Burden of fatigue in cryopyrin-associated periodic syndromes
Bibliographic record
Abstract
Objectives Fatigue in cryopyrin-associated periodic syndromes (CAPS) often persists despite controlling inflammation. This study explores fatigue burden, its link to disease activity, and factors tied to chronic fatigue in autoinflammatory conditions. Methods A single-centre study (2007-2024) of children and adults with CAPS analysed demographics, clinical data, inflammation, treatment, disease activity, and fatigue scores. Relationships between these factors and predictors of chronic fatigue were assessed. Results A total of 108 patients with CAPS were included; 53 were female (49%). Median age at diagnosis was 11 years (range, 0-73); median follow-up duration 7 years (range, 1-18). Muckle-Wells syndrome was the most common phenotype (86%). At the time of diagnosis, hearing loss was present in 30%, aseptic meningitis in 6%. At enrolment, fatigue was the most common symptom, affecting 100 patients (93%), with a median fatigue score of 7 (0-10, visual analogue scale); high disease activity was present in 67%. At the last visit, fatigue remained prevalent, reported in 83 (77%), with a median score of 3 (range, 0-10). Importantly, only 4% of patients had high disease activity, whereas 83% had inactive disease. Factors influencing chronic fatigue in CAPS included age at diagnosis ( P < .001, Cohen's d = −0.776, β = −2.15), NLRP3 mutation status ( P = .009, Cohen's d = −0.516, β = −1.48), disease activity ( P < .001), familial CAPS (62% vs 38%, P = .132, Cohen's d = −0.298, β = −0.87), and the COVID-19 pandemic ( P < .001). Conclusions Fatigue persists in autoinflammatory diseases despite effective disease control. Further research is needed to identify mechanisms and interventions.
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How this classification was reachedexpand
Full frame distilled prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. Learned from the 10,348 direct Codex labels and 10,348 direct Gemma labels. Candidate is the union of thresholded teacher heads; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels or direct frontier model labels.
Codex and Gemma teacher scores by category
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.000 | 0.000 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.000 |
| Bibliometrics | 0.000 | 0.000 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.000 | 0.000 |
| Open science | 0.001 | 0.000 |
| Research integrity | 0.000 | 0.000 |
| Insufficient payload (model declined to judge) | 0.000 | 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 teacher head, 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".