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.

Outpatient management of primary spontaneous pneumothorax.

The outpatient management of primary spontaneous pneumothorax (PSP) is still debated. The risk of a tension pneumothorax is used to justify active treatment like chest-tube drainage, although outpatient management can reduce both the time in hospital and the cost of treatment. It is also likely to be the patient's choice. This report is a reappraisal of the situations for which outpatient management, by monitoring alone, or using minimally invasive techniques, can be considered.

Exclusive ambulatory management of spontaneous pneumothorax with pigtail catheters, a prospective multicentric study.

OBJECTIVE: Spontaneous pneumothorax occurs most frequently in young active patients. Published guidelines do not all agree about its initial management; most patients are hospitalised and treated with chest tube. This prospective multicentric cohort study was designed to assess the potential of ambulatory management. METHODS: We included all consecutive patients with large spontaneous primary (PSP) and secondary pneumothoraces (SSP) presenting at the Lorient, Vitré and Rennes hospitals between December 2013 and July 2016. They were treated with a small-bore pigtail catheter and one-way valve and managed as outpatients following a specific protocol. When this failed, patients were hospitalised on day 4 for suction and surgical pleurodesis was envisaged on day 6. Patients were followed-up for one-year to assess relapse. RESULTS: Of the 148 patients included (129 PSP, 19 SSP), 122 (82⋅4%) were managed exclusively as outpatient with success in 84⋅5% of PSP and 68⋅4% of SSP patients. There were few complications: 13 vaso-vagal episodes and 3 minor bleedings. The one-year recurrence rates were 33⋅1% for PSP and 52⋅6% for SSP (p = 0⋅114 Hazard Ratio = 0⋅538; IC95% [0⋅249-1⋅161]). CONCLUSION: These results are consistent with our previous study and confirm that this exclusive ambulatory management of spontaneous pneumothoraces can be successfully implemented in new centres with a high success rate and few complications.

Ambulatory management of large spontaneous pneumothorax with pigtail catheters.

STUDY OBJECTIVE: There is no consensus about the management of large spontaneous pneumothoraces. Guidelines recommend either needle aspiration or chest tube drainage and most patients are hospitalized. We assess the efficiency of ambulatory management of large spontaneous pneumothoraces with pigtail catheters. METHODS: From February 2007 to January 2011, all primary and secondary large spontaneous pneumothoraces from Lorient's hospital (France) were managed with pigtail catheters with a 1-way valve. The patients were discharged immediately and then evaluated every 2 days according to a specific algorithm. RESULTS: Of the 132 consecutive patients (110 primary, 22 secondary), 103 were exclusively managed as outpatients, with full resolution of the pneumothorax by day 2 or 4, which represents an ambulatory success rate of 78%. Mean time (SD) of drainage was 3.4 days (1.8). Seven patients were initially hospitalized but quickly discharged and had full resolution by day 2 or 4, leading to a total success rate of 83%. The use of analgesics was low. The 1-year recurrence rate was 26%. If successful, this outpatient management is potentially cost saving, with a mean cost of $926, assuming up to 2 outpatient visits and 1 chest radiograph, compared with $4,276 if a chest tube was placed and the patient was admitted to the hospital for 4 days. CONCLUSION: Ambulatory management with pigtail catheters with 1-way valves could be a reasonable first-line of treatment for large spontaneous pneumothoraces. Compared with that of other studies, our protocol does not require hospitalization and is cost saving.