Tumor Necrosis Factor Inhibitors Provide Longterm Clinical Benefits in Pediatric and Young Adult Patients with Blau Syndrome
Bibliographic record
Abstract
To the Editor: Blau syndrome is an autoinflammatory disease caused by mutations in the NOD2 gene1. The initial symptoms of Blau syndrome usually include polyarthritis and rash followed by uveitis. In addition, various clinical manifestations beyond the classic clinical triad have been reported in patients with Blau syndrome2,3,4. Although controlling ocular and articular involvements are critical to improve prognosis, no specific therapy for Blau syndrome has yet been established. The use of thalidomide, immunosuppressives [methotrexate (MTX) or azathioprine], and biologic therapy [tumor necrosis factor-α (TNF-α) or interleukin (IL)-1β inhibitor] has been reported in corticosteroid-refractory cases1,4,5,6; however, the small number of patients with Blau syndrome in these studies and case reports makes drawing a definite conclusion difficult. To address these issues, we report a series of 6 patients (3 men and 3 women) with Blau syndrome who received biologic therapy between 2005 and 2013 at the Kagoshima University Hospital, Kagoshima, Japan. Clinical findings and laboratory data were collected from their medical records. No patient had achieved clinical remission with prior treatments, including nonsteroidal antiinflammatory drugs, MTX, and systemic … Address correspondence to Dr. H. Wakiguchi, Department of Pediatrics, Yamaguchi University Graduate School of Medicine, 1-1-1 Minamikogushi, Ube, Yamaguchi 755-8505, Japan. E-mail: hiroyuki{at}yamaguchi-u.ac.jp
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How this classification was reachedexpand
Full frame machine prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.
Distilled classifier scores by category (both heads)
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.000 | 0.003 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.001 | 0.000 |
| Bibliometrics | 0.000 | 0.000 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.000 | 0.001 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.001 | 0.001 |
| Insufficient payload (model declined to judge) | 0.001 | 0.000 |
Machine scores (provisional)
The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.
Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.
How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".