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<datestamp>2017-12-21</datestamp>
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<publisher>HAL CCSD</publisher>
<title lang=fr>Une cause rare de maladie kystique pulmonaire : maladie à dépôts de chaînes légères d'immunoglobuline. [An unusual cause of cystic lung disease: Light chain deposition disease].</title>
<creator>Luraine, Régis</creator>
<creator>Sohier, Laurent</creator>
<creator>Kerjouan, Mallorie</creator>
<creator>Desrues, Benoit</creator>
<creator>Delaval, Philippe</creator>
<creator>Jouneau, Stéphane</creator>
<contributor>Service de pneumologie ; Hôpital Pontchaillou - CHU Pontchaillou [Rennes]</contributor>
<contributor>Service de pneumologie [Lorient] ; centre hospitalier Bretagne sud</contributor>
<contributor>Contaminants Chimiques, immunité et Inflammation ; Institut de recherche, santé, environnement et travail [Rennes] (Irset) ; Université d'Angers (UA) - Université des Antilles et de la Guyane (UAG) - Université de Rennes 1 (UR1) - École des Hautes Études en Santé Publique [EHESP] (EHESP) - Institut National de la Santé et de la Recherche Médicale (INSERM) - Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ) - Université d'Angers (UA) - Université des Antilles et de la Guyane (UAG) - Université de Rennes 1 (UR1) - École des Hautes Études en Santé Publique [EHESP] (EHESP) - Institut National de la Santé et de la Recherche Médicale (INSERM) - Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ) - Service de pneumologie ; Hôpital Pontchaillou - CHU Pontchaillou [Rennes] - Hôpital Pontchaillou - CHU Pontchaillou [Rennes]</contributor>
<description>International audience</description>
<source>ISSN: 0761-8425</source>
<source>Revue des Maladies Respiratoires</source>
<publisher>Elsevier Masson</publisher>
<identifier>hal-00873356</identifier>
<identifier>https://hal.archives-ouvertes.fr/hal-00873356</identifier>
<identifier>https://hal.archives-ouvertes.fr/hal-00873356/document</identifier>
<identifier>https://hal.archives-ouvertes.fr/hal-00873356/file/DA_pots_chaines_lA_gA_res_non_amyloides_version_finale.pdf</identifier>
<source>https://hal.archives-ouvertes.fr/hal-00873356</source>
<source>Revue des Maladies Respiratoires, Elsevier Masson, 2013, 30 (7), pp.567-71. 〈10.1016/j.rmr.2013.02.003〉</source>
<identifier>DOI : 10.1016/j.rmr.2013.02.003</identifier>
<relation>info:eu-repo/semantics/altIdentifier/doi/10.1016/j.rmr.2013.02.003</relation>
<identifier>PUBMED : 24034462</identifier>
<relation>info:eu-repo/semantics/altIdentifier/pmid/24034462</relation>
<language>fr</language>
<subject lang=fr>Immunoglobulines Kappa</subject>
<subject lang=fr>Non amyloïde</subject>
<subject lang=fr>Pneumopathie infiltrante diffuse</subject>
<subject lang=fr>Kystes pulmonaires</subject>
<subject lang=fr>Maladie à dépôts de chaînes légères</subject>
<subject>[SDV.MHEP.PSR] Life Sciences [q-bio]/Human health and pathology/Pulmonology and respiratory tract</subject>
<type>info:eu-repo/semantics/article</type>
<type>Journal articles</type>
<description lang=en>INTRODUCTION: Light chain deposition disease is a rare clinical entity characterized by deposition of monoclonal immunoglobulin light chains in organs. The kidneys are almost always affected, while the lung manifestations that have been reported, including nodular or diffuse disease, especially cystic lesions, are unusual. CASE REPORT: We report the case of a 60-year-old man with a diffuse infiltrative lung disease characterized by numerous apical cysts. The diagnosis of light chain deposition cystic lung disease was obtained by surgical lung biopsy. Light chain deposits in the salivary glands were the only extrapulmonary manifestation. Despite 12 chemotherapy cycles, the patient's lung function and radiological appearances worsened. CONCLUSION: This is the fourth case describing a cystic lung disease due to light chain deposition in the literature. It highlights the need for comprehensive investigations so as not to miss this rare cause of cystic lung disease, which appears to be related to a primary pulmonary lymphoproliferative disorder. The only treatment that appears to be effective is lung transplantation.</description>
<date>2013-09</date>
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