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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Published in:Respiratory research 2024-04, Vol.25 (1), p.159-159, Article 159
Main Authors: 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: Article
Language:English
Subjects:
Adult
Airway management
Antibodies
Biopsy
Body organs
Bone marrow
Bronchiectasis
Bronchoscopy
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
Patients
Phenotype
Phenotypes
Retrospective Studies
Scientific imaging
Serum
Survival
Tomography
Transplantation
Young Adult
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Summary: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