Definition of relapse criteria in patients with rapidly progressive systemic sclerosis treated with autologous haemopoietic stem cell transplantation
Bibliographic record
Abstract
Systemic sclerosis (SSc) is a rare systemic autoimmune disease characterized by the accumulation of extracellular collagen matrix in tissues and target organs, such as skin, lung, gut, and heart [ 1 ]. The clinical spectrum of SSc is largely heterogeneous, but usually two distinct forms are recognized., i.e., the limited cutaneous (lc) and the diffuse cutaneous (dc) SSc. The two variants strongly differ in the terms of skin extension, type and severity of internal organ involvement, and expected survival. Among patients with dcSSc, a subset may be characterized by a rapidly progressive course with early appearance and quick worsening of skin and internal organ involvement, and consequently a high mortality rate within the first five years after first non-Raynaud phenomenom symptoms [ 2 ]. In these cases of rapidly progressive dcSSc, autologous haematopoietic stem cell transplantation (AHSCT) has been recognized as a standard-of-care therapy option since 2017 [ 3 , 4 , 5 , 6 ]. This statement was the direct consequence of the consistent results obtained in three randomized controlled trials where this procedure had been shown to be superior to traditional immunosuppressive therapy in improving skin involvement, preserving lung function, and reducing mortality rates [ 6 ]. These results have been further confirmed in a recent retrospective study where AHSCT was shown to be superior to rituximab in improving all the above-mentioned outcomes [ 7 ]. Nevertheless, several aspects of AHSCT warrant further consideration. First, transplantation related mortality still exists, although it has been significantly reduced thanks to the important progress made, with better pretransplant evaluation of cardiac and pulmonary involvement and improved selection of patients at lower risk of complications [ 8 ]. Another question to be answered is how long the effects of AHSCT will last. Preliminary data indicate that the incidence of disease progression could happen between 4 and 6 years after transplantation and that disease response varied according to patients [ 9 ]. However, although the observed clinical response after AHSCT has been defined since the early pivotal trials (ASSIT, ASTIS, SCOT) and the absence of response or disease progression after AHSCT is easy to define as its counterpart, no dedicated and validated tools are currently available to precisely define the disease relapse after AHSCT. Acquiring the moment of relapse after prior response to AHSCT may make it easier to adopt therapeutic interventions that may allow to maintain the disease remission induced by AHSCT.
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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 | 0.002 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.001 | 0.001 |
| Bibliometrics | 0.002 | 0.001 |
| Science and technology studies | 0.001 | 0.001 |
| Scholarly communication | 0.001 | 0.001 |
| Open science | 0.001 | 0.001 |
| Research integrity | 0.001 | 0.001 |
| Insufficient payload (model declined to judge) | 0.002 | 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".