# Post-Infectious Bronchiolitis Obliterans as a Model of Immune-Mediated Airway Fibrosis: A Pediatric Case Report

**Authors:** Rosamaria Terracciano, Martina Mazzoni, Alessandro Rossi, Fabio Antonelli, Pierluigi Vuilleumier, Daniela Melis, Annalisa Allegorico

PMC · DOI: 10.3390/ijms27041804 · International Journal of Molecular Sciences · 2026-02-13

## TL;DR

A child with severe lung disease after a viral infection showed improvement with an individualized immune treatment, suggesting a new approach to managing this rare condition.

## Contribution

This case highlights PIBO as an immune-mediated fibrotic disorder and suggests potential for precision medicine strategies.

## Key findings

- The patient showed clinical improvement with an immunomodulatory treatment strategy.
- Radiologic findings indicated partial improvement in airway fibrosis.
- The case suggests better outcomes may be possible with tailored immune therapies.

## Abstract

Post-infectious bronchiolitis obliterans (PIBO) is a rare but severe chronic lung disease of childhood, characterized by irreversible small-airway obstruction following severe lower respiratory tract infections early in life. The disease course is often progressive and associated with long-term respiratory morbidity, while effective disease-modifying therapies remain limited. We report the case of a young child who developed severe PIBO following adenovirus pneumonia complicated by prolonged respiratory failure and multisystem involvement. Diagnosis was based on persistent respiratory symptoms, characteristic radiologic findings, and poor response to conventional anti-inflammatory treatment. Given the severity of the clinical course and steroid-refractory disease, an individualized immunomodulatory strategy, including hydroxychloroquine, was initiated within a multidisciplinary framework. During follow-up, the patient showed progressive clinical improvement, with gradual weaning from continuous oxygen supplementation, fewer respiratory exacerbations, simplification of systemic therapies, and radiologic findings consistent with partial improvement. Although causal conclusions regarding treatment efficacy cannot be drawn from a single case, the overall disease trajectory appeared more favorable than typically reported in PIBO cohorts. This case supports the emerging view of PIBO as an immune-mediated airway fibrotic disorder and underscores the importance of integrating detailed clinical phenotyping with evolving molecular insights to inform future precision medicine in pediatric post-infectious airway disease.

## Linked entities

- **Chemicals:** hydroxychloroquine (PubChem CID 3652)
- **Diseases:** bronchiolitis obliterans (MONDO:0015265), respiratory failure (MONDO:0021113)

## Full-text entities

- **Genes:** CHI3L1 (chitinase 3 like 1) [NCBI Gene 1116] {aka ASRT7, CGP-39, GP-39, GP39, HC-gp39, HCGP-3P}, TADA2A (transcriptional adaptor 2A) [NCBI Gene 6871] {aka ADA2, ADA2A, KL04P, TADA2L, hADA2}, IL6 (interleukin 6) [NCBI Gene 3569] {aka BSF-2, BSF2, CDF, HGF, HSF, IFN-beta-2}, IL17A (interleukin 17A) [NCBI Gene 3605] {aka CTLA-8, CTLA8, IL-17, IL-17A, IL17, ILA17}, DSG2 (desmoglein 2) [NCBI Gene 1829] {aka CDHF5, HDGC}, TGFB1 (transforming growth factor beta 1) [NCBI Gene 7040] {aka CAEND1, CED, DPD1, IBDIMDE, LAP, TGF-beta1}, CASP1 (caspase 1) [NCBI Gene 834] {aka ICE, IL1BC, P45}, CD8A (CD8 subunit alpha) [NCBI Gene 925] {aka CD8, CD8alpha, IMD116, Leu2, p32}, CAMP (cathelicidin antimicrobial peptide) [NCBI Gene 820] {aka CAP-18, CAP18, CRAMP, FALL-39, FALL39, HSD26}, CD46 (CD46 molecule) [NCBI Gene 4179] {aka AHUS2, MCP, MIC10, TLX, TRA2.10}, MUC1 (mucin 1, cell surface associated) [NCBI Gene 4582] {aka ADMCKD, ADMCKD1, ADTKD2, CA 15-3, CD227, Ca15-3}, CD4 (CD4 molecule) [NCBI Gene 920] {aka CD4mut, IMD79, Leu-3, OKT4D, T4}, IFNG (interferon gamma) [NCBI Gene 3458] {aka IFG, IFI, IMD69}, MYBPC3 (myosin binding protein C3) [NCBI Gene 4607] {aka CMD1MM, CMH4, FHC, LVNC10, MYBP-C, cMyBP-C}, IL1B (interleukin 1 beta) [NCBI Gene 3553] {aka IL-1, IL1-BETA, IL1F2, IL1beta}, KRT8 (keratin 8) [NCBI Gene 3856] {aka CARD2, CK-8, CK8, CYK8, K2C8, K8}, CXADR (CXADR cell adhesion molecule) [NCBI Gene 1525] {aka CAR, CAR4/6, HCAR}, IL27 (interleukin 27) [NCBI Gene 246778] {aka IL-27, IL-27A, IL27A, IL27p28, IL30, p28}, CXCL8 (C-X-C motif chemokine ligand 8) [NCBI Gene 3576] {aka GCP-1, GCP1, IL8, LECT, LUCT, LYNAP}, IL18 (interleukin 18) [NCBI Gene 3606] {aka IGIF, IL-18, IL-1g, IL1F4}, POSTN (periostin) [NCBI Gene 10631] {aka OSF-2, OSF2, PDLPOSTN, PN}
- **Diseases:** Pulmonary arterial hypertension (MESH:D000081029), apnea (MESH:D001049), bronchiectasis (MESH:D001987), viral (MESH:D014777), hypertension (MESH:D006973), thrombotic occlusion (MESH:D013927), co-infections (MESH:D060085), Adenovirus infection (MESH:D000257), tachypnea (MESH:D059246), PIBO (MESH:D001989), toxicity (MESH:D064420), cough (MESH:D003371), hemiparesis (MESH:D010291), ischemic stroke (MESH:D002544), immune system disorders (MESH:D007154), ischemic lesions (MESH:D017202), post (MESH:D000094025), SARS-CoV-2 infection (MESH:D000086382), adenoviral infection (MESH:D007239), idiopathic pulmonary fibrosis (MESH:D054990), interstitial lung disease (MESH:D017563), autosomal dominant hypertrophic cardiomyopathy (MESH:D002312), thromboembolic (MESH:D013923), ischemic-infarct lesions (MESH:D007238), wheezing (MESH:D012135), epileptiform abnormalities (MESH:D014277), bacterial infections (MESH:D001424), measles (MESH:D008457), Infectious Triggers (MESH:D003141), Pulmonary hypertension (MESH:D006976), chronic (MESH:D002908), tissue injury (MESH:D017695), Bronchiolitis (MESH:D001988), hypopnea (MESH:D012891), cardiac abnormalities (MESH:D018376), immune dysregulation (OMIM:614878), respiratory disease (MESH:D012140), small (MESH:D018288), critical illness (MESH:D016638), pulmonary involvement (MESH:C566343), Airway Fibrosis (MESH:D005355), airway inflammation (MESH:D007249), respiratory tract infection (MESH:D012141), injury (MESH:D014947), interstitial and small-airway disorders (MESH:D056151), lung injury (MESH:D055370), varicella (MESH:D002644), ischemic (MESH:D002545), fibrotic lung disease (MESH:D008171), dyspnea (MESH:D004417), adenovirus pneumonia (MESH:D011014), hypercapnia (MESH:D006935), thrombophilia (MESH:D019851), inherited thrombophilic disorder (MESH:C540694), inflammatory lung disorders (MESH:D016726), airflow limitation (MESH:D029424), functional (MESH:D003291), bronchiolitis obliterans syndrome (MESH:D000092122), hypoxic (MESH:D002534), common cold symptoms (MESH:D003139)
- **Chemicals:** sodium (MESH:D012964), Hydroxychloroquine (MESH:D006886), montelukast (MESH:C093875), fluticasone (MESH:D000068298), macrolides (MESH:D018942), salmeterol (MESH:D000068299), furosemide (MESH:D005665), steroid (MESH:D013256), levetiracetam (MESH:D000077287), reactive oxygen species (MESH:D017382), polysialic acid (MESH:C021319), amlodipine (MESH:D017311), oxygen (MESH:D010100), esomeprazole (MESH:D064098), Azithromycin (MESH:D017963), micafungin (MESH:D000077551), phenobarbital (MESH:D010634), fluticasone propionate/salmeterol (MESH:D000068297), acetylsalicylic acid (MESH:D001241)
- **Species:** Respiratory syncytial virus (no rank) [taxon 12814], Homo sapiens (human, species) [taxon 9606], Candida [taxon 1535326], Adenoviridae (family) [taxon 10508], human gammaherpesvirus 4 (Epstein Barr virus, no rank) [taxon 10376], Rubella virus (no rank) [taxon 11041], Human respirovirus 3 (no rank) [taxon 11216], Enterovirus (genus) [taxon 12059], Mycoplasmoides pneumoniae (Filterable agent of primary atypical pneumonia, species) [taxon 2104], Human alphaherpesvirus 3 (Varicella-zoster virus, no rank) [taxon 10335]
- **Mutations:** p. Trp1086Ter, c.3258G>A

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## References

18 references — full list in the complete paper: https://tomesphere.com/paper/PMC12940845/full.md

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Source: https://tomesphere.com/paper/PMC12940845