A case of common variable immune deficiency with lung disease–not just bronchiectasis
Bibliographic record
Abstract
Introduction: Common Variable Immune Deficiency (CVID) is the most prevalent form of severe antibody deficiency in children and adults. Most patients suffer recurrent, mainly sinopulmonary, infections. Despite adequate IVIG replacement therapy, chronic lung disease continues to be a main cause of morbidity and mortality. The term granulomatous-lymphocytic interstitial lung disease (GLILD) is frequently used to describe interstitial lung disease associated with immune dysregulation in primary antibody deficiency, such as CVID. Aim: To describe the case of a 10-year-old male with CVID who developed GLILD and his response to treatment with Rituximab. Discussion: Our patient is a young male with CVID and no genetic diagnosis, whose lung functions and general condition continued to deteriorate despite adequate intravenous immunoglobulin replacement therapy and mycophenolate mofetil treatment. After the diagnosis of GLILD, we initiated treatment with a 4-dose weekly course of Rituximab with prompt resolution of his interstitial disease. Although GLILD is a well described condition that accompanies CVID as a manifestation of immune dysregulation, it is still under recognized, especially in the pediatric population. Among experts, there is little uniformity when it comes to diagnostic and treatment approaches. Recent studies showed improved outcomes when using combination therapy with Rituximab, such as in our patient. Statement of Novelty: We shed light on GLILD, an important condition that accompanies CVID, and demonstrate an excellent response to the steroid sparing agent Rituximab. This is a crucial aspect when considering therapeutic choices for the pediatric population.
Fetched live from OpenAlex and de-inverted. Abstracts are not stored in this database: the inverted indexes are 8.6 GB of the frame’s 9.3 GB of text, and the host has 13 GB free.
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) | 0.001 | 0.001 |
| Meta-epidemiology (broad) | 0.001 | 0.001 |
| Bibliometrics | 0.002 | 0.001 |
| Science and technology studies | 0.002 | 0.002 |
| Scholarly communication | 0.001 | 0.002 |
| Open science | 0.001 | 0.002 |
| Research integrity | 0.005 | 0.004 |
| 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".