Phenotypes and outcome of diffuse pulmonary non-amyloid light chain deposition disease

Light chain deposition disease (LCDD) is a very rare entity. Clinical manifestations of LCDD vary according to the organs involved. Data on pulmonary LCDD are scarce and limited to small series or case reports. This study aimed to describe the characteristics and outcome of diffuse pulmonary non-amy...

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Veröffentlicht in:	Respiratory research 2024-04, Vol.25 (1), p.159-159, Article 159
Hauptverfasser:	Lestelle, François, Beigelman, Catherine, Rotzinger, David, Si-Mohamed, Salim, Nasser, Mouhamad, Wemeau, Lidwine, Hirschi, Sandrine, Prevot, Grégoire, Roux, Antoine, Bunel, Vincent, Gomez, Emmanuel, Sohier, Laurent, Pradier, Helene Morisse, Gaubert, Martine Reynaud, Gondouin, Anne, Lazor, Romain, Glerant, Jean-Charles, Bejui, Françoise Thivolet, Colombat, Magali, Cottin, Vincent
Format:	Artikel
Sprache:	eng
Schlagworte:	Adult Airway management Analysis Antibodies Biopsy Body organs Bone marrow Bronchiectasis Bronchoscopy Cancer Case reports Chemotherapy Cloning Cysts Cysts - pathology Deposition Diagnosis Dyspnea Female Females Health services Histology Humans Immunoglobulin Light Chains Immunoglobulins Immunohistochemistry Immunomodulation Life Sciences Light Light chain deposition disease Light levels Lung Lung - diagnostic imaging Lung - pathology Lung cysts Lung transplantation Lung transplants Lungs Male Mass spectrometry Microscopy Patient outcomes Patients Phenotype Phenotypes Retrospective Studies Scientific imaging Serum Survival Tomography Transplantation Transplantation of organs, tissues, etc Young Adult
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Zusammenfassung:	Light chain deposition disease (LCDD) is a very rare entity. Clinical manifestations of LCDD vary according to the organs involved. Data on pulmonary LCDD are scarce and limited to small series or case reports. This study aimed to describe the characteristics and outcome of diffuse pulmonary non-amyloid LCDD localized to the lungs. A multicenter retrospective cohort study was conducted. Clinical characteristics were collected, and chest CTs were centrally reviewed. The diagnosis of pulmonary non-amyloid LCDD was confirmed by immunohistochemistry. Thirty-one cases were identified (68% female), with a median age at diagnosis of 50 years (IQR 20). Baseline FEV1/FVC was
ISSN:	1465-993X 1465-9921 1465-993X 1465-9921
DOI:	10.1186/s12931-024-02798-y