Four Cases of Anti-PM/Scl Antibody-positive Juvenile Overlap Syndrome with Features of Myositis and Systemic Sclerosis
Bibliographic record
Abstract
To the Editor: Overlap syndromes of systemic sclerosis (SSc)/myositis are rare and have primarily been described in adults. Relatively few cases of childhood SSc/myositis overlap have been reported1,2,3,4,5,6,7. In our case series, 4 patients diagnosed with juvenile overlap syndrome of myositis with SSc are described (Table 1). All 4 cases were positive for anti-PM/Scl and eventually developed symptoms consistent with overlap SSc/myositis. View this table: Table 1. Four pediatric patients with SSc/myositis overlap syndrome. Patient 1 was an 8-year-old white male who presented with diffuse hand swelling, tight skin, and contractures of the metacarpophalangeal (MCP), proximal interphalangeal (PIP), and distal interphalangeal joints and wrists of 3-month duration. His muscle strength was normal. He had Gottron papules over his MCP and PIP joints, cracking of the tips of his fingers and toes (without ulceration), and erythema over his elbows and knees. He denied Raynaud phenomenon (RP), fatigue, dysphagia, or dyspnea. Myositis antibody panel demonstrated positive anti-PM/Scl antibodies, and he was diagnosed with overlap SSc/myositis syndrome. One year after diagnosis, he had substantial improvement in his functional ability while receiving therapy, but not complete symptom resolution. Patient 2 was an African American female diagnosed with juvenile dermatomyositis (JDM) at age 6 when she … Address correspondence to Dr. L. Fotis, Pediatrics, Washington University in St. Louis School of Medicine, One Children’s Place, St. Louis, Missouri 63110-1010, USA. E-mail: Fotis_l{at}kids.wustl.edu
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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 | 0.001 | 0.004 |
| Meta-epidemiology (narrow) | 0.001 | 0.001 |
| Meta-epidemiology (broad) | 0.001 | 0.001 |
| Bibliometrics | 0.001 | 0.001 |
| Science and technology studies | 0.001 | 0.001 |
| Scholarly communication | 0.001 | 0.001 |
| Open science | 0.002 | 0.001 |
| Research integrity | 0.004 | 0.002 |
| Insufficient payload (model declined to judge) | 0.003 | 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".