Complement C5-blocking Agent in Refractory Dermatomyositis
Bibliographic record
Abstract
Dermatomyositis (DM) is a rare autoimmune disease that belongs to the group of idiopathic inflammatory myopathies (IIM). The usual treatment of DM was based on steroids, methotrexate, and azathioprine. Intravenous immunoglobulin (IVIG), rituximab (RTX), or other biological treatments recently emerged as steroid-sparing agents1. About 10% to 30% of patients will develop a refractory or chronic persistent disease2, suggesting that alternative treatments should be tested. Here, we report a case of acute refractory DM with concomitant thrombotic microangiopathy (TMA) that improved with the C5-blocking agent eculizumab in the setting of other medications. According to the French law regarding observational retrospective studies (Jardé Law, 2016), written informed consent to publish the material was obtained from the patient, but approval by a specific ethics board was waived. A 19-year-old female patient, without medical history or medications, developed moderate arm and limb muscle weakness, Raynaud phenomenon, Gottron papules, mechanic’s hand, and edema of limb, arm, and periorbital regions. One month later, she was admitted to the hospital for severe acrocyanosis (Figure 1A–B), and examination showed elevated muscle enzymes [creatine … Address correspondence to Professor S. Faguer, Département de Néphrologie et Transplantation d’organes, INSERM U1048 (équipe 12, I2MC), Hôpital Rangueil 1, Avenue Jean Poulhes, 31000 Toulouse, France. E-mail: faguer.s{at}chu-toulouse.fr
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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 | 0.001 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.001 | 0.000 |
| Bibliometrics | 0.001 | 0.000 |
| Science and technology studies | 0.001 | 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) | 0.001 | 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".