We found a match
Your institution may have access to this item. Find your institution then sign in to continue.
- Title
IL‐1 receptor antagonist defect (DIRA) in a pediatric patient, receiving adalimumab with good clinical response.
- Authors
Bustamante‐Ogando, Juan Carlos; Scheffler‐Mendoza, Selma; Yamazaki‐Nakashimada, Marco Antonio; Saez‐de‐Ocariz, Marimar
- Abstract
IL-1 receptor antagonist defect (DIRA) in a pediatric patient, receiving adalimumab with good clinical response Dear Editor, The deficiency of interleukin-1 receptor antagonist (DIRA) is a rare autosomal recessive autoinflammatory syndrome (AIS) caused by loss-of-function mutations in I IL1RN i leading to an excessive proinflammatory response mediated by upregulated IL-1 .1 Clinical picture includes systemic inflammatory response and multi-organ involvement, particularly in skin, bone, and joints.1,2 The gold standard for treatment is anti-IL-1-directed therapy2,3; however, anti-IL-1 biologics are not available worldwide. Interestingly, Aksentijevich I et al i . showed in their initial report that homozygous DIRA patients had elevated serum IL-1, IL-6, IL-8, and TNF.1 We started anti-TNF therapy with adalimumab, and our patient showed a dramatic clinical response, which is currently maintained after 4 years.
- Subjects
CHILD patients; ADALIMUMAB; ONYCHOMYCOSIS; IDIOPATHIC femoral necrosis; INTERLEUKIN-1 receptors
- Publication
International Journal of Dermatology, 2021, Vol 60, Issue 5, p639
- ISSN
0011-9059
- Publication type
Article
- DOI
10.1111/ijd.15411