L’histiocytose langerhansienne pulmonaire de l’adulte
La Revue de Médecine Interne, ISSN: 0248-8663, Vol: 24, Issue: 4, Page: 230-236
2003
- 7Citations
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Metrics Details
- Citations7
- Citation Indexes7
- CrossRef6
Article Description
Propos. – Cette mise au point concerne l’histiocytose langerhansienne, encore appelée histiocytose X ou granulomatose à cellules de Langerhans. La forme pulmonaire isolée de l’adulte est une affection rare qui touche préférentiellement le sujet jeune, fumeur. La maladie est caractérisée par des lésions granulomateuses s’attaquant aux bronchioles distales qu’elles détruisent progressivement, laissant place à des bronchiolectasies prenant l’aspect de kystes cicatriciels caractéristiques sur coupes. Les lésions florides sont riches en cellules de Langerhans, cellules présentatrices d’antigène appartenant à la lignée des cellules dendritiques, associées à des lymphocytes T et des éosinophiles. Le diagnostic repose en pratique clinique sur la convergence d’arguments épidémiologiques, cliniques, radiographiques et surtout tomodensitométriques (association typique de nodules et de kystes prédominant dans les deux tiers supérieurs des 2 champs pulmonaires). Une confirmation histopathologique, examen de référence, s’impose dans les formes moins typiques. L’évolution clinique est favorable dans au moins deux tiers des cas, la régression étant facilitée dans certains cas par le sevrage tabagique. Toutefois, le pronostic est plus réservé en cas de survenue à un âge tardif, de baisse sévère du rapport VEMS/CV et de TLCO et la maladie peut alors réduire l’espérance de vie. Actualité et points forts. – La pathogénie de l’histiocytose langerhansienne pulmonaire demeure inconnue, mais plusieurs arguments suggèrent qu’une réponse immunitaire incontrôlée initiée par les cellules de Langerhans et dirigée contre l’épithélium bronchiolaire est à l’origine de la maladie. D’autres arguments suggèrent la présence d’un terrain génétique particulier, favorisant par exemple une plus grande sensibilité des cellules de Langerhans à des facteurs (GM-CSF, entre autres) qui influencent leur croissance et leur survie. Perspectives. – Bien que la maladie demeure de cause inconnue, les progrès récents concernant ses mécanismes immunopathologiques devraient favoriser à terme la mise au point de stratégies thérapeutiques nouvelles visant à réguler le nombre ou l’activation des cellules de Langerhans aux sites des lésions. Introduction. – Adult pulmonary Langerhans’cell histiocytosis, also referred to as histiocytosis X, is a disorder of unknown etiology which affects preferentially young smokers. The disease is characterized by granulomatous lesions which progressively invade and destroy distal airways, leading to the formation of characteristic cicatricial kystic lesions. Florid granulomas contain numerous Langerhans’cells, antigen-presenting cells of the dendritic cell lineage, associated with T lymphocytes and eosinophils. The diagnosis rests on the combination of clinical and radiologic data, and particularly on high-resolution CT scan findings showing a typical association of nodular and cystic changes, predominantly in the upper and middle lobes. Further evaluation with surgical lung biopsy is indicated in less typical situations. Current knowledge and key points. – The pathogenesis of Langerhans’cell histiocytosis is not fully understood, but several arguments suggest that the disease results from an abnormal immune reaction initiated by Langerhans’cells and directed against the bronchial epithelium. Other arguments suggest the presence of genetic abnormalities susceptible, for example, to increase the sensitivity of these cells to cytokines (GM-CSF, or others) known to influence their survival and maturation. Future prospects and projects. – These recent advances in the pathogenesis of Langerhans’cell histiocytosis could promote the development of new therapeutic strategies designed to regulate the number and activated state of Langerhans’cells in specific lesions.
Bibliographic Details
http://www.sciencedirect.com/science/article/pii/S0248866303000559; http://dx.doi.org/10.1016/s0248-8663(03)00055-9; http://www.scopus.com/inward/record.url?partnerID=HzOxMe3b&scp=0038373574&origin=inward; http://www.ncbi.nlm.nih.gov/pubmed/12706779; http://linkinghub.elsevier.com/retrieve/pii/S0248866303000559; http://api.elsevier.com/content/article/PII:S0248866303000559?httpAccept=text/xml; http://api.elsevier.com/content/article/PII:S0248866303000559?httpAccept=text/plain; https://linkinghub.elsevier.com/retrieve/pii/S0248866303000559; http://dx.doi.org/10.1016/s0248-8663%2803%2900055-9; https://dx.doi.org/10.1016/s0248-8663%2803%2900055-9
Elsevier BV
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