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

BACKGROUND: 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. STUDY DESIGN AND METHODS: 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. RESULTS: Thirty-one cases were identified (68% female), with a median age at diagnosis of 50 years (IQR 20). Baseline FEV1/FVC was < 0.70 in 45% of patients. Mean (± SD) FEV1 and DLCO were 86% ± 26.2 and 52% ± 23.9, respectively. CT revealed peculiar patterns of thin-walled cysts (58%) and thin-walled cystic bronchiectases (27%). Increased serum kappa light chain was found in 87% of patients. Histological analysis showed kappa light chain deposits in all patients, except one with lambda chain deposits. Median annual FEV1 decline was 127 ml (IQR 178) and median DLCO decline was 4.3% (IQR 4.3). Sixteen patients received immunomodulatory treatment or chemotherapy; serum light chain levels decreased in 9 cases (75%), without significant improvement in FEV1 (p = 0.173). Overall, 48% of patients underwent bilateral lung transplantation. Transplant-free survival at 5 and 10 years were 70% and 30%, respectively. An annual FEV1 decline greater than 127 ml/year was associated with increased risk of death or transplantation (p = 0.005). CONCLUSIONS: Diffuse pulmonary LCDD is characterised by female predominance, a peculiar imaging pattern with bronchiectasis and/or cysts, progressive airway obstruction and severe DLCO impairment, and poor outcome. Lung transplantation is a treatment of choice.

Hypnosis associated with 3D immersive virtual reality technology during bronchoscopy under local anesthesia.

Background: Patients undergoing flexible bronchoscopy under local anesthesia usually experience anxiety before and during the procedure. Different non-pharmacological techniques, including music and hypnosis, are used to distract patients' attention, and to reduce anxiety. The new technique "virtual reality hypnosis (VRH)", defined as a hypnotic induction suggestion delivered by personalized virtual reality software, can generate a simulation of a lifelike environment. No study has described the use of VRH during bronchoscopy. The objective is to investigate the anxiety reducing effect and the satisfaction of patients, physicians, and nurses using VRH during bronchoscopy. Methods: VRH was proposed to all patients who experienced anxiety before undergoing flexible bronchoscopy under local anesthesia. Local anesthesia was performed using 5% lidocaine spray only. No sedation was used. After the procedure, patients, physicians and nurses filled a standardized satisfaction form. Results: Twenty consecutive patients who reported pre-procedure anxiety were included. The sex ratio was 16 women/4 men, the median age was 65 years. Eight patients (40%) had undergone a previous bronchoscopy under local anesthesia. The median duration of the procedure was 10 minutes, and all procedures were completed. The median level of anxiety of patients decreased from 9/10 before the procedure to 4/10 during the procedure. The median satisfaction rate regarding the use of VRH was 10/10. All patients agreed to use VRH again in case of a new bronchoscopy procedure. Conclusions: This preliminary report has shown that VRH was useful to reduce patients' anxiety during bronchoscopy under local anesthesia. VRH was easily implemented in the routine practice.