RT Journal Article
SR Electronic
T1 Light chain deposition disease involving the airways: diagnosis by fibreoptic bronchoscopy
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP 1057
OP 1060
DO 10.1183/09031936.00134406
VO 29
IS 5
A1 M. Colombat
A1 V. Gounant
A1 H. Mal
A1 P. Callard
A1 B. Milleron
YR 2007
UL http://erj.ersjournals.com/content/29/5/1057.abstract
AB Light chain deposition disease (LCDD) infrequently affects the lungs and usually causes damage to the parenchyma, while bronchial involvement appears to be very rare. The present authors report the case of a 64-yr-old female with LCDD characterised by asymptomatic airway involvement. Ten months after excision of a poorly differentiated vaginal carcinoma, a routine chest computed tomography (CT) scan revealed two lung cysts, several bilateral nodules and diffuse bronchial thickening. Pulmonary function tests were normal. Fibreoptic bronchoscopy showed marked diffuse mucosal thickening with highly conspicuous vascular plexuses. Nonamyloidal deposits were found in the bronchial wall, but no definite diagnosis could be proposed. On follow-up, the patient was still asymptomatic and the CT scan and endoscopic appearance remained unchanged. The final diagnosis of κ LCDD was established 18 months later by another series of bronchial biopsies with frozen samples. Interestingly, electron microscopy showed dense granular deposits associated with nonamyloidal fibrils. An increased number of lung cysts were observed 32 months after identification of bronchial abnormalities, confirming the progressive nature of the disease. No extrapulmonary deposits or immunoproliferative disorder were found. In conclusion, light chain deposition disease, which may remain latent for several years, can entirely involve large airways and may be diagnosed by bronchial biopsy.