Disabling pansclerotic morphoea: a century of discovery
Bibliographic record
Abstract
BACKGROUND: Disabling pansclerotic morphoea (DPM) is a rare systemic inflammatory disorder at the severe end of the localized scleroderma spectrum. It primarily affects children < 14 years old. DPM is characterized by rapid sclerosis with circumferential involvement that frequently extends to the fascia, muscle and bone. Disease progression often involves the development of sclerotic plaques, chronic skin ulcers and painful joint contractures, leading to patient immobility and a high mortality rate. Internal organ fibrosis is typically absent. The aggressive and systemic nature of DPM leads patients to seek multidisciplinary care. Current treatments are targeted toward immunomodulation and measures to preserve mobility while limiting infection, but they often have limited efficacy. OBJECTIVES: To summarize all patients with DPM reported in the English-language literature, common clinical symptoms, laboratory investigations and treatments reported to date. METHODS: A literature search was conducted on PubMed and Google Scholar. All English-language original articles, case reports, abstracts and letters to the editor were included. Each publication was reviewed for diagnosis, clinical presentation, available laboratory/histological studies, treatment and outcome. RESULTS: We identified 52 reports comprising 86 patients published up to December 2023. Assessment of published cases suggested that the number of treatments does not influence disease outcome and that female patients are younger at the time of reported death. CONCLUSIONS: Clinician familiarity and awareness of common DPM symptoms are important for an accurate and early diagnosis. Knowledge of treatments that have been reported to be effective in mitigating disease progression may be helpful in expanding the available treatment options.
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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 | 0.003 | 0.010 |
| Meta-epidemiology (narrow) | 0.001 | 0.000 |
| Meta-epidemiology (broad) | 0.002 | 0.001 |
| Bibliometrics | 0.009 | 0.008 |
| Science and technology studies | 0.001 | 0.002 |
| Scholarly communication | 0.003 | 0.006 |
| Open science | 0.001 | 0.001 |
| Research integrity | 0.002 | 0.002 |
| Insufficient payload (model declined to judge) | 0.002 | 0.001 |
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".