Deficiency of Interleukin-1 Receptor Antagonist: A Case with Late Onset Severe Inflammatory Arthritis, Nail Psoriasis with Onychomycosis and Well Responsive to Adalimumab Therapy
المؤلفون المشاركون
Aksu, Guzide
Akarcan, Sanem
Moriya, Kunihiko
Migaud, Melanie
Puel, Anne
Casanova, Jean-Laurent
Kutukculer, Necil
Karaca, Neslihan
المصدر
العدد
المجلد 2019، العدد 2019 (31 ديسمبر/كانون الأول 2019)، ص ص. 1-6، 6ص.
الناشر
Hindawi Publishing Corporation
تاريخ النشر
2019-08-04
دولة النشر
مصر
عدد الصفحات
6
التخصصات الرئيسية
الملخص EN
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.
نمط استشهاد جمعية علماء النفس الأمريكية (APA)
Kutukculer, Necil& Puel, Anne& Akarcan, Sanem& Moriya, Kunihiko& Karaca, Neslihan& Migaud, Melanie…[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،Vol. 2019, no. 2019, pp.1-6.
https://search.emarefa.net/detail/BIM-1136863
نمط استشهاد الجمعية الأمريكية للغات الحديثة (MLA)
Kutukculer, Necil…[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 No. 2019 (2019), pp.1-6.
https://search.emarefa.net/detail/BIM-1136863
نمط استشهاد الجمعية الطبية الأمريكية (AMA)
Kutukculer, Necil& Puel, Anne& Akarcan, Sanem& Moriya, Kunihiko& Karaca, Neslihan& Migaud, Melanie…[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. Vol. 2019, no. 2019, pp.1-6.
https://search.emarefa.net/detail/BIM-1136863
نوع البيانات
مقالات
لغة النص
الإنجليزية
الملاحظات
Includes bibliographical references
رقم السجل
BIM-1136863
قاعدة معامل التأثير والاستشهادات المرجعية العربي "ارسيف Arcif"
أضخم قاعدة بيانات عربية للاستشهادات المرجعية للمجلات العلمية المحكمة الصادرة في العالم العربي
تقوم هذه الخدمة بالتحقق من التشابه أو الانتحال في الأبحاث والمقالات العلمية والأطروحات الجامعية والكتب والأبحاث باللغة العربية، وتحديد درجة التشابه أو أصالة الأعمال البحثية وحماية ملكيتها الفكرية. تعرف اكثر