Dyspnea and cough in a 68-year-old female with light chain deposition disease

Abstract

Light chain deposition disease (LCDD) is a rare hematologic disorder characterized by non-amyloid monoclonal immunoglobulin light chain deposition in multiple organs. Pulmonary LCDD (PLCDD) is an uncommon manifestation of LCDD usually seen in middle-aged patients presenting with radiologic cystic and nodular findings. We report the case of a 68-year-old female who presented with shortness of breath and atypical chest pain. Chest computerized tomography (CT) scan revealed numerous diffuse but basilar predominant pulmonary cysts and mild bronchiectasis without nodular disease. Given concomitant abnormal renal function and hepatic laboratory indices, she underwent biopsy of both organs confirming the presence of LCDD. Directed chemotherapy was initiated and stabilized renal and hepatic progression, but on follow-up imaging, pulmonary disease appeared worse. While therapeutic options targeting other organ involvement are available, their directed efficacy for progressive lung disease is not well known.

Keywords: Cystic lung disease; Light chain deposition disease.

Fig. 1

Computed tomography images of PLCDD cystic disease at presentation (Panel row 1, A-D; with arrows pointing towards parenchymal cysts) and at 3-years of follow-up (Panel row 2, A-D), demonstrating mild progression in terms of increased cyst size and number.