A25: The Association of Immunogenetic and Environmental Factors with Disease Course in Patients with Juvenile Idiopathic Inflammatory Myopathies
Notice bibliographique
Résumé
Background/Purpose: Three disease courses can be distinguished in patients with juvenile idiopathic inflammatory myopathies (JIIM): monocyclic (M), polycyclic (P), and chronic continuous (CC). Predictors of disease course could result in improved therapy and help focus research efforts. We examined the association of immunogenetic and environmental factors with disease course in a large JIIM cohort. Methods: We enrolled 240 Caucasian patients with JIIM (204 dermato‐, 21 poly‐, and 15 overlap myositis) diagnosed from 1980–2010 in the US and Canada into IRB‐approved studies. The ratio of girls to boys was 3:1. Median age at diagnosis was 7 years (IQR: 5–12). Physician questionnaires with clinical and demographic data and patient blood samples were obtained. Follow‐up was through medical record review. The disease course classification was as follows: M—no active disease and off medication within 2 years of diagnosis (n = 62); P—disease recurrence after definite remission (n = 62); and CC—persistent disease or continuation of medication for more than 2 years (n = 116). Genetic data included: high resolution HLA‐DRB1 and HLA‐DQA1 alleles, and peptide binding motifs; immunoglobulin gamma heavy and kappa light chain phenotypes and allotypes; and TNFα (−238, −308), IL‐1α (−889, +4845), and IL‐1β (−511, −3953) polymorphisms. Environmental data included: documentation of infections within 6 months prior to illness onset; average and highest ultraviolet (UV) index for the month prior to illness onset and prior to diagnosis based on residential location; season of illness onset; and geoclimatic regions and planting zones of residential location at illness onset. Results: HLA‐DRB1*1501 was present more frequently in the M group (22%) compared to the P (5%; OR [95% CI]=5.7 [1.1–29.0]; p=0.04) and CC groups (7%; OR=4.0 [12.4–1.3]; p=0.02). In patients with dermatomyositis only, the F25 peptide binding was present less frequently in the M group (18%) compared to the P (41%; OR = 0.3 [0.1–0.8]; p = 0.02) and CC groups (39%; OR = 0.3 [0.1–0.8]; p = 0.02). The other HLA alleles and peptide binding motifs, immunoglobulin gamma heavy and kappa light chain phenotypes and allotypes, and cytokine polymorphisms (including TNFα‐308) were not associated with disease course. Infections 6 months prior to illness onset were more often present in the P group (40%) compared to the M (18%; OR = 3.0 [1.3–7.2]; p = 0.01) and CC groups (21%; OR = 2.5 [1.2–5.2]; p = 0.01). Three‐quarter of these infections were respiratory. In girls, the average UV index in the month before diagnosis was higher in the P group (median [IQR] = 5.7 [4.0–6.6]) compared to the M group (median [IQR] = 4.3 [1.2–5.6]; p = 0.01). Season of illness onset, geoclimatic regions, and planting zones did not differ among the 3 disease courses. Conclusion: Immunogenetic factors, including HLA alleles, and environmental factors, including a history of preceding infections and the UV index at diagnosis, were significantly associated with disease course in this population of Caucasian patients with JIIM. Future studies are needed to confirm these associated factors and to identify the value of these factors in determining prognosis.
Récupéré en direct depuis OpenAlex et désinversé. Les résumés ne sont pas conservés dans cette base de données : les index inversés représentent 8,6 Go des 9,3 Go de texte de la base, et le serveur dispose de 13 Go libres.
Comment cette classification a été obtenuedéplier
Prédiction machine sur la base complète
Imitation des enseignantsNi prévalence calibrée, ni vérité terrain. Validation humaine à venir. Le volet Gemma est une étiquette directe du modèle pour chaque travail de la base, lue sur la notice réduite au titre. Le volet Codex est un classifieur appris des 10 348 étiquettes directes de Codex et calibré sur les taux pondérés de l'échantillon; les champs sans appui suffisant ne portent aucun appel Codex. Le mode candidate est l'union des deux volets; le consensus est leur intersection. Ces sorties portent le statut machine_predicted_unvalidated et ne sont pas des étiquettes humaines.
Scores du classifieur distillé par catégorie (deux têtes)
| Catégorie | Codex | Gemma |
|---|---|---|
| Métarecherche | 0,000 | 0,002 |
| Méta-épidémiologie (sens strict) | 0,000 | 0,000 |
| Méta-épidémiologie (sens large) | 0,000 | 0,000 |
| Bibliométrie | 0,001 | 0,001 |
| Études des sciences et des technologies | 0,001 | 0,000 |
| Communication savante | 0,000 | 0,000 |
| Science ouverte | 0,000 | 0,000 |
| Intégrité de la recherche | 0,000 | 0,001 |
| Charge utile insuffisante (le modèle a refusé de juger) | 0,002 | 0,000 |
Scores machine (provisoires)
Les deux têtes enseignantes du modèle étudiant, lues sur ce travail. Un score ordonne la base pour la relecture; il n'affirme jamais une catégorie, et le statut de validation accompagne chaque rangée tel quel.
Scores de référence d'un modèle non mature (critères de maturité non atteints, 7 itérations). Un score ordonne; il n'affirme jamais une catégorie.
score_only:v0-immature-baseline · tel quel depuis la passe de notation : score_only signifie que le nombre peut ordonner les travaux, et qu'aucune étiquette de catégorie n'en découleClassification
machine, non validéePrédiction automatique; un appel candidat d’une seule source (Gemma direct ou Codex distillé), pas un consensus.
Le détail, modèle par modèle et score par score, se trouve en fin de page sous « Comment cette classification a été obtenue ».