[A case of bronchiectasis due to light chain deposition disease]. / Histoire naturelle d'une forme bronchectasiante de la maladie à dépôts de chaînes légères.

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.

[Occupational asthma due to hard metals hypersensitivity]. / Asthme professionnel par hypersensibilité aux métaux durs.

The authors report the case of a worker in the hard metal industry presenting with asthma due to cobalt and nickel. The diagnosis was supported by the history, positive skin tests and lymphocyte activation as well as elevated levels of the metals in the urine and BAL. Challenge led to a delayed asthmatic reaction occurring 3.5 to 24 hours after exposure. The BAL contained high levels of tungsten and cobalt, the level of the latter doubling 48 hours after exposure. After the provocation test a nasal and broncho-alveolar eosinophilia was observed. The possibility of a delayed hypersensitivity reaction to metals is discussed by the authors.