light chain deposition disease LCDD
- related: cystic lung diseases
- tags: #literature #boards
LCDD is also a progressive cystic lung disease that can lead to respiratory failure. LCDD is a monoclonal immunoglobulin deposition disease related to amyloid light chain amyloidosis in which fibril formation does not occur but fragments of monoclonal light chains are deposited in the tissues. LCDD can cause renal disease—such as nephrotic syndrome and/or renal failure leading to end-stage kidney disease requiring dialysis—but not renal AML (choice B is incorrect). It can also affect other organs such as liver, heart, salivary glands, peripheral nerves, GI tract, and skin. Patients may progress to overt multiple myeloma. HRCT findings can vary from multiple small round cysts in a diffuse distribution mimicking LAM to large cystic lesions associated with reticulonodular opacities mimicking PLCH. Histopathologically, LCDD is characterized by kappa light chain deposition in the alveolar walls, small airways, and vessels accompanied by emphysematous changes and dilation of small airways.1