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Pathomechanisms of cyst formation in pulmonary light chain deposition disease

¹ Service d'Anatomie Pathologique, Hôpital Tenon, ⁴ Service de Pneumologie, Hôpital Bichat, ⁵ Service d'Anatomie Pathologique, Hôpital Européen Georges Pompidou, Paris, ² Inserm, UMRS 903, IFR53, ³ Laboratoire d'Histologie, UFR Médecine, CHU Maison Blanche, Reims, and ⁶ Service de Pneumologie, Hôpital Foch, Suresnes, France.

CORRESPONDENCE: M. Colombat, Service d'Anatomie Pathologique, Hôpital Tenon, 4 Rue de la Chine, 75020 Paris, France. Fax: 33 156017876. E-mail: colombatm{at}yahoo.fr

Keywords: Cyst, elastic fibre, light chain deposition disease, lung, matrix metalloproteinases

Received: October 8, 2007
Accepted March 25, 2008

Cystic lung light chain deposition disease (CL-LCDD) is a recently described rare disorder characterised by numerous cysts and diffuse monoclonal nonamyloid light chain deposits surrounded by macrophagic giant cells. The mechanisms responsible for cyst development remain unknown.

The objectives of the present study were to analyse the major components of the pulmonary extracellular matrix in CL-LCDD and to determine the influence of metalloproteinases (MMPs) by comparison with other cystic lung disorders.

A virtually complete degradation of the elastic network was found in CL-LCDD. To a lesser degree, loss of fibrillar and basement membrane collagens was also observed. Macrophagic giant cells expressed MMP-1, MMP-2, MMP-9, MMP-12 and MMP-14 and in situ zymography highlighted a strong gelatinolytic activity. As in CL-LCDD, cystic lesions in Langerhans' cell histiocytosis (LCH) and lymphangioleiomyomatosis (LAM) were characterised by the lack of elastic fibres. Similarly, MMP were expressed in CL-LCDD and LCH but the labelled cells were different. In contrast, few MMPs were detected in LAM.

In conclusion, elastolysis is common to cystic lung light chain deposition disease and other cystic lung disorders, suggesting its implication in cyst formation. Moreover, in cystic lung light chain deposition disease, a role of metalloproteinases in elastolysis is strongly indicated by the metalloproteinase expression and activity pattern.