Association of atypical skin manifestations at the onset of systemic juvenile idiopathic arthritis with difficult-to-treat disease: A retrospective multicenter study

Eveillard, Laurye-Anne; Quartier, Pierre; Ouldali, Naïm; Bader‐Meunier, Brigitte; Aeschlimann, Florence; Abasq‐Thomas, C.; Ballot, Claire; Bouric, Pauline; Desdoits, A.; Dumaine, Cécile; Galeotti, Caroline; Hentgen, Véronique; Lefèvre‐Utile, Alain; Chausset, Aurélie; Hubiche, T.; Kupfer‐Bessaguet, I.; Leclerq-Mercier, Stéphanie; Mallet, S.; Melki, Isabelle; Merlin, Étienne; Miquel, J.; Piram, Maryam; Talmud, Déborah; Garcelon, Nicolas; Vinit, Caroline; Welfringer, A; Bourrat, E.; Meinzer, Ulrich

doi:10.1016/j.jaad.2022.07.055

Cited by 4 publications

(1 citation statement)

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“…Important indicators of DRESS used in the RegiSCAR scoring system 34 are fever, lymphadenopathy, rash, and organ involvement, which unfortunately overlap with typical characteristics of active sJIA 1,19,20 . Other features such as eosinophilia, nonevanescent pruritic skin rashes, and elevated liver enzymes, although sometimes still present, are less typical for sJIA and may indeed point to the possibility of drug hypersensitivity 35,36 . Because our patients were regularly sampled during the first 2 years (at least every month in the first 3 months and at least every 3 months thereafter), we assessed the occurrence of eosinophilia and liver enzyme elevations, stratified based on the HLA‐DRB1*15:01 background (Table 3).…”

Section: Resultsmentioning

confidence: 99%

Recombinant Interleukin‐1 Receptor Antagonist Is an Effective First‐Line Treatment Strategy in New‐Onset Systemic Juvenile Idiopathic Arthritis, Irrespective of HLA‐DRB1 Background and IL1RN Variants

Erkens

Calis

Verwoerd

et al. 2023

Arthritis & Rheumatology

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ObjectiveHLA‐DRB1*15:01 has been recently associated with interstitial lung disease (LD), eosinophilia, and drug reactions in systemic juvenile idiopathic arthritis (sJIA). Additionally, genetic variants in IL1RN have been linked to poor response to anakinra. We sought to reproduce these findings in a prospective cohort study of new‐onset sJIA patients treated with anakinra as first‐line therapy.MethodsHLA and IL1RN risk alleles were identified via whole genome sequencing. Treatment responses and complications were compared between carriers versus non‐carriers.ResultsSeventeen of 65 patients (26%) carried HLA‐DRB1*15:01, comparable to the general population, and there was enrichment for HLA‐DRB1*11:01, a known risk locus for sJIA. The rates of clinical inactive disease (CID) at 6 months, 1 and 2 years were generally high, irrespective of HLA‐DRB1 or IL1RN variants, but significantly lower in carriers of an HLA‐DRB1*11:01 allele. One patient, an HLA‐DRB1*15:01 carrier, developed sJIA‐LD. Of the three patients with severe drug reactions to biologics, one carried HLA‐DRB1*15:01. The prevalence of eosinophilia did not significantly differ between HLA‐DRB1*15:01 carriers and non‐carriers at disease‐onset (6.2% vs 14.9%, p=0.67) nor after the start of anakinra (35.3% versus 37.5% in the first 2 years of disease).ConclusionWe observed high rates of CID using anakinra as first‐line treatment irrespective of HLA‐DRB1 or IL1RN variants. Only one of the 17 HLA‐DRB1*15:01 carriers developed sJIA‐LD, and of the 3 patients with drug reactions to biologics, only one carried HLA‐DRB1*15:01. Although thorough monitoring for the development of drug hypersensitivity and refractory disease courses in sJIA, including sJIA‐LD remains important, our data support the early start of biological therapy in new‐onset sJIA patients irrespective of HLA‐DRB1 background or IL1RN variants.image

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Section: Resultsmentioning

confidence: 99%