Impact of Systemic Sclerosis–Associated Interstitial Lung Disease, With and Without Pulmonary Hypertension, on Survival: A Systematic Review
Bibliographic record
Abstract
Many people with systemic sclerosis-associated interstitial lung disease (SSc-ILD) deal with pulmonary hypertension (PH), which seriously reduces their chances of survival. The study aims to evaluate and compare survival outcomes and causes of mortality in patients with SSc-ILD with and without coexisting PH and to assess the implications for clinical management. A comparative review of observational studies and cohort data was conducted to analyze mortality patterns, underlying mechanisms, and treatment responses in SSc-ILD patients stratified by PH status. Based on the Newcastle-Ottawa Scale (NOS) assessment, the overall methodological quality of the included studies was high. Specifically, 16 studies were classified as high quality, with NOS scores ranging from seven to eight, reflecting strong design and execution across the selection, comparability, and outcome domains. Five studies were rated as moderate quality, each scoring six, often due to selection or comparability component limitations. PH commonly affects a significant proportion of patients with SSc-ILD, particularly older adults, individuals of African American ethnicity, and those with reduced diffusing capacity of the lungs. Overall survival tends to be lower in patients with the pulmonary arterial hypertension (PAH) subtype compared to others. A decline in forced vital capacity (FVC) of 10% or more within the first year is linked with higher mortality risk, especially when combined with a marked drop in lung diffusing capacity. Among the various subgroups, patients with both ILD and PH show the poorest five-year survival rates. Important predictors of mortality include the presence of ILD, reduced lung function, and decreased cardiac output. The study concluded that the presence of pulmonary hypertension significantly worsens survival in SSc-ILD patients, primarily through cardiopulmonary failure. These findings highlight the urgent need for early detection, risk stratification, and tailored therapeutic strategies to improve prognosis in this vulnerable population.
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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 | 0.007 | 0.033 |
| Meta-epidemiology (narrow) | 0.001 | 0.001 |
| Meta-epidemiology (broad) | 0.009 | 0.010 |
| Bibliometrics | 0.010 | 0.011 |
| Science and technology studies | 0.001 | 0.001 |
| Scholarly communication | 0.003 | 0.002 |
| Open science | 0.002 | 0.002 |
| Research integrity | 0.002 | 0.001 |
| Insufficient payload (model declined to judge) | 0.004 | 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".