Severe Rheumatoid Arthritis Developing in Conjunction with Gorlin Syndrome
Bibliographic record
Abstract
To the Editor: Gorlin syndrome, also known as nevoid basal cell carcinoma syndrome, is a rare multisystemic genodermatosis classically characterized by the development of numerous basal cell carcinomas (BCC)1,2,3. Gorlin syndrome is caused by germline inactivating mutations in the patched ( PTCH ) gene, resulting in constitutively active hedgehog signaling1,2. The hedgehog signaling pathway is important in the regulation of stem cell and progenitor cell proliferation, the maintenance and regeneration of adult tissues, and finally in carcinogenesis, including the development of sporadic BCC2,4. Vismodegib, an oral inhibitor of hedgehog signaling, has demonstrated efficacy in Gorlin syndrome, as well as in patients with locally advanced or metastatic BCC2,4. Recently, dysregulated hedgehog signaling has also been implicated in the pathogenesis of rheumatoid arthritis (RA). Hedgehog signaling is highly active in the synovium of patients with RA in vivo , as well as in cultured fibroblast-like synoviocytes (FLS) from patients in vitro 5,6. In a mechanistically similar fashion to the malignant keratinocytes within … Address correspondence to Dr. J.F. Scott, University Hospitals Case Medical Center, 11100 Euclid Ave., Cleveland, Ohio 44106, USA. E-mail: jeffrey.scott{at}uhhospitals.org
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How this classification was reachedexpand
Full frame distilled prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. Learned from the 10,348 direct Codex labels and 10,348 direct Gemma labels. Candidate is the union of thresholded teacher heads; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels or direct frontier model labels.
Codex and Gemma teacher scores by category
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.001 | 0.000 |
| 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 | 0.000 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.001 | 0.001 |
| Insufficient payload (model declined to judge) | 0.000 | 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 teacher head, 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".