Rare interstitial lung diseases: a narrative review
Bibliographic record
Abstract
Background and Objective: Interstitial lung diseases (ILDs) encompass over 200 entities. Among them, fibrosing lung diseases, have recently generated special interest due to the emerging therapies for their management. However, it is important to deepen our knowledge of other less prevalent ILD, since many of them are associated with a poor prognosis. This narrative review aims to provide a practical and up-to-date description of some poorly recognized ILD. It covers rare idiopathic interstitial pneumonias and their histologic patterns, genetic disorders with interstitial lung involvement (Hermansky-Pudlak syndrome), and ILD associated with benign proliferation of pulmonary lymphoid tissue, namely follicular bronchiolitis and granulomatous-lymphocytic interstitial lung disease. Methods: Electronic searches of PubMed and Google Scholar using specific keywords were conducted. Articles underwent screening for relevance, covering review articles, meta-analyses, systematic reviews, case series, prospective studies, society guidelines, editorials in peer-reviewed journals; scientific books on the subject. The data included was limited to English and Spanish publications. Key Content and Findings: Despite the low prevalence of these diseases, the increased recognition of radiological patterns, pathological features, and diagnostic procedures, have permitted their better characterization. This review highlights epidemiology, clinical presentation, diagnosis, natural history, and treatment. Conclusions: Lesser-studied ILD represent a diagnostic and therapeutic challenge and can be frequently misdiagnosed. Also, due to the lack of randomized controlled trials, there are no well-established therapeutic options. Further studies or registries are needed to improve accurate diagnosis and management.
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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 | 0.001 |
| Meta-epidemiology (narrow) | 0.001 | 0.001 |
| Meta-epidemiology (broad) | 0.004 | 0.006 |
| Bibliometrics | 0.001 | 0.001 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.000 | 0.000 |
| Open science | 0.001 | 0.000 |
| Research integrity | 0.000 | 0.001 |
| 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 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".