Clinical Manifestations and Treatment in Patients With Relapsing Polychondritis: A Multicenter Observational Cohort Study
Bibliographic record
Abstract
OBJECTIVE: Relapsing polychondritis (RP) is a rare, heterogeneous, multisystem disease lacking standard treatment guidelines. This study describes clinical manifestations in association with approaches to treatment. METHODS: Adults with physician-diagnosed RP were recruited into a multicenter observational cohort study. Clinical manifestations, organ damage, and medication history were recorded at the baseline study visit. Treatments received for RP at any time point before the initial visit were categorized into three groups: group 1 was treated with glucocorticoids (GCs) or no drugs, group 2 was treated with nonbiologic immunosuppressive (IS) drugs excluding JAK inhibitors (JAKis) with or without GCs, and group 3 was treated with JAKis or biologic IS drugs with or without nonbiologic IS drugs or GCs. RESULTS: Included in the study were 195 patients with RP who were predominantly female (167, 86%) and White (174, 89%), with a mean age of 49 ± 13years. All patients had ear, nose, or airway involvement, and 163 (83%) had musculoskeletal manifestations of RP. All patients had at least three clinical manifestations with median of 11 (range 3-19). GC treatment was given to 186 (95%) patients. Organ damage was seen in 80 (41%) patients. Treatment groups 1, 2, and 3 had 37 (19%), 55 (28%), and 103 (53%) patients, respectively. Patients in group 3 were more likely to have organ damage, arthritis, and subglottic stenosis. CONCLUSION: Patients with RP have a high burden of clinical manifestations with resultant damage. Physicians typically treat RP with GCs, and the use of other immunosuppressive medications is variable. Absence of a consensus approach to treatment underscores the need for clinical trials and treatment guidelines for RP.
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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 | 0.001 |
| Meta-epidemiology (broad) | 0.001 | 0.001 |
| Bibliometrics | 0.001 | 0.001 |
| Science and technology studies | 0.001 | 0.000 |
| Scholarly communication | 0.001 | 0.001 |
| Open science | 0.001 | 0.001 |
| Research integrity | 0.001 | 0.001 |
| Insufficient payload (model declined to judge) | 0.001 | 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".