactly by the pediatricians, pediatric rheumatologists, and immunologists; and molecular analysis targeting this defect must be performed as early as possible.
    • File Description:
      electronic resource
    • ISSN:
      2090-6609
      2090-6617
    • Relation:
      https://doaj.org/toc/2090-6609; https://doaj.org/toc/2090-6617
    • Accession Number:
      10.1155/2019/1902817
    • Rights:
      Journal Licence: CC BY
    • Accession Number:
      edsdoj.9922703a771e4f30854b1412a7efd86c
  • Citations
    • ABNT:
      NECIL KUTUKCULER et al. Deficiency of Interleukin-1 Receptor Antagonist: A Case with Late Onset Severe Inflammatory Arthritis, Nail Psoriasis with Onychomycosis and Well Responsive to Adalimumab Therapy. Case Reports in Immunology, [s. l.], v. 2019, 2019. DOI 10.1155/2019/1902817. Disponível em: http://search.ebscohost.com/login.aspx?direct=true&site=eds-live&db=edsdoj&AN=edsdoj.9922703a771e4f30854b1412a7efd86c&custid=s8280428. Acesso em: 27 maio. 2020.
    • AMA:
      Necil Kutukculer, Anne Puel, Sanem Eren Akarcan, et al. Deficiency of Interleukin-1 Receptor Antagonist: A Case with Late Onset Severe Inflammatory Arthritis, Nail Psoriasis with Onychomycosis and Well Responsive to Adalimumab Therapy. Case Reports in Immunology. 2019;2019. doi:10.1155/2019/1902817.
    • APA:
      Necil Kutukculer, Anne Puel, Sanem Eren Akarcan, Kunihiko Moriya, Neslihan Edeer Karaca, Melanie Migaud, Jean-Laurent Casanova, & Guzide Aksu. (2019). Deficiency of Interleukin-1 Receptor Antagonist: A Case with Late Onset Severe Inflammatory Arthritis, Nail Psoriasis with Onychomycosis and Well Responsive to Adalimumab Therapy. Case Reports in Immunology, 2019. https://doi.org/10.1155/2019/1902817
    • Chicago/Turabian: Author-Date:
      Necil Kutukculer, Anne Puel, Sanem Eren Akarcan, Kunihiko Moriya, Neslihan Edeer Karaca, Melanie Migaud, Jean-Laurent Casanova, and Guzide Aksu. 2019. “Deficiency of Interleukin-1 Receptor Antagonist: A Case with Late Onset Severe Inflammatory Arthritis, Nail Psoriasis with Onychomycosis and Well Responsive to Adalimumab Therapy.” Case Reports in Immunology 2019 (January). doi:10.1155/2019/1902817.
    • Harvard:
      Necil Kutukculer et al. (2019) ‘Deficiency of Interleukin-1 Receptor Antagonist: A Case with Late Onset Severe Inflammatory Arthritis, Nail Psoriasis with Onychomycosis and Well Responsive to Adalimumab Therapy’, Case Reports in Immunology, 2019. doi: 10.1155/2019/1902817.
    • Harvard: Australian:
      Necil Kutukculer, Anne Puel, Sanem Eren Akarcan, Kunihiko Moriya, Neslihan Edeer Karaca, Melanie Migaud, Jean-Laurent Casanova & Guzide Aksu 2019, ‘Deficiency of Interleukin-1 Receptor Antagonist: A Case with Late Onset Severe Inflammatory Arthritis, Nail Psoriasis with Onychomycosis and Well Responsive to Adalimumab Therapy’, Case Reports in Immunology, vol. 2019, viewed 27 May 2020, .
    • MLA:
      Necil Kutukculer, et al. “Deficiency of Interleukin-1 Receptor Antagonist: A Case with Late Onset Severe Inflammatory Arthritis, Nail Psoriasis with Onychomycosis and Well Responsive to Adalimumab Therapy.” Case Reports in Immunology, vol. 2019, Jan. 2019. EBSCOhost, doi:10.1155/2019/1902817.
    • Chicago/Turabian: Humanities:
      Necil Kutukculer, Anne Puel, Sanem Eren Akarcan, Kunihiko Moriya, Neslihan Edeer Karaca, Melanie Migaud, Jean-Laurent Casanova, and Guzide Aksu. “Deficiency of Interleukin-1 Receptor Antagonist: A Case with Late Onset Severe Inflammatory Arthritis, Nail Psoriasis with Onychomycosis and Well Responsive to Adalimumab Therapy.” Case Reports in Immunology 2019 (January 1, 2019). doi:10.1155/2019/1902817.
    • Vancouver/ICMJE:
      Necil Kutukculer, Anne Puel, Sanem Eren Akarcan, Kunihiko Moriya, Neslihan Edeer Karaca, Melanie Migaud, et al. Deficiency of Interleukin-1 Receptor Antagonist: A Case with Late Onset Severe Inflammatory Arthritis, Nail Psoriasis with Onychomycosis and Well Responsive to Adalimumab Therapy. Case Reports in Immunology [Internet]. 2019 Jan 1 [cited 2020 May 27];2019. Available from: http://search.ebscohost.com/login.aspx?direct=true&site=eds-live&db=edsdoj&AN=edsdoj.9922703a771e4f30854b1412a7efd86c&custid=s8280428