Light chain deposition disease involving the airways: diagnosis by fibreoptic bronchoscopy

Abstract

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 k 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.

Blood stem cell transplantation to treat cystic lung light chain deposition disease

“…CL-LCDD has been reported in five cases [1,2,5]. The patients were predominantly female, were 20-64 years-old and were light or nonsmokers.…”

“…CL-LCDD should be considered among the causes of multiple cystic lung diseases, especially with nodes or bronchiectasis. According to this case and COLOMBAT et al [2], the level of sFLC and endobronchial biopsies using tissue frozen during a flexible bronchoscopy are useful for diagnosis.…”

“…Deaths from these medications exceed deaths from all illicit drugs combined [1]. Methadone (for pain treatment) is involved in one third of US prescription opioid overdose deaths despite accounting for only 5% of dispensed opioids [2]. There is a dose-dependent increase in the severity of central sleep apnoea (CSA) with methadone [3-5] and sleep disordered breathing is a contributing factor in methadone-related deaths [2].…”

“…A third presentation involves the airways [2,3]. In nodular disease, patients are usually asymptomatic or have moderate respiratory symptoms (e.g.…”

Natural history and outcome of light chain deposition disease

“…9,12,16,17 Liver LCDD was confirmed histologically in both cases in which it was suspected biochemically, and lung LCDD was confirmed by surgical lung biopsy in a patient with panacinar emphysema. 18 …”