Idiopathic Pleuroparenchymal Fibroelastosis
Bibliographic record
Abstract
Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare form of idiopathic interstitial pneumonia. The disease is characterized by fibrosis of the pleura and subpleural lung parenchyma predominantly affecting the upper lobes. Various triggers have been proposed as inciting factors in the development of the disease. Diagnosis is made clinically in conjunction with radiographic findings and histopathology when available. There are no known effective treatment options and several cases of lung transplantation have been reported. We report a case of an 86-year-old female who presented to the emergency department with worsening dyspnea and hypoxia. She had a history of unexplained pneumomediastinum and a 20 - 25 pounds unintentional weight loss over 10 months. Computed tomography (CT) of the chest without contrast revealed radiographic evidence of IPPFE. Despite symptomatic management with antibiotics, diuretics, and steroids, her condition continued to deteriorate. Unfortunately, our patient was not a candidate for a lung transplant. She was transitioned to hospice care and succumbed to her disease. IPPFE is a rare disease with an unknown prevalence. It has a median survival rate of 2 years. Usually, there is an overlap with interstitial lung diseases, making it challenging to diagnose. There are only a few cases reported in the literature, and there are currently no guidelines available on the appropriate management of this debilitating disease. We recommend more cases be reported, and further research is done to establish better criteria for diagnosis and management.
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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 | 0.002 |
| Meta-epidemiology (narrow) | 0.001 | 0.000 |
| Meta-epidemiology (broad) | 0.001 | 0.001 |
| Bibliometrics | 0.001 | 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) | 0.003 | 0.001 |
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".