ArticleJCI insight2020
MAPK mutations and cigarette smoke promote the pathogenesis of pulmonary Langerhans cell histiocytosis.
Article in JCI insight, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 13 papers.
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Who cites it
13 citing papers in PubMed, 43 citations in OpenAlex.
- Blood MMP-7 and TNF-α levels as potential prognostic biomarkers for adult pulmonary Langerhans cell histiocytosis.Scientific reports · 2026Article
- A 26-year-old with persistent cough of unclear origin.Breathe (Sheffield, England) · 2025Article
- BRAF V600E-Mutant Diffuse Pulmonary Langerhans Cell Histiocytosis Successfully Treated With Dabrafenib Plus Trametinib: A Case Report.Respirology case reports · 2025Article
- Impact of Sex on Lung Function in Adult Langerhans Cell Histiocytosis.Life (Basel, Switzerland) · 2025Article
- Circulating monocytes decrease significantly following disease-directed therapy and may reflect disease expansion in Langerhans Cell Histiocytosis.Annals of hematology · 2024Article
- Bronchoalveolar cytokine profile differentiates Pulmonary Langerhans cell histiocytosis patients from other smoking-related interstitial lung diseases.Respiratory research · 2023Article
- Article
- Clarifying the relationship between pulmonary langerhans cell histiocytosis and Alpha 1 antitrypsin deficiency.Orphanet journal of rare diseases · 2021Article
- Adult pulmonary Langerhans cell histiocytosis might consist of two distinct groups: isolated form and extrapulmonary recidivism type.Annals of translational medicine · 2021Article
- Epstein-Barr Virus-Positive Langerhans Cell Sarcoma: Is There a Link? A Case Report.Frontiers in oncology · 2021Article
- Article
- Update on Pulmonary Langerhans Cell Histiocytosis.Frontiers in medicine · 2020Review
- Smoking-related interstitial lung disease: A narrative review.Chronic respiratory diseaseReview
Corrections and comments
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Authors and funding
13 authors at 5 institutions in 3 countries.
Funding
Abstract
Pulmonary Langerhans cell histiocytosis (PLCH) is a rare smoking-related lung disease characterized by dendritic cell (DC) accumulation, bronchiolocentric nodule formation, and cystic lung remodeling. Approximately 50% of patients with PLCH harbor somatic BRAF-V600E mutations in cells of the myeloid/monocyte lineage. However, the rarity of the disease and lack of animal models have impeded the study of PLCH pathogenesis. Here, we establish a cigarette smoke-exposed (CS-exposed) BRAF-V600E-mutant mouse model that recapitulates many hallmark characteristics of PLCH. We show that CD11c-targeted expression of BRAF-V600E increases DC responsiveness to stimuli, including the chemokine CCL20, and that mutant cell accumulation in the lungs of CS-exposed mice is due to both increased cellular viability and enhanced recruitment. Moreover, we report that the chemokine CCL7 is secreted from DCs and human peripheral blood monocytes in a BRAF-V600E-dependent manner, suggesting a possible mechanism for recruitment of cells known to dominate PLCH lesions. Inflammatory lesions and airspace dilation in BRAF-V600E mice in response to CS are attenuated by transitioning animals to filtered air and treatment with a BRAF-V600E inhibitor, PLX4720. Collectively, this model provides mechanistic insights into the role of myelomonocytic cells and the BRAF-V600E mutation and CS exposure in PLCH pathogenesis and provides a platform to develop biomarkers and therapeutic targets.
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Registered trials
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