Proposal of a new clinical entity: Paraprotein negative IL-1 mediated inflammatory dermatosis (PANID) that may precede Schnitzler syndrome
Why this work is in the frame
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Bibliographic record
Abstract
Schnitzler syndrome (SchS) is an autoinflammatory disease that is defined by the presence of 2 obligate criteria; an IgM or IgG monoclonal paraprotein and a chronic urticarial rash. Typically, there is an excellent clinical response to IL-1 antagonism. There are reports in the literature of a variant type of SchS that does not fulfil the 2 obligate criteria but responds to IL-1 blockade. Equally, there are reports of an urticarial rash preceding the development of a paraprotein by several years. We describe 3 cases in this manuscript. The first fits the Strasbourg diagnostic criteria of SchS, Simon and Asli (2013); however, with several decades of diagnostic delay. The second case at initial presentation did not fit the major criteria for SchS; however, later developed a monoclonal IgM. Finally we report, a third case that has not yet been confirmed to have a monoclonal IgM/IgG at the time of writing despite 12 years of symptoms and in whom a somatic autoinflammatory disorder remains within the differential. All cases responded strikingly to anakinra, an IL-1 receptor blocker. We propose a new clinical entity, paraprotein negative IL-1 mediated inflammatory dermatosis (PANID), that may act as a precursor or risk factor for the development of SchS or other autoinflammatory conditions.
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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 | 0.000 | 0.001 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.000 |
| Bibliometrics | 0.000 | 0.001 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.000 | 0.000 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.000 | 0.000 |
| 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 it