Raras
Buscar doenças, sintomas, genes...
Bronquiolite obliterante
ORPHA:1303CID-10 · J44.8CID-11 · CA26.0DOENÇA RARA
lungInício todas idadesHerança Unknown
Sinônimos clínicos: Bronquiolite obliterativa · Bronquiolite constritiva

Uma doença pulmonar que está principalmente ligada ao mau funcionamento contínuo de um pulmão após um transplante. Ela se caracteriza por inflamação e cicatrização (fibrose) das paredes dos bronquíolos (pequenos tubos de ar dentro dos pulmões). Isso faz com que os bronquíolos se estreitem, resultando em uma obstrução progressiva e irreversível da passagem de ar.

Mantido por Agente Raras·Colaborar como especialista →

Introdução

O que você precisa saber de cara

📋
Informacoes curadas por IA — podem conter imprecisoes

Uma doença pulmonar que está principalmente ligada ao mau funcionamento contínuo de um pulmão após um transplante. Ela se caracteriza por inflamação e cicatrização (fibrose) das paredes dos bronquíolos (pequenos tubos de ar dentro dos pulmões). Isso faz com que os bronquíolos se estreitem, resultando em uma obstrução progressiva e irreversível da passagem de ar.

Pesquisas ativas
5 ensaios
761 total registrados no ClinicalTrials.gov
Publicações científicas
4.340 artigos
Último publicado: 2026 Mar

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
Unknown
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.0
Worldwide
Início
All ages
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: J44.8
Você se identifica com essa condição?
O Raras está aqui pra te apoiar — com ou sem diagnóstico

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Entender a doença

Do básico ao detalhe, leia no seu ritmo

Preparando trilha educativa...

Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Partes do corpo afetadas

🫁
Pulmão
9 sintomas
🛡️
Imunológico
1 sintomas

+ 6 sintomas em outras categorias

Características mais comuns

90%prev.
Obstrução das vias aéreas
Muito frequente (99-80%)
90%prev.
Razão FEV1/FVC reduzida
Muito frequente (99-80%)
90%prev.
Dispneia
Muito frequente (99-80%)
90%prev.
Capacidade vital forçada reduzida
Muito frequente (99-80%)
90%prev.
Bronquiolite obliterante
Muito frequente (99-80%)
90%prev.
Tosse
Muito frequente (99-80%)
16sintomas
Muito frequente (9)
Frequente (4)
Ocasional (2)
Muito raro (1)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 16 características clínicas mais associadas, ordenadas por frequência.

Obstrução das vias aéreasAirway obstruction
Muito frequente (99-80%)90%
Razão FEV1/FVC reduzidaReduced FEV1/FVC ratio
Muito frequente (99-80%)90%
DispneiaDyspnea
Muito frequente (99-80%)90%
Capacidade vital forçada reduzidaReduced forced vital capacity
Muito frequente (99-80%)90%
Bronquiolite obliteranteBronchiolitis obliterans
Muito frequente (99-80%)90%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico4.340PubMed
Últimos 10 anos200publicações
Pico2025144 papers
Linha do tempo
2026Hoje · 2026🧪 1994Primeiro ensaio clínico📈 2025Ano de pico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Genética e causas

O que está alterado no DNA e como passa nas famílias

🧬

Nenhum gene associado encontrado

Os dados genéticos desta condição ainda estão sendo catalogados.

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

Carregando...

Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
3Fase 32
2Fase 23
·Pré-clínico2
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 7 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — Bronquiolite obliterante

🗺️

Selecione um estado ou use sua localização para ver resultados.

Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.

Pesquisa ativa

Ensaios clínicos abertos e novidades científicas recentes

🟢 Recrutando agora

2 pesquisas recrutando participantes. Converse com seu médico sobre a possibilidade de participar.

Outros ensaios clínicos

761 ensaios clínicos encontrados, 5 ativos.

Distribuição por fase
Ver todos no ClinicalTrials.gov
🧪 Está conduzindo uma pesquisa?
Divulgue para pacientes e familiares que acompanham esta doença.
Divulgar pesquisa →

Publicações mais relevantes

Timeline de publicações
1.450 papers (10 anos)

Mostrando amostra de 200 publicações de um total de 1.450

#1

Ruxolitinib and Tocilizumab for Child Bronchiolitis Obliterans After Toxic Epidermal Necrolysis.

Pediatrics2026 Mar 12

Toxic epidermal necrolysis (TEN) is a life-threatening mucocutaneous disorder with rare but potentially fatal respiratory complications. We present an exceptional documented case of TEN with severe bronchial obstruction successfully treated with tocilizumab and ruxolitinib in an 11-year-old patient. Despite early methylprednisolone pulse therapy, a repeat bronchoscopy showed progressive obstruction of proximal bronchial lumen by synechiae and mucosal membranes. Following multidisciplinary discussion, treatment was intensified with tocilizumab and ruxolitinib, resulting in marked respiratory improvement and decreased proinflammatory cytokines in bronchoalveolar lavage. No adverse effects were observed during 8 months of follow-up. Recent research supports Janus kinase inhibitors as targeted therapy in TEN.

#2

BET inhibition blunts antibody production and macrophage-mediated fibrosis to restore lung function in murine cGVHD.

Blood2026 Mar 02

Chronic graft-versus-host disease (cGVHD) significantly contributes to late mortality after allogeneic stem cell transplantation, with bronchiolitis obliterans syndrome (BOS) being a particularly lethal and treatment-resistant complication despite available therapies. Bromodomain and Extra terminal (BET) proteins are epigenetic readers driving inflammatory transcriptional programs across multiple cell types. We hypothesized that BET inhibition would suppress inflammatory T and B cells while also decreasing macrophage polarization to a profibrotic phenotype, alleviating disease. In an established BOS cGVHD model, BET inhibition reduced germinal center formation and response through a reduction of the CXCL13:CXCR5 axis, inflammatory Tfh/GC B cells in the spleen along with a reduction in plasma cell infiltration within the lung. Mice with cGVHD had elevated pathogenic IgG1 and IgM, both in circulation and deposited on lung tissue, which was attenuated under BET inhibition. Single-cell RNA sequencing analysis revealed distinct cell states in the BOS lung vs. control. In cGVHD mice, GSEA analysis revealed upregulation of profibrotic Arginase1 and Tgfb1 expression in alveolar macrophages (AM) and interstitial macrophages (IM), which was significantly reduced with BET inhibition. Furthermore, BET inhibition targeted lung-infiltrating M2 macrophages, through selective depletion of CD206+FcgR+ IMs and AMs, ultimately resulting in reduced collagen deposition and improved lung function. Our findings reveal a previously unrecognized mechanistic axis of BET regulation during cGVHD fibrosis and highlight BET inhibition as a promising therapeutic strategy.

#3

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

International journal of molecular sciences2026 Feb 13

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.

#4

Targeting integrin beta 4 in diacetyl-induced anoikis of the airway epithelium.

Cell death discovery2026 Feb 26

Diacetyl (DA) is a flavoring chemical commonly found in food and beverages. When inhaled at occupationally relevant concentrations, DA can cause bronchiolitis obliterans (BO), yet the mechanisms remain poorly understood. Common to all forms of BO is airway epithelial injury, with failed epithelial cell survival contributing to BO development. The purpose of the current study was to target integrin beta 4 (ITGβ4) - one of the primary integrins that connect airway epithelial cells to the basement membrane - in DA-exposed airway epithelial cells to prevent adhesion-related cell apoptosis ('anoikis'). Sprague-Dawley rats were exposed to 200 parts-per-million DA vapor or filtered air for 6 hours per day for 5 consecutive days, then monitored for 5 weeks post-exposure and assessed for airway remodeling using Trichrome staining and the hydroxyproline assay. ITGβ4 protein expression was assessed via western blot as well as co-immunofluorescent staining using common airway epithelial cell markers. In parallel, primary human airway epithelial cells and human bronchial epithelial cells (16HBE14o-) were grown in vitro, exposed to DA, and treated with the pan-caspase inhibitor Z-VAD-FMK or transfected with ITGB4. End-points included viability staining, extracellular caspase 3/7 activity, and ITGβ4 protein expression. Rats exposed to DA vapors developed significant airway remodeling with increased total lung collagen content and sub-epithelial airway collagen deposition. Airway epithelial ITGβ4 expression remained decreased weeks after DA exposure with expansion of pan-cytokeratin positive epithelial cells, independent of ciliated and club cell markers. In parallel, DA-exposed human airway epithelial cells exposed in vitro developed significant anoikis. Treatment with Z-VAD-FMK reduced anoikis and improved ITGβ4 cytoplasmic surface expression but failed to improve total ITGβ4 protein expression. ITGB4 overexpression failed to suppress ITGβ4 cleavage or prevent anoikis. In summary, DA exposure in both rats and human airway epithelial cells results in caspase-mediated cleavage of ITGβ4. Future studies targeting post-translational modifications of ITGβ4 may prevent airway epithelial cell anoikis and fibrotic remodeling.

#5

Longitudinal Study of Late Acute and Chronic Graft-Versus-Host Disease after Allogeneic Hematopoietic Cell Transplantation: A Long-Term Follow-up Study from the Chronic Graft-Versus-Host Disease Consortium.

Transplantation and cellular therapy2026 Feb

Late acute and chronic graft-versus-host disease (GVHD) contribute to morbidity and death after allogeneic hematopoietic cell transplantation (HCT). A prior national Chronic GVHD Consortium longitudinal study enrolled patients pre- or early post-HCT and identified the incidence of late acute GVHD, chronic GVHD, and chronic GVHD subtypes of bronchiolitis obliterans syndrome (BOS) and cutaneous sclerosis. We now report 10-yr follow-up from that study in a long-term follow-up analysis (N = 911 subjects). Late acute GVHD occurred in 11% at a median of 5.5 mo. Chronic GVHD in total occurred in 54% with median onset of 7.4 mo. BOS (4% of subjects, median onset 12.6 mo) and cutaneous sclerosis (10% of subjects, median onset of 17.2 mo) were less frequent and had later-onset. The long-term analysis demonstrated additional GVHD events occurring beyond 1 to 2 yr post-HCT. Inter-conversion between GVHD types was common, and notably included new development of BOS and cutaneous sclerosis after initial chronic GVHD presentation without these manifestations. GVHD-free, relapse-free survival (GVHD-DFS, survival without relapse or development of late acute or chronic GVHD) was 22% at 2 yr, 18% at 5 yr, and 15% at 10 yr post-HCT. Non-relapse mortality continued to increase beyond 2 yr, with 10-yr estimates up to 35% (late acute), 31% (chronic GVHD), 62% (BOS), and 36% (cutaneous sclerosis). Durable complete discontinuation of immune suppression was uncommon for all GVHD types. These data suggest that extended surveillance for late acute and chronic GVHD is needed post-HCT due to late occurrences, and that associated mortality is high through 10 yr post-GVHD onset.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC1.835 artigos no totalmostrando 197

2026

Efficacy and safety of a new cladribine-based conditioning regimen for allogeneic hematopoietic stem cell transplantation in children with relapsed or refractory acute myeloid leukemia.

Frontiers in medicine
2026

Post-transplant Cyclophosphamide Reduces Bronchiolitis Obliterans Syndrome Risk Through Chronic Graft-versus-Host Disease Prevention: A Multicenter Cohort Study.

Chest
2026

Mycoplasma pneumoniae-induced Postinfectious Bronchiolitis Obliterans in a Child: A Case Report From Taiwan.

The Pediatric infectious disease journal
2026

Swyer-James-MacLeod Syndrome: A Rare Cause of Unilateral Hyperlucent Lung in a Two-Year-Old Male Child.

Cureus
2026

A Tale of Two Lungs: 99mTc-MAA Pulmonary Scintigraphy Reveals Unilateral Chronic Lung Allograft Dysfunction (CLAD) After Bilateral Living-Donor LobarLung Transplants (LDLLT).

Clinical nuclear medicine
2026

Knowledge structure and academic influence in lung transplantation: a systematically bibliometric analysis of highly cited documents and multi-perspective extensive review.

Journal of thoracic disease
2026

Ruxolitinib and Tocilizumab for Child Bronchiolitis Obliterans After Toxic Epidermal Necrolysis.

Pediatrics
2026

BET inhibition blunts antibody production and macrophage-mediated fibrosis to restore lung function in murine cGVHD.

Blood
2026

Bronchiolitis Obliterans Syndrome Following High-Dose Chemotherapy and Autologous Hematopoietic Stem Cell Transplantation in a Pediatric Patient With Neuroblastoma.

Cureus
2026

Management of CMV Pneumonia, DAH, and BOS Following HSCT in a Child with β-Thalassemia: A Case Report.

Journal of inflammation research
2026

Effects of Nutritional Support Combined With Respiratory Training on Pulmonary Function in Patients With Obliterative Bronchiolitis: A Propensity Score Matching Study.

British journal of hospital medicine (London, England : 2005)
2026

Lung retransplantation after initial single lung transplantation.

JHLT open
2026

Updates, Management, and Future of Diagnosing and Managing Chronic Lung Allograft Dysfunction.

Journal of clinical medicine
2026

Interleukin-2 Receptor Antagonist Induction Therapy in Lung Transplantation-A Meta-Analysis of Reconstructed Time-to-Event Data.

Journal of clinical medicine
2026

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

International journal of molecular sciences
2026

Novel translational pulmonary MRI in pediatrics: A Review of the last 10 years.

The European respiratory journal
2026

Targeting integrin beta 4 in diacetyl-induced anoikis of the airway epithelium.

Cell death discovery
2026

Thoracic Surgery - notes for its history in Brazil (Part II): Lung transplantation.

Portuguese journal of cardiac thoracic and vascular surgery
2026

Longitudinal Study of Late Acute and Chronic Graft-Versus-Host Disease after Allogeneic Hematopoietic Cell Transplantation: A Long-Term Follow-up Study from the Chronic Graft-Versus-Host Disease Consortium.

Transplantation and cellular therapy
2026

Machine learning and bioinformatics-based identification of mitophagy-related diagnostic biomarkers in bronchiolitis obliterans.

Medicine
2026

Immune gene correlation networks differentiate both chronic lung allograft dysfunction and survival.

American journal of transplantation : official journal of the American Society of Transplantation and the American Society of Transplant Surgeons
2026

Long-term Outcome of Lung Retransplantation for Chronic Lung Allograft Dysfunction After En-bloc Heart and Lung Transplantation.

European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery
2026

Successful Bilateral-Lung Transplantation With Long Survival Time for Multicentric Castleman Disease With Fatal Pulmonary Involvement.

Transplantation proceedings
2026

Bronchiolitis obliterans complicating follicular lymphoma: a case series and clinical insights.

International journal of hematology
2026

Survival improvement with sirolimus plus tacrolimus immunosuppression for treatment of bronchiolitis obliterans syndrome after lung transplantation.

JHLT open
2026

Association between adjunctive corticosteroid therapy and clinical outcomes in children with severe viral community-acquired pneumonia: A retrospective cohort study.

Medicine
2026

Steroid-Refractory Cryptogenic Organising Pneumonia (COP) in a Patient With Mannose-Binding Lectin (MBL) Deficiency.

Respirology case reports
2026

Characteristics and significance of bronchiolitis obliterans syndrome after hematopoietic stem cell transplantation at bronchoscopy.

Respiratory research
2026

Prediction of Long-Term Mortality in Acute Hypercapnic Respiratory Failure with Use of Low-Flow Veno-Venous Extracorporeal CO2 Removal: A Retrospective Single-Center Study.

Blood purification
2025

Determinants of Asthma Control in Jordanian Children: The Role of Comorbidities and FeNO Levels.

Children (Basel, Switzerland)
2026

Passenger Lymphocyte Syndrome in Pediatric Lung Transplantation: A Recipient With Previous Hematopoietic Stem Cell Transplantation.

Pediatric transplantation
2026

Bronchiolitis Obliterans as an Initial Presentation in an Adolescent With HIV: A Case Report.

Pediatric pulmonology
2025

Immunoglobulin levels of children with bronchiolitis obliterans syndrome after hematopoietic cell transplantation.

Frontiers in medicine
2026

Diacetyl-induced occupational lung disease: insights from two cases.

Clinical toxicology (Philadelphia, Pa.)
2026

Autoantibody repertoire analysis in paraneoplastic pemphigus reveals novel targets linked to mucocutaneous blistering and bronchiolitis obliterans.

Communications medicine
2026

Antineutrophil Cytoplasmic Antibodies Contribute to Airway Inflammation via Induction of Neutrophil Extracellular Traps in Children With Bronchiolitis Obliterans.

The clinical respiratory journal
2026

From the archives of MD Anderson Cancer Center: Paraneoplastic autoimmune multiorgan syndrome (PAMS) associated with stroma-rich Castleman disease.

Annals of diagnostic pathology
2026

Immunodeficiency-Associated Childhood Interstitial Lung Diseases: Data from the Türkiye chILD Registry.

Balkan medical journal
2026

Outcomes of Lung Transplantation for Bronchiolitis Obliterans after Hematopoietic Stem Cell Transplantation Compared with Those for Idiopathic Pulmonary Fibrosis.

Yonsei medical journal
2025

Spatial Transcriptomics Analysis of Macrophage-to-Myofibroblast Transition in Bronchiolitis Obliterans Following Hematopoietic Stem Cell Transplantation.

Transplantation and cellular therapy
2026

Quantitative CT and Artificial Intelligence in Chronic Lung Disease.

Journal of thoracic imaging
2025

Classical complement activation in light and heavy chain deposition disease with acquired cutis laxa and bronchiolitis obliterans: a case report of monoclonal gammopathy of clinical significance.

Frontiers in immunology
2026

Cystatin C prevents tissue injury after lung transplantation.

Life science alliance
2025

Diacetyl-induced bronchiolitis obliterans involves ubiquitin C upregulation and fibrosis-related gene activation in rats.

Frontiers in pharmacology
2025

[Therapy of follicular lymphoma associated bronchiolitis obliterans with lung transplantation: 2 cases and literature review].

Zhonghua jie he he hu xi za zhi = Zhonghua jiehe he huxi zazhi = Chinese journal of tuberculosis and respiratory diseases
2026

Fungal colonization and CLAD in lung transplant recipients: Implications for antifungal prophylaxis and graft survival.

JHLT open
2025

Cardiorespiratory fitness in patients with post-infectious bronchiolitis obliterans: a cross-sectional study.

ERJ open research
2025

Psittacosis chlamydia pneumonia complicated with organizing pneumonia: a case report and literature review.

Frontiers in medicine
2026

To Respond or Not to Respond: That's the Question in Bronchiolitis Obliterans Syndrome Treatment.

Transplantation
2025

Extracorporeal Photopheresis for Chronic Graft-Versus-Host Disease According to 2014 NIH Consensus Criteria.

Journal of clinical apheresis
2025

Ruxolitinib as second-line therapy for bronchiolitis obliterans syndrome after hematopoietic cell transplantation: a retrospective multicenter study.

Annals of hematology
2025

HMGA1 drives EMT in obliterative bronchiolitis through epigenetic regulation of chromatin accessibility in pulmonary epithelial cells.

Clinical epigenetics
2026

Current therapeutics for sclerotic and fibrotic manifestations of chronic graft-versus-host disease.

Bone marrow transplantation
2025

A European multi-center, randomized controlled trial of Pirfenidone in bronchiolitis obliterans syndrome after bilateral lung transplantation.

The European respiratory journal
2025

[A case of unicentric chest wall Castleman disease-associated paraneoplastic autoimmune multiorgan syndrome].

Zhonghua jie he he hu xi za zhi = Zhonghua jiehe he huxi zazhi = Chinese journal of tuberculosis and respiratory diseases
2025

Risk for severe refractory Mycoplasma pneumoniae pneumonia complicated with bronchitis obliterans among children.

Medicine
2025

Regulatory T cell therapy in lung transplantation: bridging the gap from bench to bedside.

Frontiers in immunology
2025

Beyond systemic immunity: the mucosal frontier in bronchiolitis obliterans syndrome pathogenesis.

The European respiratory journal
2025

Pulmonary small lymphocytic lymphoma combined with paraneoplastic pemphigus and bronchiolitis obliterans: a case report.

BMC pulmonary medicine
2025

Mycoplasma Pneumoniae bronchiolitis and hypoxemia: A retrospective cohort study on risk and prognosis.

PloS one
2025

Risk factors for bronchiolitis obliterans in children with community-acquired pneumonia and analysis of CT findings and clinical manifestations of pneumonia after the diagnosis of bronchiolitis obliterans.

Annals of medicine
2026

Development and validation of a deep learning-based algorithm for quantifying bronchiolitis obliterans in paediatric computed tomography.

The British journal of radiology
2025

Retransplant- and Bronchiolitis Obliterans Syndrome-Free Survival among COVID Lung Transplant Recipients: A National Cohort Study.

Annals of the American Thoracic Society
2025

Bronchiolitis Obliterans in Unicentric Castleman Disease Without Paraneoplastic Pemphigus Requiring Bilateral Lung Transplantation.

Cureus
2026

Bronchiectasis as a Distinct Entity in Patients With Bronchiolitis Obliterans Syndrome After Hematopoietic Cell Transplantation.

Transplantation and cellular therapy
2025

Between hospital and home: Respiratory rehabilitation challenges and needs among caregivers of children with post-infectious bronchiolitis obliterans.

Journal of pediatric nursing
2025

Noninfectious Severe Pulmonary Complications in Immunocompromised Critically Ill Patients.

Seminars in respiratory and critical care medicine
2025

Specific risk factors for heart-lung transplantation.

JHLT open
2025

Pharmacological EZH2 inhibition attenuates post-transplant obliterative bronchiolitis by reprogramming macrophage polarization.

Biochemical pharmacology
2026

Serum belumosudil concentrations in a patient receiving a combination of 3 gastric acid-suppressing agents: A case report.

American journal of health-system pharmacy : AJHP : official journal of the American Society of Health-System Pharmacists
2025

Bronchiolitis Obliterans in an Adult Male After Admission With Panton-Valentine Leucocidin Staphylococcal Pneumonia.

Cureus
2026

Clinical Characteristics, Treatment Response, and Outcomes of Bronchiolitis Obliterans Syndrome in Children with Hematologic Malignancies Undergoing Haploidentical Hematopoietic Cell Transplantation with Granulocyte Colony-Stimulating Factor/Antithymocyte Globulin-Based Protocol: A Single-Center Study.

Transplantation and cellular therapy
2025

Risk factors for bronchiolitis obliterans syndrome after hematopoietic stem cell transplantation: a systematic review and meta-analysis.

BMC pulmonary medicine
2026

Characterization of Spirometric Response to Standard-of-care Treatment in Lung Allograft Recipients With Bronchiolitis Obliterans and the Utility of Spirometric Criteria for Rescue Therapy: Implications for the Design of Risk-stratified Clinical Trials.

Transplantation
2026

Respiratory Virus Infections and Pulmonary Impairment After Allogeneic Hematopoietic Cell Transplantation.

The Journal of infectious diseases
2025

Identification of Surrogate Outcome Biomarkers in Lung Allograft Recipients With Bronchiolitis Obliterans: Implications for Randomized Controlled Trials.

Transplantation direct
2025

Pulmonary Graft-Versus-Host Disease After Hematopoietic Cell Transplantation.

Clinical journal of oncology nursing
2025

Development and Validation of a Nomogram for Predicting Bronchiolitis Obliterans in Children with Severe Adenovirus Pneumonia: Identification of Key Risk Factors.

Pediatric health, medicine and therapeutics
2025

Low-dose versus high-dose methylprednisolone for children with severe Mycoplasma pneumoniae pneumonia (MCMP): A randomized controlled trial.

Pediatric investigation
2025

Cryptogenic Organizing Pneumonia.

Seminars in respiratory and critical care medicine
2025

[Consensus on early screening, prevention, diagnosis and treatment of post-infectious bronchiolitis obliterans (2025)].

Zhonghua er ke za zhi = Chinese journal of pediatrics
2025

Clinical Implementation of Inspiratory-Expiratory Chest CT: Defining Quality Criteria for Diagnostic Quality and Detection of Concurrent FEV1 Decline following Lung Transplantation.

Radiology. Cardiothoracic imaging
2025

Taiwan consensus on pulmonary chronic graft-versus-host disease: A joint statement from the Taiwan Society of Blood and Marrow Transplantation (TBMT) and Taiwan Society of Pulmonary and Critical Care Medicine (TSPCCM).

British journal of haematology
2025

[Hyaline vascular Castleman disease with indolent T-lymphoblastic proliferation complicated by paraneoplastic autoimmune multiorgan syndrome: report of a case].

Zhonghua bing li xue za zhi = Chinese journal of pathology
2025

Noninfectious pulmonary complications after hematopoietic cell transplantation: a comprehensive review.

Respiratory medicine
2025

Respiratory viral infections in lung transplantation: Recent advances in epidemiology, clinical impact, and therapeutic approaches.

JHLT open
2025

Increased reflux burden on pre-transplant reflux testing independently predicts significant pulmonary function decline after lung transplantation.

World journal of transplantation
2025

Four decades of heart-lung transplantation: Milestones and outcomes in advanced cardiorespiratory failure.

ESC heart failure
2025

Neurological manifestations of chronic graft versus host disease.

Internal medicine journal
2025

Lung transplantation in a patient with severe bronchiolitis obliterans secondary to Castleman disease.

Respiratory medicine case reports
2025

Bronchiectasis in Bronchiolitis Obliterans Syndrome after Hematopoietic Cell Transplantation.

Transplantation and cellular therapy
2025

Anesthesia management for lung transplant in children with obliterated bronchiolitis after hematopoietic stem cell transplantation: a single-center experience.

Journal of thoracic disease
2025

Inhibition of BRD4 prevents peribronchial fibrosis in mice with cutaneous lewisite exposure.

Frontiers in molecular biosciences
2025

Treatment Options for Bronchiolitis Obliterans Secondary to Hematopoietic Cell Transplantation in Children: A Systematic Review.

Clinical transplantation
2025

Safe and effective antireflux surgery in lung transplant recipients: preliminary results.

Wideochirurgia i inne techniki maloinwazyjne = Videosurgery and other miniinvasive techniques
2025

Machine Learning for 1-Year Graft Failure Prediction in Lung Transplant Recipients: The Korean Organ Transplantation Registry.

Clinical transplantation
2025

[Recommendations on the prevention and management of radiation-induced lung injury from the Association francophone pour les soins oncologiques de support and the Société française de radiothérapie oncologique].

Cancer radiotherapie : journal de la Societe francaise de radiotherapie oncologique
2025

Pulmonary outcomes of pediatric patients with postinfectious bronchiolitis obliterans treated with pulse methylprednisolone and intravenous immunoglobulin: a single-center cohort study.

European journal of pediatrics
2025

From Pulmonary Hyperlucency to Pulmonary Hypertension: A Case Report of Swyer-James-MacLeod Syndrome.

Cureus
2025

The usefulness of transbronchial lung cryobiopsy for chronic lung allograft dysfunction: A case report.

Respiratory investigation
2025

A Severe Infectious Hit to the Lungs With Poor Recovery? Time to Think of Post-Infectious Bronchiolitis Obliterans.

Acta paediatrica (Oslo, Norway : 1992)
2025

Pediatric Bronchiolitis Obliterans and Bronchiolitis Obliterans Syndrome: Nursing Considerations.

Critical care nurse
2025

Cluster analysis for clinical, radiological, and histopathological profiling in chronic pulmonary graft-versus-host disease.

European journal of internal medicine
2025

Current Practices on Prescribing and Deprescribing for Patients on Long-Term Antibiotic Treatment for Chronic Pulmonary Conditions: An Umbrella Review by the European Society of Clinical Pharmacy (ESCP).

COPD
2025

Long-term outcomes of the international EXPAND trial of Organ Care System (OCS) Lung preservation for lung transplantation.

EClinicalMedicine
2025

Indications for pediatric lung transplantation in 2025: A new era.

JHLT open
2025

Bronchodilator Response: Utility and implications in patients undergoing hematopoietic stem cell transplantation.

Respiratory medicine
2025

Postinfectious Bronchiolitis Obliterans in Children.

Canadian respiratory journal
2025

One versus two: How much does it matter? A single-center retrospective study evaluating 1-day extracorporeal photopheresis schedule for treating patients with chronic lung allograft rejection.

Transfusion
2025

Atelectasis predicts poor prognosis in pediatric macrolides-unresponsive Mycoplasma pneumoniae pneumonia with A2063/2064G mutations treated with azithromycin.

Frontiers in cellular and infection microbiology
2025

En-Bloc Versus Bilateral Sequential Technique for Pediatric Lung Transplantation.

Pediatric transplantation
2025

Disease Classification of Pulmonary Xenon Ventilation MRI Using Artificial Intelligence.

Academic radiology
2025

It takes two: Aberrant repair and low-grade inflammation characterize bronchiolitis obliterans syndrome after lung transplantation in serum proteomic analysis.

JHLT open
2025

Respiratory Burden in Hematology-Related Bronchiectasis: Clinical Characteristics and Treatable Traits.

Respiration; international review of thoracic diseases
2025

Addressing Knowledge Gaps in the Early Detection of Bronchiolitis Obliterans Syndrome after Hematopoietic Cell Transplantation: An Official American Thoracic Society Research Statement.

American journal of respiratory and critical care medicine
2025

Clinical Impact of Vaping.

Toxics
2025

A model developed by machine learning and principal component analysis for predicting bronchiolitis obliterans syndrome after allogeneic HSCT in a Chinese population with hematologic malignancies.

Computers in biology and medicine
2025

Pirfenidone for the treatment of bronchiolitis obliterans syndrome related to chronic graft-versus-host disease.

Blood advances
2025

Younger Age and Viral Triggers Were Associated With Post-Infectious Bronchiolitis Obliterans in French Children.

Acta paediatrica (Oslo, Norway : 1992)
2025

Inflammatory Markers and Microbiome Dysbiosis in Hematopoietic Cell Transplant Recipients with Lung Graft-versus-Host Disease.

Transplantation and cellular therapy
2025

Postoperative outcomes of antireflux surgery in lung transplant recipients.

Journal of gastrointestinal surgery : official journal of the Society for Surgery of the Alimentary Tract
2025

Critical care illness in allogeneic hematopoietic stem-cell transplantation recipients with chronic graft versus host disease.

Critical care (London, England)
2025

A new anchor point for gut microbiome to regulate complications of allogeneic hematopoietic stem cell transplantation: oxidative stress.

Experimental hematology
2025

Respiratory management of pediatric patient with bronchiolitis obliterans syndrome during general anesthesia surgery: a case report.

Translational pediatrics
2025

[Risk factors for bronchiolitis obliterans after Mycoplasma pneumoniae bronchiolitis in children].

Zhonghua er ke za zhi = Chinese journal of pediatrics
2025

Corrigendum to Diagnosis of post-hematopoietic stem cell transplant bronchiolitis obliterans syndrome in children: time for a rethink? Transplantation and Cellular Therapy. 2024 Aug;30(8):760-769.

Transplantation and cellular therapy
2025

Bronchiectasis after allogeneic hematopoietic cell transplantation - an underdiagnosed complication.

Respiratory medicine
2025

Efficacy of Intravenous Methylprednisolone Pulses in Children With Postinfectious Bronchiolitis Obliterans.

Pediatric pulmonology
2025

Survival of patients with advanced chronic lung allograft dysfunction and the role of redo transplantation.

JHLT open
2025

Exhaled breath particles as a diagnostic tool for bronchiolitis obliterans syndrome in lung transplant recipients: a longitudinal study.

Frontiers in transplantation
2025

FPPS Downregulation Attenuates Endoplasmic Reticulum Stress in Pulmonary Epithelial Cells.

The journal of gene medicine
2025

Pediatric lung transplantation in China, 2019-2023.

World journal of pediatrics : WJP
2025

Association of Fc gamma-binding protein rs1464897604 polymorphism with bronchiolitis obliterans syndrome in lung transplant recipients.

The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation
2025

What's new in the management of pulmonary complications in allogeneic stem cell transplantation?

Expert review of respiratory medicine
2025

Diagnostic potential of MMP-9, IL-8, and MPO in bronchoalveolar lavage fluid as combined biomarkers for pediatric post-infectious bronchiolitis obliterans.

Journal of immunology (Baltimore, Md. : 1950)
2025

Impaired IgA mucosal immunity following lung transplantation: a potential trigger for bronchiolitis obliterans syndrome.

The European respiratory journal
2025

Case report: refractory atelectasis after infection of adenovirus and Mycoplasma Pneumoniae in an immunocompetent patient.

BMC pulmonary medicine
2025

Use of quantitative CT chest imaging to derive and assess a radiographic phenotype of deployment-related constrictive bronchiolitis.

Respiratory research
2025

Glomerular disease in chronic graft versus host disease.

Nefrologia
2025

The impact of allogeneic hematopoietic stem cell transplantation on pulmonary complications in adults with inborn errors of immunity.

The Journal of allergy and clinical immunology
2025

[Immunological Aspects after Lung Transplantation].

Zentralblatt fur Chirurgie
2025

Changes in Chronic Graft-versus-Host Disease Treatment Over Time: A 15-Years Survey Within Allogeneic Hematopoietic Stem Cell Transplant Centers in Germany, Austria, and Switzerland.

Transplantation and cellular therapy
2025

Role of genetic modifiers on pulmonary consequences of cancer therapy in children.

Scientific reports
2025

cGAS/STING signalling in macrophages aggravates obliterative bronchiolitis via an IFN-α-dependent mechanism after orthotopic tracheal transplantation in mice.

Clinical and translational medicine
2025

Post-RSV Bronchial Web Formation in a Lung Transplant Recipient: A Case Report.

Respirology case reports
2025

Effect of Artificial Food Additives on Lung Health-An Overview.

Medicina (Kaunas, Lithuania)
2025

Lung Transplantation From Donors With a History of Substance Use.

Clinical transplantation
2025

An Autopsy Case of Bronchiolitis Obliterans Associated With Oral Lichen Planus and Non-Hodgkin Lymphoma.

Cureus
2025

Evaluating Metabolic Signatures in the Serum of South Korean Patients with Humidifier Disinfectant-Associated Lung Injury Identified through Untargeted Metabolomics.

Environmental health perspectives
2025

Clinical features of HAdV-55 in children with respiratory tract infections: a retrospective case series and literature review.

BMC infectious diseases
2025

Pediatric Pulmonology 2024 Year in Review: Rare and Diffuse Lung Disease.

Pediatric pulmonology
2025

Clinical characteristics of Mycoplasma pneumoniae pneumonia with airway involvement in children.

Pediatric investigation
2025

Bronchiolitis obliterans due to toxic epidermal necrolysis with structural evaluation using computed tomography bronchography: A case report.

Acute medicine &amp; surgery
2025

Shared roles of immune and stromal cells in the pathogenesis of human bronchiolitis obliterans syndrome.

JCI insight
2025

Chronic Lung Disease after Hospitalization with Adenovirus or Respiratory Syncytial Virus.

The Pediatric infectious disease journal
2025

Clinical course of children with postinfectious bronchiolitis obliterans with versus without comorbid bronchopulmonary dysplasia.

Clinical and experimental pediatrics
2025

Unicentric Castleman disease complicated with bronchiolitis obliterans: A single-centre retrospective study from China.

British journal of haematology
2025

Adenoviral Infections in Immunocompetent Children.

Indian journal of pediatrics
2025

The Role of Pulmonary Genetic Variations in the Pathogenesis of Pediatric Postinfectious Bronchiolitis Obliterans.

Thoracic research and practice
2025

Computed tomography-based radiomic features combined with clinical parameters for predicting post-infectious bronchiolitis obliterans in children with adenovirus pneumonia: a retrospective study.

PeerJ
2025

Outbreak of Post-Infectious Bronchiolitis Obliterans (PIBO) After Adenovirus Infection: A Case Series and Review of the Literature.

Pediatric pulmonology
2025

Risk factors for bronchiolitis obliterans development in children after Mycoplasma pneumoniae pneumonia: a retrospective study of 981 patients.

Italian journal of pediatrics
2025

Pulmonary function testing in pediatric allogeneic stem cell transplant recipients to monitor for Bronchiolitis obliterans syndrome: a systematic review.

BMC pediatrics
2025

Contribution of post-infectious bronchiolitis obliterans to non-cystic fibrosis bronchiectasis in children.

The international journal of tuberculosis and lung disease : the official journal of the International Union against Tuberculosis and Lung Disease
2024

Antireflux surgery for prevention of chronic lung allograft dysfunction (CLAD) onset after lung transplantation: The earlier the better before CLAD onset? A single-center series of 284 patients.

JHLT open
2025

Concomitant abdominal organ transplantation alongside lung transplantation: An ISHLT transplant database analysis.

JHLT open
2025

Lung transplantation for pulmonary chronic graft-versus-host disease: a missed opportunity?

JHLT open
2025

Dynamic 19F-MRI of pulmonary ventilation in lung transplant recipients with and without chronic lung allograft dysfunction.

JHLT open
2025

Radiological distribution patterns in restrictive chronic lung allograft dysfunction: Impact on survival across all phenotypes.

JHLT open
2024

A novel web-based tool for lung transplant patients undergoing extracorporeal photopheresis.

JHLT open
2025

Automated CT Image Processing for the Diagnosis, Prediction, and Differentiation of Phenotypes in Chronic Lung Allograft Dysfunction After Lung Transplantation.

Clinical transplantation
2025

Emerging trends and hotspots in animal experimental research on lung transplantation from 2004 to 2023: a bibliometric analysis.

Journal of thoracic disease
2025

Multiple Myeloma Complicated With Bronchiolitis Obliterans Syndrome After Allogeneic Haematopoietic Stem Cell Transplantation and Subsequently Underwent Lung Transplantation Successfully: A Case Report.

Respirology case reports
2025

DNAH9 variants in children with post-infectious bronchiolitis/bronchitis obliterans.

Orphanet journal of rare diseases
2025

Effect of chronic lung allograft dysfunction phenotypes on the outcome after lung retransplantation: A retrospective single-center data analysis.

JTCVS open
2025

Efficacy of Pomalidomide on Motor Performance and Functional Abilities in Patients with Steroid Refractory Chronic Graft Versus Host Disease: A Randomized Clinical Study.

Transplantation and cellular therapy
2025

Missed Diagnosis of Cystic Fibrosis in Developing Countries-Need to Raise the Awareness!

Pediatric pulmonology
2025

[Post-infectious bronchiolitis obliterans in children: A rare and potentially devastating disease].

Revue des maladies respiratoires
2025

CIP2A promotes bronchiolitis obliterans by activating the NF‑κB pathway.

Molecular medicine reports
2025

Mustard lung with a unique oxidative stress profile as an independent pulmonary disease.

International immunopharmacology
2025

Deficiency of T follicular helper cell Tet3 DNA demethylation inhibits pathogenic IgG2c class switching and chronic GVHD.

Blood
2025

A case report of Castleman disease with paraneoplastic pemphigus and bronchiolitis obliterans: Challenges and key takeaways.

International journal of surgery case reports
2025

Sulfur dioxide exposure of mice induces peribronchiolar fibrosis-A defining feature of deployment-related constrictive bronchiolitis.

PloS one
2025

Bronchiolitis in Adults: Etiology, Diagnostic, and Therapeutic Approach.

Respiration; international review of thoracic diseases
2025

Harnessing deep learning to detect bronchiolitis obliterans syndrome from chest CT.

Communications medicine
2025

Effectiveness of oral platelet lysate gel to treat oral mucosal manifestations associated with chronic graft versus host disease.

Blood transfusion = Trasfusione del sangue
2025

Airway associated inflammation in post-transplant cystic fibrosis patients as a predictor of chronic lung allograft dysfunction (CLAD).

Journal of clinical pathology
2024

Donor-derived cell-free DNA in chronic lung allograft dysfunction phenotypes: a pilot study.

Frontiers in transplantation
2024

Immune dysregulation as a driver of bronchiolitis obliterans.

Frontiers in immunology
2024

Efficacy of transbronchial biopsy and frequency of pneumothorax in lung transplant recipients: single center experience.

BMC pulmonary medicine
2025

Clonal GZMK+CD8+ T cells are identified as a hallmark of the pathogenesis of cGVHD-induced bronchiolitis obliterans syndrome after allogeneic hematopoietic stem cell transplantation.

EBioMedicine
2024

Effects of paclitaxel and methotrexate associated with cholesterol-rich nanoemulsions on ischemia-reperfusion injury after unilateral lung transplantation in rats.

Scientific reports
2024

Construction and validation of a nomogram model to predict bronchiolitis Mycoplasma pneumoniae pneumonia in children.

Scientific reports
2025

High-titer rheumatologic markers in serum of veterans with severe pulmonary complications 25-30 years after sulfur mustard exposure.

International immunopharmacology
2025

Distinct inflammatory profiles in mustard lung: A study of sulfur mustard-exposed patients with serious pulmonary complications.

International immunopharmacology
2025

Association of hormone profile with various respiratory disorders in sulfur mustard-chemical veterans.

International immunopharmacology
2025

Low-dose heparin sodium as a protective factor against bronchiolitis obliterans formation after adenovirus infection.

Annals of medicine
Ver todos os 1.835 no EuropePMC

Associações

Organizações que acompanham esta doença — pra ter apoio e orientação

Comunidade internacional de pacientes, famílias e profissionais de saúde dedicada à Bronquiolite Obliterante (BO). Reúne testemunhos, informações sobre cuidados, medicamentos e profissionais especializados. Apoiada pela associação Hope for Gabriel.

Comunidades

Grupos ativos de quem convive com esta doença aqui no Raras

Ainda não existe comunidade no Raras para Bronquiolite obliterante

Pacientes, familiares e cuidadores se organizam em comunidades pra compartilhar experiências, fazer perguntas e se apoiar. Você pode ser o primeiro.

Tire suas dúvidas

Perguntas, dicas e experiências compartilhadas aqui na página

Participe da discussão

Faça login para postar dúvidas, compartilhar experiências e interagir com especialistas.

Fazer login

Doenças relacionadas

Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico

Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. Ruxolitinib and Tocilizumab for Child Bronchiolitis Obliterans After Toxic Epidermal Necrolysis.
    Pediatrics· 2026· PMID 41812997mais citado
  2. BET inhibition blunts antibody production and macrophage-mediated fibrosis to restore lung function in murine cGVHD.
    Blood· 2026· PMID 41770848mais citado
  3. Post-Infectious Bronchiolitis Obliterans as a Model of Immune-Mediated Airway Fibrosis: A Pediatric Case Report.
    International journal of molecular sciences· 2026· PMID 41751939mais citado
  4. Targeting integrin beta 4 in diacetyl-induced anoikis of the airway epithelium.
    Cell death discovery· 2026· PMID 41741435mais citado
  5. Longitudinal Study of Late Acute and Chronic Graft-Versus-Host Disease after Allogeneic Hematopoietic Cell Transplantation: A Long-Term Follow-up Study from the Chronic Graft-Versus-Host Disease Consortium.
    Transplantation and cellular therapy· 2026· PMID 41706088mais citado
  6. Risk factors and prediction models for bronchitis obliterans after severe Mycoplasma pneumoniae pneumonia with plastic bronchitis in children.
    Pak J Med Sci· 2026· PMID 41994393recente
  7. Distinct central and peripheral airway features in bronchiolitis obliterans and COPD: an observational study.
    BMC Pulm Med· 2026· PMID 41987165recente
  8. Pulmonary complications in pediatric hematopoietic stem cell transplantation: an overview for pediatricians.
    Curr Opin Pediatr· 2026· PMID 41983728recente
  9. Early BAL microRNA Signatures Delineate Biological Trajectories Towards CLAD After Lung Transplantation.
    Cells· 2026· PMID 41972701recente
  10. Pulmonary Function Deficit and Clinical Associations in Childhood Acute Lymphoblastic Leukaemia Survivors: A National Retrospective ALL-STAR Lungs Cohort Study.
    Pediatr Pulmonol· 2026· PMID 41969139recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:1303(Orphanet)
  2. MONDO:0015265(MONDO)
  3. GARD:5961(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. Q56013703(Wikidata)

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Bronquiolite obliterante
Compêndio · Raras BR

Bronquiolite obliterante

ORPHA:1303 · MONDO:0015265
Prevalência
Unknown
Herança
Not applicable
CID-10
J44.8 · Outras formas especificadas de doença pulmonar obstrutiva crônica
CID-11
Ensaios
5 ativos
Início
All ages
Prevalência
0.0 (Worldwide)
MedGen
UMLS
C0006272
EuropePMC
Wikidata
Papers 10a
DiscussaoAtiva

Nenhuma novidade ainda. O agente esta monitorando.

0membros
0novidades

📋 Origem dos dados

Esta página agrega dados de fontes públicas e oficiais. Dados sobre cobertura no SUS (PCDT, CEAF) são verificados ativamente por agente proativo (ver badge no infobox). Demais dados têm atribuição de fonte + data da última sincronização — clique para abrir o original.

Doença rara (ontologia)
fonte: Orphanet
Identificador unificado
fonte: MONDO
Dado público estruturado
fonte: Wikidata
Medicamentos aprovados FDA
fonte: FDA OpenFDA