For the past 4 years the chest radiograph had shown progressive, reticulonodular shadows, especially in the mid and lower zones. A CT showed multiple cystic lesions up to 1.5 cm. Transbronchial biopsy showed small nodules of amyloid with focal calcification. There was a mild lymphoplasmacytic infiltrate that showed lambda restriction. Whereas in some cases the deposits are confined to the vessels, nodules also occur, as in our case.
Perivascular deposits destroy normal muscle and elastic tissue.
Deposits differ from amyloid in that they are PAS positive, do not stain with Congo red or show green birefringence, and have an electron dense, granular, but not fibrillar, appearance by electron microscopy. Signs and symptoms in both diseases depend on the organ involved and extent of involvement and not the type of deposit. Both recur in transplants. Although both diseases may be systemic, both may be local. Primary LCDD may have a similar cause.