Published ahead of print on January 6, 2006, doi:10.1164/rccm.200510-1620CR Am. J. Respir. Crit. Care Med., Volume 173, Number 7, April 2006, 777-780 A more recent version of this article appeared on April 1, 2006
Submitted on October 17, 2005 Pulmonary Cystic Disorder Related to Light Chain Deposition DiseaseMagali Colombat1*,1 Service d'Anatomie Pathologique, Hopital Beaujon, Clichy, France, 2 Service de Pneumologie, Hopital Foch, Suresnes, France, 3 Service d'Anatomie Pathologique, Hopital Saint-Louis, Paris, France, 4 Service de Radiologie, Hopital Avicenne, Bobigny, France, 5 Service de Pneumologie, Hopital Avicenne, Bobigny, France, 6 Service de Pneumologie, Hopital Beaujon, Clichy, France, 7 Service de Pneumologie, Hopital Intercommunal, Creteil, France, 8 Service de Medecine Interne, Groupe Hospitalier Pitie-Salpetriere, Paris, France, 9 Service d'Anatomie Pathologique, Hopital Europeen Georges Pompidou, Paris, France * To whom correspondence should be addressed. E-mail: colombatm{at}yahoo.fr.
Light chain deposition disease (LCDD) is a rare disorder that very uncommonly affects the lung. We report 3 cases of severe cystic pulmonary LCDD leading to lung transplantation. Such a presentation has never been previously reported. The 3 patients present with a progressive obstructive pulmonary pattern associated with numerous cysts diffusely distributed in both lungs. The disease was histologically characterized by non amyloid amorphous deposits in the alveolar walls, the small airways and the vessels. It was associated with emphysematous-like changes and small airway dilatation. Monotypic Key words: light chain deposition disease, cystic lung disorder, lung transplantation
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light chain fixation was demonstrated on the abnormal deposits and along the basement membranes. Electron microscopy revealed coarsely granular electron-dense deposits in the same localizations. Mild extrapulmonary deposits were found in salivary glands in 1 case. No immunoproliferative disorder was identified. We conclude that LCDD may primarily affect the lung, present as a pulmonary cystic disorder, and lead to severe respiratory insufficiency.
