PMID- 30686554
OWN - NLM
STAT- MEDLINE
DCOM- 20200116
LR  - 20200116
IS  - 1776-2588 (Electronic)
IS  - 0761-8425 (Linking)
VI  - 36
IP  - 4
DP  - 2019 Apr
TI  - [A case of bronchiectasis due to light chain deposition disease].
PG  - 538-542
LID - S0761-8425(18)31016-7 [pii]
LID - 10.1016/j.rmr.2018.11.008 [doi]
AB  - INTRODUCTION: The natural history of orphan lung diseases is often unclear. We 
      report the long-term follow-up of a case of bronchiectasis due to pulmonary non 
      amyloid light chain deposition disease (LCDD). CASE REPORT: A 50-year-old woman 
      who was a smoker, was diagnosed with diffuse thin walled bronchiectasis of 
      uncertain origin after presenting with a respiratory tract infection. Ten years 
      later, the combination of bronchiectasis, the appearance of pulmonary cysts and 
      the identification of increased kappa free light chains evoked the diagnosis of 
      pulmonary LCDD. The diagnosis was confirmed by lung biopsy. No 
      immunoproliferative disorder was identified. During the 12 years follow-up, 
      dyspnea worsened progressively and bronchiectasis and lung cysts extended leading 
      to multicystic lung disease. Pulmonary function tests did not show any 
      ventilatory defect but a small decrease in carbon monoxide transfer factor 
      occurred. CONCLUSION: We describe the evolution of a rare presentation of 
      isolated pulmonary LCDD, characterized by cystic diffuse atypical bronchiectasis 
      with thin walls, associated with progressive cystic destruction of the lung 
      parenchyma. The possibility of pulmonary LCDD should be considered in cases of 
      atypical bronchiectasis of unknown etiology.
CI  - Copyright (c) 2019 SPLF. Published by Elsevier Masson SAS. All rights reserved.
FAU - Millet, F
AU  - Millet F
AD  - Departement de pneumologie, hopitaux de Brabois, CHU de Nancy, rue du Morvan, 
      54500 Vandoeuvre-les-Nancy, France. Electronic address: floriane-millet@orange.fr.
FAU - Gomez, E
AU  - Gomez E
AD  - Departement de pneumologie, hopitaux de Brabois, CHU de Nancy, rue du Morvan, 
      54500 Vandoeuvre-les-Nancy, France.
FAU - Hirschi, S
AU  - Hirschi S
AD  - Service de pneumologie, nouvel hopital Civil, hopitaux universitaires de 
      Strasbourg, 67000 Strasbourg, France.
FAU - Petit, I
AU  - Petit I
AD  - Departement de radiologie, hopitaux de Brabois, CHU de Nancy, 54500 
      Vandoeuvre-les-Nancy, France.
FAU - Chenard, M-P
AU  - Chenard MP
AD  - Service d'anatomo-pathologie, hopitaux universitaires de Strasbourg, 67000 
      Strasbourg, France.
FAU - Mouget, B
AU  - Mouget B
AD  - Departement de pneumologie, hopitaux de Brabois, CHU de Nancy, rue du Morvan, 
      54500 Vandoeuvre-les-Nancy, France.
FAU - Guillaumot, A
AU  - Guillaumot A
AD  - Departement de pneumologie, hopitaux de Brabois, CHU de Nancy, rue du Morvan, 
      54500 Vandoeuvre-les-Nancy, France.
FAU - Chaouat, A
AU  - Chaouat A
AD  - Departement de pneumologie, hopitaux de Brabois, CHU de Nancy, rue du Morvan, 
      54500 Vandoeuvre-les-Nancy, France.
FAU - Colombat, M
AU  - Colombat M
AD  - Service d'anatomo-pathologie, CHU de Toulouse, 31300 Toulouse, France.
FAU - Chabot, F
AU  - Chabot F
AD  - Departement de pneumologie, hopitaux de Brabois, CHU de Nancy, rue du Morvan, 
      54500 Vandoeuvre-les-Nancy, France.
LA  - fre
PT  - Case Reports
PT  - Journal Article
TT  - Histoire naturelle d'une forme bronchectasiante de la maladie a depots de chaines 
      legeres.
DEP - 20190124
PL  - France
TA  - Rev Mal Respir
JT  - Revue des maladies respiratoires
JID - 8408032
RN  - 0 (Immunoglobulin Light Chains)
SB  - IM
MH  - Bronchiectasis/diagnosis/*etiology/pathology
MH  - Female
MH  - Humans
MH  - Immunoglobulin Light Chains/metabolism
MH  - Immunoglobulin Light-chain Amyloidosis/*complications/diagnosis/pathology
MH  - Middle Aged
MH  - Paraproteinemias/*complications/diagnosis/pathology
MH  - Smokers
OTO - NOTNLM
OT  - Bronchectasies
OT  - Bronchiectasis
OT  - Immunoglobulines kappa
OT  - Kappa-immunoglobulin light chain
OT  - Kystes pulmonaires
OT  - Light chain deposition disease
OT  - Maladie a depots de chaines legeres
OT  - Non amyloides
OT  - Non-amyloid
OT  - Pulmonary cysts
EDAT- 2019/01/29 06:00
MHDA- 2020/01/17 06:00
CRDT- 2019/01/29 06:00
PHST- 2017/10/06 00:00 [received]
PHST- 2018/11/13 00:00 [accepted]
PHST- 2019/01/29 06:00 [pubmed]
PHST- 2020/01/17 06:00 [medline]
PHST- 2019/01/29 06:00 [entrez]
AID - S0761-8425(18)31016-7 [pii]
AID - 10.1016/j.rmr.2018.11.008 [doi]
PST - ppublish
SO  - Rev Mal Respir. 2019 Apr;36(4):538-542. doi: 10.1016/j.rmr.2018.11.008. Epub 2019 
      Jan 24.